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Mandorfer M, Abraldes JG, Berzigotti A. Non-invasive assessment of portal hypertension: Liver stiffness and beyond. JHEP Rep 2025; 7:101300. [PMID: 40034396 PMCID: PMC11874574 DOI: 10.1016/j.jhepr.2024.101300] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/17/2024] [Revised: 11/08/2024] [Accepted: 12/05/2024] [Indexed: 03/05/2025] Open
Abstract
Portal hypertension (PH) leads to life-threatening clinical manifestations such as bleeding from gastro-oesophageal varices, ascites and its complications, and portosystemic encephalopathy. It can develop because of advanced chronic liver disease (ACLD) or due to rarer causes such as vascular liver disease. Reference standard methods to assess PH in ACLD include the measurement of hepatic venous pressure gradient and endoscopy, which have limitations due to their high resource utilisation and invasiveness. Non-invasive tests (NITs) have entered clinical practice and allow invasive procedures to be reserved for patients with indeterminate findings on NITs or for specific clinical questions. In this review, we present an update on the role of NITs, and in particular ultrasound elastography, to diagnose PH in ACLD and vascular liver disease, and to stratify the risk of liver-related events. We also provide insights into the open research questions and design of studies in this field.
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Affiliation(s)
- Mattias Mandorfer
- Department of Medicine III, Division of Gastroenterology and Hepatology, Medical University of Vienna, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Department of Medicine III, Division of Gastroenterology and Hepatology, Medical University of Vienna, Vienna, Austria
| | - Juan G. Abraldes
- Division of Gastroenterology (Liver Unit). University of Alberta, Edmonton, Alberta, Canada
| | - Annalisa Berzigotti
- Department of Visceral Surgery and Medicine, Inselspital, Bern University Hospital, University of Bern, Switzerland
- Department for BioMedical Research, Visceral Surgery and Medicine, University of Bern, Switzerland
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Li P, Schiano TD, Thung SN, Ward SC, Fiel MI. Shared Features of Obliterative Portal Venopathy, Normal Liver, and Chronic Liver Disease: A Histologic and Morphometric Analysis. Mod Pathol 2025; 38:100739. [PMID: 39986468 DOI: 10.1016/j.modpat.2025.100739] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2024] [Revised: 01/27/2025] [Accepted: 02/06/2025] [Indexed: 02/24/2025]
Abstract
Obliterative portal venopathy (OPV) is a cause of noncirrhotic portal hypertension, and its diagnosis is challenging, as the features are heterogeneous, subtle, and may be mistaken as "normal." We sought to compare OPV cases (n = 72; 326 total portal tracts [PT]) with 2 control groups: control group 1 comprised of normal liver (n = 40; 192 PTs) and control group 2 comprised of liver biopsies with chronic liver disease with OPV features (n = 40; 200 PTs). Morphometry was applied to determine the overall PT area and the luminal area of dystrophic portal veins (PVs). The frequency of absent native PVs was determined. Using trichrome-stained slides, approximately 5 PTs were randomly selected for morphometry utilizing Philips IntelliSite Pathology Solution 3.3. Clinical data were extracted from electronic health records. Of the 326 PTs in the OPV cases, phlebosclerosis was found in 31.6%, densely fibrotic PTs in 12.7%, dystrophic PVs in 31.4%, and absent native PVs in 44.5%. When comparing the OPV group with control group 1, dystrophic PVs, absent native PVs, phlebosclerosis, fibrotic PTs, greater luminal area of dystrophic PV, and a higher ratio of dystrophic PV area to PT area were more frequently found in the OPV group. No significant difference in overall PT area was found. When comparing control group 2 with OPV cases, densely fibrotic PTs were more frequent when compared with OPV cases. This study shows that absent native PVs are the most frequent feature in OPV. Other features that are less frequent but still significantly different from normal liver include dystrophic PVs, greater luminal area of dystrophic PVs, phlebosclerosis, and PT fibrosis. Except for densely fibrotic PTs in control group 2, all other features showed similar frequency as OPV. Pathologists should be aware that OPV features may be present in liver biopsies from both normal and chronic liver diseases.
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Affiliation(s)
- Peizi Li
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, New York
| | - Thomas D Schiano
- Division of Liver Diseases, Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, New York
| | - Swan N Thung
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, New York
| | - Stephen C Ward
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, New York
| | - M Isabel Fiel
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, New York.
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Bihari C, Dhariwal S, Shasthry SM, Rastogi A, Sharma MK, Sarin SK. Dissociation in hepatic vein pressure gradient, liver stiffness measurement and complications in histological subtypes of porto-sinusoidal vascular disease. J Clin Pathol 2025; 78:169-176. [PMID: 38242555 DOI: 10.1136/jcp-2023-209321] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2023] [Accepted: 01/08/2024] [Indexed: 01/21/2024]
Abstract
BACKGROUND AND AIMS Portosinusoidal vascular disease (PSVD) is a broad term encompassing varied histological patterns with changes in portal tracts and sinusoids without cirrhosis. We aimed to assess whether there is any clinical and pathological difference among the various histological categories of PSVD. PATIENTS AND METHODS This study included liver biopsy cases classified as PSVD (2020-2022). Clinical and laboratory parameters were obtained from the electronic records. PSVD cases were histologically categorised as obliterative portal venopathy (OPV), OPV with fibrosis (OPV-F), incomplete septal cirrhosis (ISC), nodular regenerative hyperplasia (NRH), mega sinusoids with fibrosis (MSF) and unclassified. Follow-up complications were recorded. RESULTS PSVD categories were OPV (45 (26%)), OPV-F (37 (21.4%)), ISC (20 (11.6%)), NRH (19 (11%)), MSF (19 (11%)) and unclassified (33 (19%)). Elevated hepatic venous pressure gradient (HVPG) was noted in OPV-F (10 (IQR: 12-14.7)) and ISC (12 (IQR: 9-14)) mm Hg with higher fibrosis quantity in liver tissue and elevated procollagen III aminoterminal propeptide, which correlated with HVPG. On immunohistochemistry, OPV-F and ISC showed lesser expression of ADAMT13 in liver biopsies (p<0.001). On follow-up, ascites development was more in OPV-F and ISC than in other categories (p=0.001). Higher liver stiffness measurement (LSM) values were recorded in MSF and NRH, compared with other categories, but it did not correlate with fibrosis in liver biopsy. CONCLUSIONS OPV-F and ISC had higher HVPG, fibrosis, and more ascites development on follow-up than the other categories of PSVD, and all are not the same. In contrast, MSF and NRH have spuriously high LSM.
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Affiliation(s)
- Chhagan Bihari
- Institute of Liver and Biliary Sciences, New Delhi, Delhi, India
| | - Sneha Dhariwal
- Institute of Liver and Biliary Sciences, New Delhi, Delhi, India
| | | | - Archana Rastogi
- Institute of Liver and Biliary Sciences, New Delhi, Delhi, India
| | | | - Shiv Kumar Sarin
- Institute of Liver and Biliary Sciences, New Delhi, Delhi, India
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Balcar L, Dominik N, Mozayani B, Semmler G, Halilbasic E, Mandorfer M, Reiberger T, Trauner M, Scheiner B, Stättermayer AF. Elevated Hepatic Copper Content in Porto-Sinusoidal Vascular Disorder (PSVD): Leading Down a Wrong Track. Liver Int 2025; 45:e16175. [PMID: 39807082 PMCID: PMC11730389 DOI: 10.1111/liv.16175] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/14/2024] [Revised: 10/23/2024] [Accepted: 11/05/2024] [Indexed: 01/16/2025]
Abstract
BACKGROUND AND AIMS Porto-sinusoidal vascular disorder (PSVD) is a rare vascular liver disorder characterised by specific histological findings in the absence of cirrhosis, which is poorly understood in terms of pathophysiology. While elevated hepatic copper content serves as diagnostic hallmark in Wilson disease (WD), hepatic copper content has not yet been investigated in PSVD. METHODS Patients with a verified diagnosis of PSVD at the Medical University of Vienna and available hepatic copper content at the time of diagnosis of PSVD were retrospectively included. Elevated hepatic copper content was correlated with cholestatic changes and WD diagnostics in PSVD and analysed for liver-related outcomes (first/further hepatic decompensation/liver-related death). RESULTS Overall, 92 patients were included into this study (mean age 49 ± 16; 57% male; median hepatic copper content was 30 [IQR: 18-55] μg/g) of whom 29 (32%) had moderately (≥ 50 μg/g) and 4 (4%) strongly (≥ 250 μg/g) elevated hepatic copper content. Elevated levels of hepatic copper were associated with younger age in multivariable linear regression analysis. After adjusting for age, decompensation status and albumin, hepatic copper content was significantly associated with the outcome of interest (log, per 10; aHR: 1.60 [95% CI: 1.14-2.25]; p = 0.007). A hepatic copper cut-off at ≥ 90 μg/g identified PSVD patients with considerable risk of liver-related outcomes (at 2 years: 51% vs. 12%). CONCLUSION Elevated hepatic copper seems frequent in patients with PSVD even in the absence of cholestatic features, especially in young patients, which makes differential diagnosis to WD challenging. Since PSVD patients with elevated hepatic copper content had increased risk for liver-related outcomes, the pathomechanisms underlying hepatic copper accumulation in PSVD should be investigated as this may open new therapeutic avenues.
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Affiliation(s)
- Lorenz Balcar
- Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Clinical Research Group MOTIONMedical University of ViennaViennaAustria
| | - Nina Dominik
- Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Clinical Research Group MOTIONMedical University of ViennaViennaAustria
| | - Behrang Mozayani
- Department of PathologyMedical University of ViennaViennaAustria
| | - Georg Semmler
- Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Clinical Research Group MOTIONMedical University of ViennaViennaAustria
| | - Emina Halilbasic
- Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
| | - Mattias Mandorfer
- Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Clinical Research Group MOTIONMedical University of ViennaViennaAustria
| | - Thomas Reiberger
- Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Clinical Research Group MOTIONMedical University of ViennaViennaAustria
| | - Michael Trauner
- Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Clinical Research Group MOTIONMedical University of ViennaViennaAustria
| | - Bernhard Scheiner
- Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Clinical Research Group MOTIONMedical University of ViennaViennaAustria
| | - Albert Friedrich Stättermayer
- Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Medicine IIIMedical University of ViennaViennaAustria
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Kolb JM, Monachese M, Rubin RA, Wang TJ, Choi A, Bazarbashi AN, Brahmbhatt B, Zakaria A, Cortes P, Kesar V, Abel WF, Chen WP, McLaren C, Tavangar A, Singal AG, Taunk P, Wallace MB, Kedia P, Lee D, Abbas A, Yeaton P, Cosgrove N, Kesar V, Chang KJ, Ryou M, Samarasena J. Endoscopic Ultrasound-Guided Portosystemic Pressure Gradient Correlates with Clinical Parameters and Liver Histology. Clin Gastroenterol Hepatol 2025:S1542-3565(25)00078-3. [PMID: 39892628 DOI: 10.1016/j.cgh.2024.12.022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/04/2024] [Revised: 12/13/2024] [Accepted: 12/24/2024] [Indexed: 02/04/2025]
Abstract
BACKGROUND AND AIMS Endoscopic ultrasound-guided portosystemic pressure gradient measurement (EUS-PPG) is a novel technique to evaluate for portal hypertension (PH), a diagnosis that can prognosticate and guide therapy for patients. This study evaluated the safety and efficacy of EUS-PPG and correlation with clinical parameters and liver histology. METHODS We conducted a multicenter, retrospective study of patients undergoing EUS-PPG from January 2020 to December 2022 for suspected liver disease or PH. Linear regression was used to examine the relationship between EUS-PPG and clinical parameters of PH, and the chi-square test, Fisher's exact test, and Wilcoxon rank sum test described correlation with liver biopsy histology and noninvasive markers of fibrosis (Fibrosis-4, APRI [aspartate aminotransferase-to-platelet ratio index]). Logistic regression was performed to identify the strongest predictor of histologic cirrhosis. RESULTS Across 8 centers, 385 patients were enrolled and 373 had successful EUS-PPG (technical success 97%). Higher median PPGs were observed in patients with than without esophageal varices (11.6 mm Hg vs 4.1 mm Hg), portal hypertensive gastropathy (10.5 mm Hg vs 3.3 mm Hg), and thrombocytopenia (7.6 mm Hg vs 4.4 mm Hg) (P < .001). Individuals with PH and clinically significant PH (PPG ≥10) were 6.7 and 3.8 times more likely to have cirrhosis on histology, respectively. EUS-PPG was the best overall predictor of biopsy-proven cirrhosis (area under the curve 0.84) compared with Fibrosis-4 (0.72), and APRI (0.54). There were 2 minor adverse events related to PPG (postprocedural pain). CONCLUSIONS EUS-PPG measurement was technically feasible and safe and demonstrated a strong correlation with clinical parameters of PH and liver histology. The strongest predictor of cirrhosis was EUS-PPG >5 mm Hg, which outperformed noninvasive markers of fibrosis.
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Affiliation(s)
- Jennifer M Kolb
- Vatche and Tamar Manoukian Division of Digestive Diseases, David Geffen School of Medicine at UCLA, Los Angeles, California
| | - Marc Monachese
- Division of Gastroenterology and Hepatology, University of California, Irvine, Orange, California
| | - Raymond A Rubin
- Transplant Hepatology, Piedmont Transplant Institute, Atlanta, Georgia
| | - Thomas J Wang
- Division of Gastroenterology, Hepatology and Endoscopy, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Alyssa Choi
- Division of Gastroenterology and Hepatology, University of California, Irvine, Orange, California
| | - Ahmad N Bazarbashi
- Division of Gastroenterology and Hepatology, Washington University School of Medicine in St. Louis, St. Louis, Missouri
| | - Bhaumik Brahmbhatt
- Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, Florida
| | - Ali Zakaria
- Division of Digestive Diseases and Nutrition, University of South Florida Morsani College of Medicine, Tampa, Florida
| | - Pedro Cortes
- Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, Florida
| | - Varun Kesar
- Gastroenterology, Virginia Tech Carilion School of Medicine, Roanoke, Virginia
| | - William F Abel
- Gastroenterology, Virginia Tech Carilion School of Medicine, Roanoke, Virginia
| | - Wen-Pin Chen
- Biostatistics Shared Resource, Chao Family Comprehensive Cancer Center, University of California, Irvine, Orange, California
| | - Christine McLaren
- Biostatistics Shared Resource, Chao Family Comprehensive Cancer Center, University of California, Irvine, Orange, California
| | - Amirali Tavangar
- Division of Gastroenterology and Hepatology, University of California, Irvine, Orange, California
| | - Amit G Singal
- Division of Digestive and Liver Diseases, University of Texas Southwestern Medical Center, Dallas, Texas
| | - Pushpak Taunk
- Division of Digestive Diseases and Nutrition, University of South Florida Morsani College of Medicine, Tampa, Florida
| | - Michael B Wallace
- Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, Florida; Division of Gastroenterology and Hepatology, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates
| | - Prashant Kedia
- Gastroenterology, Methodist Dallas Medical Center, Dallas, Texas
| | - David Lee
- Gastroenterology, Methodist Dallas Medical Center, Dallas, Texas
| | - Ali Abbas
- Division of Digestive Diseases and Nutrition, University of South Florida Morsani College of Medicine, Tampa, Florida
| | - Paul Yeaton
- Gastroenterology, Virginia Tech Carilion School of Medicine, Roanoke, Virginia
| | - Natalie Cosgrove
- Division of Gastroenterology and Hepatology, Washington University School of Medicine in St. Louis, St. Louis, Missouri
| | - Vivek Kesar
- Gastroenterology, Virginia Tech Carilion School of Medicine, Roanoke, Virginia
| | - Kenneth J Chang
- Division of Gastroenterology and Hepatology, University of California, Irvine, Orange, California
| | - Marvin Ryou
- Division of Gastroenterology, Hepatology and Endoscopy, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Jason Samarasena
- Division of Gastroenterology and Hepatology, University of California, Irvine, Orange, California.
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Semmler G, Petrenko O, Lozano JJ, Shalaby S, Sánchez-Avila JI, Marella N, Hannich T, Wöran K, Balcar L, Simbrunner B, Lampichler K, Mozayani B, Trauner M, Mandorfer M, Reiberger T, García-Pagán JC, Scheiner B. Metabolomic profiles differentiate between porto-sinusoidal vascular disorder, cirrhosis, and healthy individuals. JHEP Rep 2024; 6:101208. [PMID: 39624234 PMCID: PMC11609546 DOI: 10.1016/j.jhepr.2024.101208] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/11/2023] [Revised: 07/30/2024] [Accepted: 08/27/2024] [Indexed: 04/03/2025] Open
Abstract
Background & Aims Porto-sinusoidal vascular disorder (PSVD) is a rare and diagnostically challenging vascular liver disease. This study aimed to identify distinct metabolomic signatures in patients with PSVD or cirrhosis to facilitate non-invasive diagnosis and elucidate perturbed metabolic pathways. Methods Serum samples from 20 healthy volunteers (HVs), 20 patients with histologically confirmed PSVD or 20 patients with cirrhosis were analyzed. Metabolites were measured using liquid chromatography-mass spectrometry. Differential abundance was evaluated with Limma's moderated t-statistics. Artificial neural network and support vector machine models were developed to classify PSVD against cirrhosis or HV metabolomic profiles. An independent cohort was used for validation. Results A total of 283 metabolites were included for downstream analysis. Clustering effectively separated PSVD from HV metabolomes, although a subset of patients with PSVD (n = 5, 25%) overlapped with those with cirrhosis. Differential testing revealed significant PSVD-linked metabolic perturbations, including pertubations in taurocholic and adipic acids, distinguishing patients with PSVD from both HVs and those with cirrhosis. Alterations in pyrimidine, glycine, serine, and threonine pathways were exclusively associated with PSVD. Machine learning models utilizing selected metabolic signatures reliably differentiated the PSVD group from HVs or patients with cirrhosis using only 4 to 6 metabolites. Validation in an independent cohort demonstrated the high discriminative ability of taurocholic acid (AUROC 0.899) for patients with PSVD vs. HVs and the taurocholic acid/aspartic acid ratio (AUROC 0.720) for PSVD vs. cirrhosis. Conclusions High-throughput metabolomics enabled the identification of distinct metabolic profiles that differentiate between PSVD, cirrhosis, and healthy individuals. Unique alterations in the glycine, serine, and threonine pathways suggest their potential involvement in PSVD pathogenesis. Impact and implications Porto-sinusoidal vascular disorder (PSVD) is a vascular liver disease that can lead to pre-sinusoidal portal hypertension in the absence of cirrhosis, with poorly understood pathophysiology and no established treatment. Our study demonstrates that analyzing the serum metabolome could reveal distinct metabolic signatures in patients with PSVD, including alterations in the pyrimidine, glycine, serine and threonine pathways, potentially shedding light on the disease's underlying pathways. These findings could enable earlier and non-invasive diagnosis of PSVD, potentially reducing reliance on invasive procedures like liver biopsy and guiding diagnostic pathways.
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Affiliation(s)
- Georg Semmler
- Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Clinical Research Group MOTION, Medical University of Vienna, Vienna, Austria
| | - Oleksandr Petrenko
- Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Clinical Research Group MOTION, Medical University of Vienna, Vienna, Austria
- CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
- Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria
| | | | - Sarah Shalaby
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS). CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas). Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE-Liver). Departament de Medicina i Ciències de la Salut. Universitat de Barcelona, Spain
| | - Juan I. Sánchez-Avila
- CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
| | - Nara Marella
- CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
| | - Thomas Hannich
- CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
| | - Katharina Wöran
- Department of Pathology, Medical University of Vienna, Vienna, Austria
| | - Lorenz Balcar
- Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Clinical Research Group MOTION, Medical University of Vienna, Vienna, Austria
| | - Benedikt Simbrunner
- Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Clinical Research Group MOTION, Medical University of Vienna, Vienna, Austria
| | - Katharina Lampichler
- Clinical Research Group MOTION, Medical University of Vienna, Vienna, Austria
- Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria
| | - Behrang Mozayani
- Department of Pathology, Medical University of Vienna, Vienna, Austria
| | - Michael Trauner
- Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria
- Clinical Research Group MOTION, Medical University of Vienna, Vienna, Austria
| | - Mattias Mandorfer
- Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Clinical Research Group MOTION, Medical University of Vienna, Vienna, Austria
| | - Thomas Reiberger
- Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Clinical Research Group MOTION, Medical University of Vienna, Vienna, Austria
- CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
- Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria
| | - Juan-Carlos García-Pagán
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS). CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas). Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE-Liver). Departament de Medicina i Ciències de la Salut. Universitat de Barcelona, Spain
| | - Bernhard Scheiner
- Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Clinical Research Group MOTION, Medical University of Vienna, Vienna, Austria
- Department of Surgery and Cancer, Imperial College London, London, United Kingdom
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7
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Rautou PE, Giudicelli-Lett H, Magaz M, García-Pagán JC. Comment on "Poor long-term outcome in patients with porto-sinusoidal vascular disease (PSVD): Fact or disease misclassification?". J Hepatol 2024:S0168-8278(24)02731-4. [PMID: 39608460 DOI: 10.1016/j.jhep.2024.11.031] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/20/2024] [Accepted: 11/20/2024] [Indexed: 11/30/2024]
Affiliation(s)
- Pierre-Emmanuel Rautou
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France; AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France.
| | - Heloïse Giudicelli-Lett
- AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | - Marta Magaz
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS). CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas). Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE-Liver). Departament de Medicina i Ciències de la Salut. Universitat de Barcelona, Spain
| | - Juan Carlos García-Pagán
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS). CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas). Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE-Liver). Departament de Medicina i Ciències de la Salut. Universitat de Barcelona, Spain.
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8
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Elkrief L, Hernandez-Gea V, Senzolo M, Albillos A, Baiges A, Berzigotti A, Bureau C, Murad SD, De Gottardi A, Durand F, Garcia-Pagan JC, Lisman T, Mandorfer M, McLin V, Moga L, Nery F, Northup P, Nuzzo A, Paradis V, Patch D, Payancé A, Plaforet V, Plessier A, Poisson J, Roberts L, Salem R, Sarin S, Shukla A, Toso C, Tripathi D, Valla D, Ronot M, Rautou PE. Portal vein thrombosis: diagnosis, management, and endpoints for future clinical studies. Lancet Gastroenterol Hepatol 2024; 9:859-883. [PMID: 38996577 DOI: 10.1016/s2468-1253(24)00155-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/21/2024] [Revised: 04/27/2024] [Accepted: 05/08/2024] [Indexed: 07/14/2024]
Abstract
Portal vein thrombosis (PVT) refers to the development of a non-malignant obstruction of the portal vein, its branches, its radicles, or a combination. This Review first provides a comprehensive overview of all aspects of PVT, namely the specifics of the portal venous system, the risk factors for PVT, the pathophysiology of portal hypertension in PVT, the interest in non-invasive tests, as well as therapeutic approaches including the effect of treating risk factors for PVT or cause of cirrhosis, anticoagulation, portal vein recanalisation by interventional radiology, and prevention and management of variceal bleeding in patients with PVT. Specific issues are also addressed including portal cholangiopathy, mesenteric ischaemia and intestinal necrosis, quality of life, fertility, contraception and pregnancy, and PVT in children. This Review will then present endpoints for future clinical studies in PVT, both in patients with and without cirrhosis, agreed by a large panel of experts through a Delphi consensus process. These endpoints include classification of portal vein thrombus extension, classification of PVT evolution, timing of assessment of PVT, and global endpoints for studies on PVT including clinical outcomes. These endpoints will help homogenise studies on PVT and thus facilitate reporting, comparison between studies, and validation of future studies and trials on PVT.
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Affiliation(s)
- Laure Elkrief
- Faculté de médecine de Tours, et service d'hépato-gastroentérologie, Le Centre Hospitalier Régional Universitaire de Tours, Tours, France; Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France
| | - Virginia Hernandez-Gea
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, Institut de Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas, Madrid, Spain; Departament de Medicina i Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain
| | - Marco Senzolo
- Department of Surgery, Oncology and Gastroenterology, University of Padova, Padova, Italy
| | - Agustin Albillos
- Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas, Madrid, Spain; Departamento de Gastroenterología y Hepatología, Instituto Ramón y Cajal de Investigación Sanitaria, Hospital Universitario Ramon y Cajal, Madrid, Spain
| | - Anna Baiges
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, Institut de Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas, Madrid, Spain; Departament de Medicina i Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain
| | - Annalisa Berzigotti
- Department of Visceral Surgery and Medicine, Inselspital, Bern University Hospital, Bern, Switzerland
| | - Christophe Bureau
- Service d'Hépatologie Hôpital Rangueil, Université Paul Sabatier, Toulouse, France
| | - Sarwa Darwish Murad
- Department of Gastroenterology and Hepatology, Erasmus MC University Medical Center, Rotterdam, Netherlands
| | - Andrea De Gottardi
- Gastroenterology and Hepatology Department, Ente Ospedaliero Cantonale Faculty of Biomedical Sciences of Università della Svizzera Italiana, Lugano, Switzerland
| | - François Durand
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France
| | - Juan-Carlos Garcia-Pagan
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, Institut de Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas, Madrid, Spain; Departament de Medicina i Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain
| | - Ton Lisman
- Department of Surgery, University Medical Center Groningen, Groningen, Netherlands
| | - Mattias Mandorfer
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria
| | - Valérie McLin
- Swiss Pediatric Liver Center, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland
| | - Lucile Moga
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France
| | - Filipe Nery
- Immuno-Physiology and Pharmacology Department, School of Medicine and Biomedical Sciences, University of Porto, Portugal
| | - Patrick Northup
- Transplant Institute and Division of Gastroenterology, NYU Langone, New York, NY, USA
| | - Alexandre Nuzzo
- Intestinal Stroke Center, Department of Gastroenterology, IBD and Intestinal Failure, AP-HP Hôpital Beaujon, Clichy, France; Laboratory for Vascular and Translational Science, INSERM UMR 1148, Paris, France
| | - Valérie Paradis
- Department of Pathology, AP-HP Hôpital Beaujon, Clichy, France
| | - David Patch
- Department of Hepatology and Liver Transplantation, Royal Free Hospital, London, UK
| | - Audrey Payancé
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France
| | | | - Aurélie Plessier
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France
| | - Johanne Poisson
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service de Gériatrie, Hôpital Corentin Celton (AP-HP), Paris, France
| | - Lara Roberts
- Department of Haematological Medicine, King's College Hospital NHS Foundation Trust, London, UK
| | - Riad Salem
- Northwestern Memorial Hospital, Northwestern University, Chicago, IL, USA
| | - Shiv Sarin
- Institute of Liver and Biliary Sciences, New Delhi, India
| | - Akash Shukla
- Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, India
| | - Christian Toso
- Service de Chirurgie Viscérale, Hôpitaux Universitaires de Genève, Geneva, Switzerland
| | - Dhiraj Tripathi
- Department of Liver and Hepato-Pancreato-Biliary Unit, Queen Elizabeth Hospital, University Hospitals Birmingham, Birmingham, UK; Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK
| | - Dominique Valla
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France
| | - Maxime Ronot
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service de Radiologie, AP-HP Hôpital Beaujon, Clichy, France
| | - Pierre-Emmanuel Rautou
- Centre de recherche sur l'inflammation, Université Paris-Cité, Paris, France; Service d'Hépatologie, AP-HP Hôpital Beaujon, Clichy, France.
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9
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Premkumar M, Anand AC. Porto-sinusoidal Vascular Disease: Classification and Clinical Relevance. J Clin Exp Hepatol 2024; 14:101396. [PMID: 38601747 PMCID: PMC11001647 DOI: 10.1016/j.jceh.2024.101396] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2023] [Accepted: 03/05/2024] [Indexed: 04/12/2024] Open
Abstract
Non-cirrhotic portal hypertension (NCPH) is a well-recognized clinico-pathological entity, which is associated with clinical signs and symptoms, imaging, and endoscopic features of portal hypertension (PHT), in absence of cirrhosis. In patients with NCPH without known risk factors of PHT or extrahepatic portal vein thrombosis, the condition is called idiopathic non-cirrhotic portal hypertension (INCPH). There are multiple infectious, immune related causes, systemic diseases, drug and toxin exposures, haematological disorders, and metabolic risk factors that have been associated with this INCPH. However, the causal pathogenesis is still unclear. The Vascular liver disorders interest group group recently proposed porto-sinusoidal vascular disease (PSVD) as a syndromic entity, which provides definite histopathological criteria for diagnosis of NCPH (table 1). The three classical histo-morphological lesions specific for PSVD include obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal fibrosis. The PSVD definition includes patients with portal vein thrombosis, PVT, and even those without PHT, thus broadening the scope of diagnosis to include patients who may have presented early, prior to haemodynamic changes consistent with PHT. However, this new diagnosis has pros and cons. The cons include mandating invasive liver biopsy to assess the PSVD histological triad in all patients with NCPH, an erstwhile clinical diagnosis in Asian patients. In addition, the natural history of the subclinical forms of PSVD without PHT and linear progression to develop PHT is unknown yet. In this review, we discuss the diagnosis and treatment of INCPH/PSVD, fallacies and strengths of the old and new schema, pathobiology of this disease, and clinical correlates in an Asian context. Although formulation of standardised diagnostic criteria is useful for comparison of clinical cohorts with INCPH/PSVD, prospective clinical validation in global cohorts is necessary to avoid misclassification of vascular disorders of the liver.
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Affiliation(s)
- Madhumita Premkumar
- Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
| | - Anil C. Anand
- Department of Hepatology, Kalinga Institute of Medical Sciences, Bhubaneshwar, Odisha, India
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10
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Tonutti A, Pugliese N, Ceribelli A, Isailovic N, De Santis M, Colapietro F, De Nicola S, Polverini D, Selmi C, Aghemo A. The autoimmune landscape of Porto-sinusoidal vascular disorder: What the rheumatologist needs to know. Semin Arthritis Rheum 2024; 67:152467. [PMID: 38805899 DOI: 10.1016/j.semarthrit.2024.152467] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2024] [Revised: 04/23/2024] [Accepted: 04/29/2024] [Indexed: 05/30/2024]
Abstract
Porto-sinusoidal vascular disorder (PSVD) encompasses a group of vascular disorders characterized by lesions of the portal venules and sinusoids with clinical manifestations ranging from non-specific abnormalities in serum liver enzymes to clinically overt portal hypertension and related complications. Several reports have documented cases of PSVD in patients with systemic autoimmune conditions, such as systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis. It is of note that these diseases share specific pathophysiological features with PSVD, including endothelial dysfunction, vascular inflammation, and molecular signatures. This narrative review aims to summarize the current knowledge on the association between PSVD and systemic autoimmune diseases, emphasizing the importance of promptly recognizing this condition in the rheumatological practice, and highlighting the key aspects where further research is necessary from both pathogenic and clinical perspectives.
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Affiliation(s)
- Antonio Tonutti
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Italy
| | - Nicola Pugliese
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; Division of Internal Medicine and Hepatology, Department of Gastroenterology, IRCCS Humanitas Research Hospital, Rozzano, Italy
| | - Angela Ceribelli
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Italy
| | - Natasa Isailovic
- Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Italy
| | - Maria De Santis
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Italy
| | - Francesca Colapietro
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; Division of Internal Medicine and Hepatology, Department of Gastroenterology, IRCCS Humanitas Research Hospital, Rozzano, Italy
| | - Stella De Nicola
- Division of Internal Medicine and Hepatology, Department of Gastroenterology, IRCCS Humanitas Research Hospital, Rozzano, Italy
| | - Davide Polverini
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; Division of Internal Medicine and Hepatology, Department of Gastroenterology, IRCCS Humanitas Research Hospital, Rozzano, Italy
| | - Carlo Selmi
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Italy.
| | - Alessio Aghemo
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; Division of Internal Medicine and Hepatology, Department of Gastroenterology, IRCCS Humanitas Research Hospital, Rozzano, Italy
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11
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Malik A, Malik S, Farooq A, Malik MI, Javaid S. Histopathological features of idiopathic portal hypertension: A systematic review and meta-analysis. Sci Prog 2024; 107:368504241264996. [PMID: 39053026 PMCID: PMC11282518 DOI: 10.1177/00368504241264996] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/27/2024]
Abstract
BACKGROUND Portal hypertension (PH) is a clinically significant entity that could present with life-threatening gastrointestinal bleeding. Cirrhosis is the most common cause of PH, with well-documented histopathology and etiology. However, in idiopathic portal hypertension (IPH), no single histopathologic finding is associated with PH. Our systematic review aims to identify and summarize the prevalence of the common histological findings of IPH. METHODS We systematically searched PubMed, Cochrane CENTRAL, Web of Science, and Scopus till 1ST March 2022 for studies describing the histopathological features of IPH. Data were extracted from eligible studies and pooled as events rate and 95% confidence interval (CI) using binary random-effects model by open meta-analyst software. RESULTS We included 23 retrospective studies with a total sample size of 813 patients. The overall incidence of nodular regenerative hyperplasia was 38.6%, 59.8% for portal fibrosis, 51.3% for periportal fibrosis, 39.3% for perisinusoidal fibrosis, 89.8% for portal vein sclerosis, 42.2% for portal inflammation, 53.3% for mega-sinusoids, 39.5% for thickening of portal vein branches, 93.8% for narrowing of portal veins, 53.3% for hepatic veins/venous outflow obstruction, 51.4% for aberrant portal/periportal vessels, 42.4% for shunt vessel, 50.9% for ductular proliferation, and 16.3% for steatosis. CONCLUSION Due to the relatively non-pathognomonic and non-specific nature of IPH, a combination of different histological features such as the portal and periportal fibrosis, portal vein sclerosis, mega-sinusoids, narrowing of portal veins, hepatic venous outflow obstruction, aberrant portal or periportal vessels, and ductular proliferation may be of value in diagnosing IPH as the incidence rate of these features was at approximately 50%.
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Affiliation(s)
- Adnan Malik
- Mountain Vista Medical Center, Midwestern University Program, Mesa AZ, USA
| | - Sohira Malik
- Penn State College of Medicine, Hershey, PA, USA
| | - Ahsan Farooq
- Penn State College of Medicine, Hershey, PA, USA
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12
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Kalil JA, Deschenes M, Perrier H, Zlotnik O, Metrakos P. Navigating Complex Challenges: Preoperative Assessment and Surgical Strategies for Liver Resection in Patients with Fibrosis or Cirrhosis. Biomedicines 2024; 12:1264. [PMID: 38927471 PMCID: PMC11201140 DOI: 10.3390/biomedicines12061264] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2024] [Revised: 05/28/2024] [Accepted: 06/03/2024] [Indexed: 06/28/2024] Open
Abstract
This review explores the intricacies of evaluating cirrhotic patients for liver resection while exploring how to extend surgical intervention to those typically excluded by the Barcelona Clinic Liver Cancer (BCLC) criteria guidelines by focusing on the need for robust preoperative assessment and innovative surgical strategies. Cirrhosis presents unique challenges and complicates liver resection due to the altered physiology of the liver, portal hypertension, and liver decompensation. The primary objective of this review is to discuss the current approaches in assessing the suitability of cirrhotic patients for liver resection and aims to identify which patients outside of the BCLC criteria can safely undergo liver resection by highlighting emerging strategies that can improve surgical safety and outcomes.
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Affiliation(s)
- Jennifer A. Kalil
- Department of Surgery, Royal Victoria Hospital, McGill University Health Center, 1001 Blvd Decarie, Montreal, QC H4A 3J1, Canada; (J.A.K.); (H.P.); (O.Z.)
- Cancer Research Program, McGill University Health Center, Research Institute, 1001 Blvd Decarie, Montreal, QC H4A 3J1, Canada
| | - Marc Deschenes
- Department of Medicine, Division of Gastroenterology & Hepatology & Transplantation, Royal Victoria Hospital, McGill University Health Center, 1001 Blvd Decarie, Montreal, QC H4A 3J1, Canada;
| | - Hugo Perrier
- Department of Surgery, Royal Victoria Hospital, McGill University Health Center, 1001 Blvd Decarie, Montreal, QC H4A 3J1, Canada; (J.A.K.); (H.P.); (O.Z.)
| | - Oran Zlotnik
- Department of Surgery, Royal Victoria Hospital, McGill University Health Center, 1001 Blvd Decarie, Montreal, QC H4A 3J1, Canada; (J.A.K.); (H.P.); (O.Z.)
- Cancer Research Program, McGill University Health Center, Research Institute, 1001 Blvd Decarie, Montreal, QC H4A 3J1, Canada
| | - Peter Metrakos
- Department of Surgery, Royal Victoria Hospital, McGill University Health Center, 1001 Blvd Decarie, Montreal, QC H4A 3J1, Canada; (J.A.K.); (H.P.); (O.Z.)
- Cancer Research Program, McGill University Health Center, Research Institute, 1001 Blvd Decarie, Montreal, QC H4A 3J1, Canada
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13
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Mironova M, Gopalakrishna H, Viana Rodriguez GM, Abdul Majeed N, Hitawala AA, Fuss IJ, Bergerson JRE, Faust AJ, Laurin JM, Norman-Wheeler J, Scott S, Hercun J, Redd B, Kleiner DE, Koh C, Heller T. Prospective evaluation of patients with non-cirrhotic portal hypertension: A single centre study. Aliment Pharmacol Ther 2024; 59:1527-1538. [PMID: 38629442 DOI: 10.1111/apt.17987] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2023] [Revised: 12/19/2023] [Accepted: 03/24/2024] [Indexed: 05/22/2024]
Abstract
BACKGROUND Non-cirrhotic portal hypertension (NCPH) is a spectrum of liver diseases, including porto-sinusoidal vascular disorder, with portal hypertension (PH) in the absence of cirrhosis. The natural history and diagnostic approach to NCPH are not well understood. AIM We aimed to evaluate disease progression and outcomes in NCPH. METHODS Patients with or at risk for NCPH were enrolled in a single centre prospective study; two groups were formed based on the presence of specific features of PH, such as varices, collaterals, portal hypertensive gastropathy or portal hypertensive bleeding. All participants underwent a baseline liver biopsy. Liver stiffness measurement (LSM), and imaging were repeated every 6-12 months. RESULTS Fifteen patients without specific features of PH (Group I), and 35 patients with specific features (Group II) were enrolled. The median follow-up time was 50 months. Group II had higher hepatic venous pressure gradients, non-invasive measures of PH and a lower platelet count (PLT) when compared to Group I. Rates of survival and decompensation were similar in both groups. Patients with PLT ≤100 K/mcL had lower survival compared to those with PLT >100 K/mcL. Patients with LSM ≥10 kPa had lower survival and survival without decompensation when compared to patients with LSM <10 kPa. CONCLUSIONS Patients irrespective of specific features of PH had similar survival or survival without decompensation. Patients without specific features are at risk for disease progression and should be monitored closely. Thrombocytopenia and increased LSM are associated with severe forms of liver disease, which are strongly associated with outcomes.
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Affiliation(s)
- Maria Mironova
- Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Harish Gopalakrishna
- Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Gracia Maria Viana Rodriguez
- Digestive Disease Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Nehna Abdul Majeed
- Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Asif A Hitawala
- Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Ivan J Fuss
- Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Jenna R E Bergerson
- Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Alison J Faust
- Division of Gastroenterology and Hepatology, Department of Medicine, Pennsylvania State University Milton S. Hershey Medical Center, Hershey, PA, USA
| | - Jacqueline M Laurin
- Department of Hepatology, Sibley Memorial Hospital, Johns Hopkins University, Washington, DC, USA
| | - Jaha Norman-Wheeler
- Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Shani Scott
- Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Julian Hercun
- Liver Unit, Centre Hospitalier de l'Universite de Montreal, Montreal, Quebec, Canada
| | - Bernadette Redd
- Radiology and Imaging Sciences, Clinical Center, National Institutes of Health, Bethesda, MD, USA
| | - David E Kleiner
- Department of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
| | - Christopher Koh
- Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Theo Heller
- Translational Hepatology Section, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
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14
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Willington AJ, Tripathi D. Current concepts in the management of non-cirrhotic non-malignant portal vein thrombosis. World J Hepatol 2024; 16:751-765. [PMID: 38818283 PMCID: PMC11135268 DOI: 10.4254/wjh.v16.i5.751] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2023] [Revised: 02/20/2024] [Accepted: 04/08/2024] [Indexed: 05/22/2024] Open
Abstract
Non-cirrhotic non-malignant portal vein thrombosis (NCPVT) is an uncommon condition characterised by thrombosis of the portal vein, with or without extension into other mesenteric veins, in the absence of cirrhosis or intra-abdominal malignancy. Complications can include intestinal infarction, variceal bleeding and portal biliopathy. In this article, we address current concepts in the management of NCPVT including identification of risk factors, classification and treatment, and review the latest evidence on medical and interventional management options.
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Affiliation(s)
- Adam J Willington
- Department of Hepatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2GW, United Kingdom
| | - Dhiraj Tripathi
- Department of Hepatology, University Hospitals Birmingham, Birmingham B15 2TH, United Kingdom
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15
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Yano R, Tadokoro T, Morishita A, Ibuki E, Masaki T. A Case of Idiopathic Portal Hypertension Diagnosed by Noninvasive Fibrosis Evaluation Using Elastography. Cureus 2024; 16:e56432. [PMID: 38638786 PMCID: PMC11024665 DOI: 10.7759/cureus.56432] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/18/2024] [Indexed: 04/20/2024] Open
Abstract
Idiopathic portal hypertension (IPH) is often misdiagnosed as liver cirrhosis. Because it is difficult to distinguish between the two using diagnostic imaging, invasive tests, such as pathology and hepatic vein pressure gradient measurement, are necessary to make a diagnosis. Several studies have shown that the measurement of liver and spleen stiffnesses using elastography is useful in the diagnosis of IPH; however, there are few concrete reports on this subject. Herein, we report the case of a 58-year-old woman with IPH in which elastography was helpful for the diagnosis.
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Affiliation(s)
- Rie Yano
- Gastroenterology and Neurology, Kagawa University, Kita-gun, JPN
| | - Tomoko Tadokoro
- Gastroenterology and Neurology, Kagawa University, Kita-gun, JPN
| | | | - Emi Ibuki
- Diagnostic Pathology, Kagawa University, Kita-gun, JPN
| | - Tsutomu Masaki
- Gastroenterology and Neurology, Kagawa University, Kita-gun, JPN
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16
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Gioia S, De Santis A, d'Amati G, Nardelli S, Spagnoli A, Rocco AD, Ridola L, Riggio O. Application of ultrasonography-elastography score to suspect porto-sinusoidal vascular disease in patients with portal vein thrombosis. Hepatobiliary Pancreat Dis Int 2024; 23:20-24. [PMID: 37468349 DOI: 10.1016/j.hbpd.2023.07.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/19/2022] [Accepted: 07/05/2023] [Indexed: 07/21/2023]
Abstract
BACKGROUND Porto-sinusoidal vascular disease (PSVD) and portal vein thrombosis (PVT) are causes of portal hypertension characterized respectively by an intrahepatic and a pre-hepatic obstacle to the flow in the portal system. As PVT may be a consequence of PSVD, in PVT patients at presentation, a pre-existing PSVD should be suspected. In these patients the identification of an underlying PSVD would have relevant implication regarding follow-up and therapeutic management, but it could be challenging. In this setting ultrasonography may be valuable in differential diagnosis. The aim of the study was to use ultrasonography to identify parameters to discriminate between PSVD and "pure" PVT and then to suspect PVT secondary to a pre-existing PSVD. METHODS Fifty-three patients with histologically proven PSVD and forty-eight patients affected by chronic PVT were enrolled and submitted to abdominal ultrasonography with elastography by acoustic radiation force impulse (ARFI). RESULTS ARFI was higher and superior mesenteric vein (SMV) diameter was wider in PSVD patients than in PVT patients. Thus, a prognostic score was obtained as linear combinations of the two parameters with a good discrimination capacity between PSVD and PVT (the area under the curve = 0.780; 95% confidence interval: 0.690-0.869). CONCLUSIONS A score based on ARFI and SMV diameter may be useful to suspect an underlying PSVD in patients with PVT and to identify a subgroup of patients to be submitted to liver biopsy.
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Affiliation(s)
- Stefania Gioia
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy.
| | - Adriano De Santis
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
| | - Giulia d'Amati
- Department of Radiological, Oncological, and Pathological Sciences, Sapienza University of Rome, Rome, Italy
| | - Silvia Nardelli
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
| | - Alessandra Spagnoli
- Department of Public Health and Infectious Diseases, Sapienza University of Rome, Rome, Italy
| | - Arianna Di Rocco
- Department of Public Health and Infectious Diseases, Sapienza University of Rome, Rome, Italy
| | - Lorenzo Ridola
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
| | - Oliviero Riggio
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
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17
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Campreciós G, Bartrolí B, Montironi C, Belmonte E, García-Pagán JC, Hernández-Gea V. Porto-sinusoidal vascular disorder. SINUSOIDAL CELLS IN LIVER DISEASES 2024:445-464. [DOI: 10.1016/b978-0-323-95262-0.00022-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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18
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Kagihara JE, Goyes D, Rabiee A. Diagnosis and Management of Noncirrhotic Portal Hypertension. CURRENT HEPATOLOGY REPORTS 2023; 22:252-262. [DOI: 10.1007/s11901-023-00619-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 10/10/2023] [Indexed: 01/04/2025]
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19
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Liu B, Zhang D, Dong C, Yue Z, Wang L, Fan Z, Wu Y, Zhang K, Jiang L, Ding H, Zhang Y, Wang J, Liu F. Correlation between hepatic venous pressure gradient and portal pressure gradient in patients with autoimmune cirrhotic portal hypertension and collateral branches of the hepatic vein. Hepatol Res 2023; 53:1084-1095. [PMID: 37353943 DOI: 10.1111/hepr.13939] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/08/2023] [Revised: 06/13/2023] [Accepted: 06/15/2023] [Indexed: 06/25/2023]
Abstract
AIM To assess the correlation and agreement between hepatic venous pressure gradient (HVPG) and portal pressure gradient (PPG) in patients with autoimmune liver diseases (ALD) and portal hypertension, and to investigate the extent to which hepatic vein collateralization affects the accuracy of this assessment. METHODS Ninety-eight patients with ALD between 2017 and 2021 who underwent transjugular intrahepatic portosystemic shunt with conventional and innovative 15 mL pressurized contrast were selected to measure wedged hepatic venous pressure (WHVP) and portal venous pressure and to calculate the HVPG and PPG. Pearson's correlation was used for correlation analysis between the two groups. Bland-Altman plots were plotted to estimate the agreement between paired pressures. RESULTS The r values of PPG and HVPG in the early, middle, late, and portal venous visualization were 0.404, 0.789, 0.807, and 0.830, respectively, and the R2 values were 0.163, 0.622, 0.651, and 0.690, respectively. The p value for the r and R2 values in the early group was 0.015, and the p values in the remaining groups were less than 0.001. Bland-Altman plots showed that patients in the portal venous visualization group had the narrowest 95% limits of agreement. The mean value of the difference was close to the zero-scale line. CONCLUSIONS In patients with ALD, the correlation between the HVPG and PPG was good, and the later the collateral development, the better the correlation. Hepatic vein collateral was an essential factor in underestimating WHVP and HVPG, and the earlier the collateral appeared, the more obvious the underestimation.
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Affiliation(s)
- Bowen Liu
- Department of Interventional Therapy, Beijing Shijitan Hospital, Capital Medical University, Beijing, China
| | - Dan Zhang
- Department of Interventional Therapy, Beijing Shijitan Hospital, Capital Medical University, Beijing, China
| | - Chengbin Dong
- Department of Interventional Therapy, Beijing Shijitan Hospital, Capital Medical University, Beijing, China
| | - Zhendong Yue
- Department of Interventional Therapy, Beijing Shijitan Hospital, Capital Medical University, Beijing, China
| | - Lei Wang
- Department of Interventional Therapy, Beijing Shijitan Hospital, Capital Medical University, Beijing, China
| | - Zhenhua Fan
- Department of Interventional Therapy, Beijing Shijitan Hospital, Capital Medical University, Beijing, China
| | - Yifan Wu
- Department of Interventional Therapy, Beijing Shijitan Hospital, Capital Medical University, Beijing, China
| | - Ke Zhang
- Department of General Surgery, Beijing Ditan Hospital, Capital Medical University, Beijing, China
| | - Li Jiang
- Department of General Surgery, Beijing Ditan Hospital, Capital Medical University, Beijing, China
| | - Huiguo Ding
- Department of Gastroenterology, Beijing YouAn Hospital, Capital Medical University, Beijing, China
| | - Yuening Zhang
- Department of Gastroenterology, Beijing YouAn Hospital, Capital Medical University, Beijing, China
| | - Jian Wang
- Department of Interventional Radiology and Vascular Surgery, Peking University First Hospital, Peking University, Beijing, China
| | - Fuquan Liu
- Department of Interventional Therapy, Beijing Shijitan Hospital, Capital Medical University, Beijing, China
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20
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Zhang G, Ma L, Fu L, Li M, He F, Feng L, Wang M, Jia J, Wang Y, Zhao X. Diagnostic performance of transient elastography in differentiation between porto-sinusoidal vascular liver disease and compensated cirrhosis. Liver Int 2023; 43:2513-2522. [PMID: 37614162 DOI: 10.1111/liv.15709] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/10/2023] [Revised: 07/11/2023] [Accepted: 08/10/2023] [Indexed: 08/25/2023]
Abstract
BACKGROUND AND AIMS The efficacy of transient elastography (TE) in the differential diagnosis between porto-sinusoidal vascular disease (PSVD) and compensated cirrhosis has not been sufficiently studied. We aimed to investigate the diagnostic performance of TE and identify histological lesions associated with liver stiffness. METHODS We conducted a retrospective cohort study including patients with PSVD and cirrhosis (Child-Turcotte-Pugh class A) and healthy subjects. Both the PSVD and cirrhotic patients had at least one sign of PH. The area under the receiver operating characteristic curve (AUROC) was used for differentiation. RESULTS Ninety-two patients with PSVD (median age: 53 years, 33% male), 100 patients with compensated cirrhosis and 101 healthy subjects were included. The median TE-LSM in the PSVD patients (10.0 [7.0-13.0] kPa) was significantly lower than that in the cirrhotic patients (21.0 [15.0-28.0] kPa, p < .001) but was significantly higher than that in the healthy subjects (5.1 [4.6-6.0] kPa, p < .001). The AUROCs of TE-LSM for the discrimination of PSVD from the cirrhosis and healthy subjects were 0.886 (95% CI: 0.833-0.928) and 0.913 (95% CI: 0.864-0.949), respectively. The sensitivity and specificity to discriminate PSVD from compensated cirrhosis were 78.3% and 82.0%, respectively, at a cut-off of 13.6 kPa. Furthermore, portal fibrosis and aberrant cytokeratin 7 expression of centrilobular hepatocytes were significantly associated with higher TE-LSM (≥10.0 kPa). CONCLUSION TE-LSM can be used to differentiate PSVD from compensated cirrhosis. Pathological features in association with increased liver stiffness are identified.
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Affiliation(s)
- Guanhua Zhang
- Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Lin Ma
- Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Li Fu
- International Medical Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Min Li
- Clinical Epidemiology and Evidence-Based Medicine Unit, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Fuliang He
- Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Lijuan Feng
- Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Min Wang
- Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Jidong Jia
- Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Yu Wang
- Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Xinyan Zhao
- Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
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21
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Tan HK, Tan AB, Teh KKJ, Gogna A, Too CW, Leong S, Chang JPE. Impact of catheter tip to hepatic vein ostium distance on the validity and prognostication of hepatic venous pressure gradient in cirrhosis. Sci Rep 2023; 13:16980. [PMID: 37813906 PMCID: PMC10562361 DOI: 10.1038/s41598-023-44016-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/15/2023] [Accepted: 10/03/2023] [Indexed: 10/11/2023] Open
Abstract
Hepatic venous pressure gradient (HVPG) is an accurate measure of portal hypertension in cirrhosis. However, the effect of catheter tip distance from hepatic vein ostium (HVO) on HVPG is unknown. We performed a retrospective study on 228 patients with 307 HVPGs in our institution. The objectives of this study were to assess the effect of catheter position on the validity of HVPG and its prognostication in cirrhosis. In this study, free hepatic vein pressure (FHVP) was considered optimal when difference between FHVP and inferior vena cava pressure was ≤ 2 mmHg. HVPG progressively decreased (p < 0.001) when measured at increasing distance from HVO due to an increasing FHVP (p = 0.036) but an unchanged wedged hepatic vein pressure (p = 0.343). Catheter tip distance > 5 to ≤ 8 cm [odds ratio {OR} 0.16 (95% CI 0.05-0.47), p = 0.001] and > 8 cm [OR 0.14 (95% CI 0.04-0.47), p = 0.002] compared to ≤ 3 cm from HVO were independent predictors of not achieving optimal FHVP. Baseline HVPG ≥ 16 mmHg was strongly associated with deaths due to cirrhosis and liver transplantation for end-stage liver disease compared to HVPG < 16 mmHg when FHVP was optimal (p < 0.001) but not when it was suboptimal (p = 0.359). Our study showed that FHVP is spuriously elevated when measured at > 5 cm from HVO, resulting in inaccurately low HVPG.
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Affiliation(s)
- Hiang Keat Tan
- Department of Gastroenterology and Hepatology, Singapore General Hospital, Singapore, Singapore.
| | - Alfred Bingchao Tan
- Department of Vascular and Interventional Radiology, Singapore General Hospital, Singapore, Singapore
| | - Kevin Kim Jun Teh
- Department of Gastroenterology and Hepatology, Singapore General Hospital, Singapore, Singapore
| | - Apoorva Gogna
- Department of Vascular and Interventional Radiology, Singapore General Hospital, Singapore, Singapore
| | - Chow Wei Too
- Department of Vascular and Interventional Radiology, Singapore General Hospital, Singapore, Singapore
| | - Sum Leong
- Department of Vascular and Interventional Radiology, Singapore General Hospital, Singapore, Singapore
| | - Jason Pik Eu Chang
- Department of Gastroenterology and Hepatology, Singapore General Hospital, Singapore, Singapore
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22
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Li JQ, Feng JY, Gong Y, Li WQ, Liu T. Case report: Novel DGUOK variants associated with idiopathic non-cirrhotic portal hypertension in a Han Chinese child. Front Pediatr 2023; 11:1236239. [PMID: 37830057 PMCID: PMC10565027 DOI: 10.3389/fped.2023.1236239] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/07/2023] [Accepted: 09/11/2023] [Indexed: 10/14/2023] Open
Abstract
DGUOK deficiency has primarily been associated with lethal hepatic failure with or without hypotonia, nystagmus, and psychomotor retardation, features typical of mitochondrial disease. A study in 3 Turkish children identified homozygosity for a variant in DGUOK as associated with idiopathic non-cirrhotic portal hypertension (INCPH). However, no further instances of INCPH associated with DGUOK variants have been reported. We here describe a fourth patient with DGUOK variants and childhood-onset INCPH, a 12-year-old Han Chinese boy, reporting clinical manifestations, histopathologic findings, and results of genetic studies. The child presented with hepatosplenomegaly; portal hypertension and hypersplenism were found. Vascular changes with hepatic fibrosis (Scheuer score 3) were observed on liver biopsy. Whole-exome sequencing and family analyses revealed compound heterozygosity for the DGUOK (NM_080916.3) variants c.778_781dup, (p.Thr261Serfs*28) and c.831_832del, (p.*278Thrfs*9) in the proband. These observations support ascription of instances of INCPH in children to variation in DGUOK.
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Affiliation(s)
- Jia-Qi Li
- The Center for Pediatric Liver Diseases, Children’s Hospital of Fudan University, Shanghai, China
| | - Jia-Yan Feng
- Department of Pathology, Children’s Hospital of Fudan University, Shanghai, China
| | - Ying Gong
- Department of Radiology, Children’s Hospital of Fudan University, Shanghai, China
| | - Wang-Qiang Li
- Department of Infectious Diseases, Anhui Provincial Children’s Hospital, Hefei, China
| | - Teng Liu
- The Center for Pediatric Liver Diseases, Children’s Hospital of Fudan University, Shanghai, China
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23
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Ye X, Quan X, Guo X, Wang Z, Wu H. Idiopathic non-cirrhotic portal hypertension in a patient with Talaromyces marneffei infection: a case report. BMC Infect Dis 2023; 23:125. [PMID: 36859274 PMCID: PMC9979503 DOI: 10.1186/s12879-023-08090-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2022] [Accepted: 02/16/2023] [Indexed: 03/03/2023] Open
Abstract
BACKGROUND The etiopathogenesis of idiopathic non-cirrhotic portal hypertension (INCPH) is so far poorly understood. Altered immunity, blood diseases, infections, congenital defects and drug exposure have been documented in a part of patients with INCPH owing to increased recognition of the disorder in patients with HIV, or various haematological disorders or autoimmune diseases. We aim to discuss the possible etiopathogenesis of INCPH. CASE PRESENTATION We reported that a patient with intestinal infection of T. Marneffei and hyper-IgE syndrome, a group of rare primary immunodeficiency disorders, was finally diagnosed with INCPH for gastroesophageal variceal bleeding. The diagnosis was mainly based on histopathological features. Transjugular intrahepatic portosystemic shunt was performed and there was no recurrence of melena during the six-month follow-up. CONCLUSION In the context of immunodeficiency, INCPH may associated with intestinal infections. Thus, screening for enterogenic infection and immunological disorders in patients with unexplained portal hypertension is necessary.
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Affiliation(s)
- Xiuling Ye
- grid.13291.380000 0001 0807 1581Department of Gastroenterology, West China Hospital, Sichuan University, No. 37, Guo Xue Xiang, Chengdu, 610041 People’s Republic of China
| | - Xin Quan
- grid.13291.380000 0001 0807 1581Department of Gastroenterology, West China Hospital, Sichuan University, No. 37, Guo Xue Xiang, Chengdu, 610041 People’s Republic of China
| | - Xu Guo
- grid.13291.380000 0001 0807 1581Department of Gastroenterology, West China Hospital, Sichuan University, No. 37, Guo Xue Xiang, Chengdu, 610041 People’s Republic of China
| | - Zhidong Wang
- grid.13291.380000 0001 0807 1581Department of Gastroenterology, West China Hospital, Sichuan University, No. 37, Guo Xue Xiang, Chengdu, 610041 People’s Republic of China
| | - Hao Wu
- Department of Gastroenterology, West China Hospital, Sichuan University, No. 37, Guo Xue Xiang, Chengdu, 610041, People's Republic of China.
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24
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Santopaolo F, Ponziani FR, Contegiacomo A, Pompili M, Gasbarrini A, Larghi A. Direct portal pressure gradient measurement in patients with porto-sinusoidal vascular disease. Dig Liver Dis 2023; 55:144-145. [PMID: 36376232 DOI: 10.1016/j.dld.2022.10.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/13/2022] [Accepted: 10/19/2022] [Indexed: 11/13/2022]
Affiliation(s)
- Francesco Santopaolo
- Internal Medicine and Gastroenterology, Hepatology Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy.
| | - Francesca Romana Ponziani
- Internal Medicine and Gastroenterology, Hepatology Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy
| | - Andrea Contegiacomo
- Department of Bioimaging, Institute of Radiology, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy
| | - Maurizio Pompili
- Internal Medicine and Gastroenterology, Hepatology Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy
| | - Antonio Gasbarrini
- Internal Medicine and Gastroenterology, Hepatology Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy
| | - Alberto Larghi
- Digestive Endoscopy Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italy
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25
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Jin SJ, Choi WM. Porto-Sinusoidal Vascular Disease: A Concise Updated Summary of Epidemiology, Pathophysiology, Imaging, Clinical Features, and Treatments. Korean J Radiol 2023; 24:31-38. [PMID: 36606618 PMCID: PMC9830138 DOI: 10.3348/kjr.2022.0668] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2022] [Revised: 10/06/2022] [Accepted: 10/26/2022] [Indexed: 01/03/2023] Open
Affiliation(s)
- Su Jin Jin
- Department of Gastroenterology, Liver Center, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Won-Mook Choi
- Department of Gastroenterology, Liver Center, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
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26
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Ferreira-Silva J, Gaspar R, Liberal R, Cardoso H, Macedo G. Splenic-hepatic elastography index is useful in differentiating between porto-sinusoidal vascular disease and cirrhosis in patients with portal hypertension. Dig Liver Dis 2023; 55:75-80. [PMID: 36280435 DOI: 10.1016/j.dld.2022.09.018] [Citation(s) in RCA: 10] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/12/2022] [Revised: 09/18/2022] [Accepted: 09/30/2022] [Indexed: 11/06/2022]
Abstract
INTRODUCTION In patients with portal hypertension (PH), the differential diagnosis between porto-sinusoidal vascular disease (PSVD) and cirrhosis is challenging. This study aims to evaluate the diagnostic accuracy of the SSM/LSM index in the diagnosis of PSVD. METHODS Prospective study of patients with PH and PSVD or cirrhosis. Transient liver and spleen elastography were performed and the ratio between spleen stiffness measurement (SSM) and liver stiffness measurement (LSM) was calculated. The relation of SSM/LSM with the diagnosis of PSVD was evaluated. RESULTS Forty-four patients with PSVD and 44 patients with cirrhosis were evaluated. Median age was 57.5 (IQR 49.0-64.5) years, 66.3% were males. In patients with PSVD, median SSM was 59.4 (33.5-77.7) kPa, median LSM was 6.2 (5.2-10.2) kPa and median SSM/LSM was 5.62 (3.15-9.68). In patients with cirrhosis, median SSM was 47.3 (24.3-60.3) kPa, median LSM was 27.8 (17.7-53.9) kPa and median SSM/LSM was 1.55 (1.06-3.24). The SSM/LSM AUROC was 0.940 (p<0.001). Using 2 as a cut-off, we obtained good sensitivity (86.5%), specificity (92.7%), and accuracy (89.7%) for the diagnosis of PSVD. CONCLUSION The SSM/LSM index is useful in the differential diagnosis between liver cirrhosis and PSVD. Using the cut-off of 2 we achieved a good sensitivity and specificity for diagnosing PSVD.
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Affiliation(s)
- Joel Ferreira-Silva
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal. Alameda Prof. Hernâni Monteiro 4200 - 319 Porto, Portugal; Faculty of Medicine of the University of Porto, Porto, Portugal. Alameda Prof. Hernâni Monteiro 4200 - 319 Porto, Portugal.
| | - Rui Gaspar
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal. Alameda Prof. Hernâni Monteiro 4200 - 319 Porto, Portugal; Faculty of Medicine of the University of Porto, Porto, Portugal. Alameda Prof. Hernâni Monteiro 4200 - 319 Porto, Portugal
| | - Rodrigo Liberal
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal. Alameda Prof. Hernâni Monteiro 4200 - 319 Porto, Portugal; Faculty of Medicine of the University of Porto, Porto, Portugal. Alameda Prof. Hernâni Monteiro 4200 - 319 Porto, Portugal
| | - Hélder Cardoso
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal. Alameda Prof. Hernâni Monteiro 4200 - 319 Porto, Portugal; Faculty of Medicine of the University of Porto, Porto, Portugal. Alameda Prof. Hernâni Monteiro 4200 - 319 Porto, Portugal
| | - Guilherme Macedo
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal. Alameda Prof. Hernâni Monteiro 4200 - 319 Porto, Portugal; Faculty of Medicine of the University of Porto, Porto, Portugal. Alameda Prof. Hernâni Monteiro 4200 - 319 Porto, Portugal
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27
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Lampichler K, Semmler G, Wöran K, Simbrunner B, Jachs M, Hartl L, Bauer DJM, Balcar L, Burghart L, Trauner M, Tamandl D, Ba-Ssalamah A, Mandorfer M, Reiberger T, Scheiner B, Scharitzer M. Imaging features facilitate diagnosis of porto-sinusoidal vascular disorder. Eur Radiol 2023; 33:1422-1432. [PMID: 36166087 PMCID: PMC9889423 DOI: 10.1007/s00330-022-09132-4] [Citation(s) in RCA: 13] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2022] [Revised: 08/06/2022] [Accepted: 08/29/2022] [Indexed: 02/04/2023]
Abstract
OBJECTIVES Porto-sinusoidal vascular disorder (PSVD) is a recently defined vascular liver disease. Since diagnosis remains challenging, we aimed to evaluate radiological features that are distinct between PSVD and cirrhosis. METHODS Clinical, laboratory, and radiological parameters (CT/MRI) of patients with histologically-confirmed PSVD vs. cirrhosis vs. non-cirrhotic parenchymal liver disease were retrospectively evaluated. RESULTS Sixty-three PSVD, 155 cirrhosis, and 41 non-cirrhotic patients were included. As compared to cirrhosis, PSVD patients were younger and had lower HVPG, liver stiffness, and MELD. Routine clinical and imaging findings indicative of portal hypertension were similarly common. Intrahepatic portal tract abnormalities (49% vs. 15%; p < 0.001), FNH-like lesions (30% vs. 1%; p < 0.001), and abnormal liver morphology defined as peripheral parenchymal atrophy and compensatory hypertrophy of central segments (32% vs. 7%; p < 0.001) were significantly more common in PSVD patients. Hypertrophy of segment I (70% vs. 84%; p = 0.019), atrophy of segment IV (24% vs. 47%; p = 0.001), and nodular liver surface (22% vs. 89%; p < 0.001) were more common in patients with cirrhosis. In patients with gadoxetic acid-enhanced MRI, we identified the distinct imaging feature of "periportal hyperintensity" in the hepatobiliary phase (HBP) in 42% of patients with PSVD (14/33) vs. 1% in cirrhosis (1/95) vs. 0% in non-cirrhotic controls (0/41); p < 0.001). CONCLUSIONS Diagnosis of PSVD must be considered in younger patients presenting with clinical features of portal hypertension, portal tract abnormalities, and FNH-like lesions on CT/MRI. 'Periportal hyperintensity' in the HBP of gadoxetic acid-enhanced MRI was identified as a specific radiological feature of PSVD. KEY POINTS • Cross-sectional imaging can provide essential information to identify patients with porto-sinusoidal vascular disorder (PSVD). • Intrahepatic portal tract abnormalities, FNH-like lesions, and abnormal liver morphology are common in PSVD patients. • Periportal hyperintensity on the hepatobiliary phase of gadoxetic acid-enhanced MRI seems to be specific for patients with PSVD.
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Affiliation(s)
- Katharina Lampichler
- Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria
| | - Georg Semmler
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Katharina Wöran
- Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria
| | - Benedikt Simbrunner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria
- Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria
- Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria
- CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
| | - Mathias Jachs
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Lukas Hartl
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - David Josef Maria Bauer
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Lorenz Balcar
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Lukas Burghart
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Michael Trauner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Dietmar Tamandl
- Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria
| | - Ahmed Ba-Ssalamah
- Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria
| | - Mattias Mandorfer
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Thomas Reiberger
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria.
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria.
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria.
- Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria.
- Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
- CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
| | - Bernhard Scheiner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
- Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Martina Scharitzer
- Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria
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28
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Issa Z, Gohy S, Zech F, Baldin P, Delire B, Dahlqvist G. Prevalence and characteristics of cystic fibrosis liver disease: a study highlighting the lack of histological diagnosis. Clin Res Hepatol Gastroenterol 2022; 46:101977. [PMID: 35772685 DOI: 10.1016/j.clinre.2022.101977] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/09/2021] [Revised: 04/24/2022] [Accepted: 06/08/2022] [Indexed: 02/04/2023]
Abstract
BACKGROUND AND AIMS Cystic fibrosis liver disease (CFLD) is the third leading cause of death in patients with cystic fibrosis (CF). We aim to determine the prevalence of CFLD in a cohort of adult patients with CF and to characterise liver involvement in this population highlighting the importance of histological diagnosis. METHODS We retrospectively studied a cohort of patients with CF. Inclusion criteria were age ≥ 18 and minimum 1 year of follow-up. We excluded lung transplant patients. CFLD was defined as having 2 out of 3 criteria: persistent elevation of transaminases and/or gamma-glutamyltransferase; abnormal ultrasound; and abnormal transient elastography. Non-invasive fibrosis biomarkers were calculated in CFLD patients. Adult-onset CFLD (Ad-CFLD) was defined as CFLD ≥18 years. Severe CFLD (s-CFLD) was defined as CFLD with cirrhosis and/or portal hypertension. RESULTS We included 113 patients. Median age was 29 years, 58 were male. Forty patients had CFLD. Median age at CFLD diagnosis was 10 years. Twenty-one patients had s-CFLD. Two s-CFLD patients had nodular regenerative hyperplasia, 1 had hepatocellular carcinoma and 4 underwent liver transplantation. Six patients had ad-CFLD. Both CFLD and s-CFLD groups were compared to a non-CFLD group. The CFLD group had significantly more males (p = 0.034). S-CFLD group had worse pulmonary function (p = 0.015). CONCLUSION Thirty five percent of adult patients with CF, mainly males, had CFLD. Nineteen percent had s-CFLD and had worse pulmonary function. With recent reports unravelling different pathophysiological mechanisms in CFLD, we believe it is important to better characterise liver involvement using liver biopsy.
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Affiliation(s)
- Zaina Issa
- Department of Gastroenterology, Cliniques Universitaires Saint-Luc, Brussels, Belgium.
| | - Sophie Gohy
- Department of Pneumology, Cliniques Universitaires Saint-Luc, Brussels, Belgium; Cystic Fibrosis Reference Center, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Francis Zech
- Department of Infectious Diseases, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Pamela Baldin
- Department of Anatomopathology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Bénédicte Delire
- Department of Gastroenterology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Géraldine Dahlqvist
- Department of Gastroenterology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
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29
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De Gottardi A, Sempoux C, Berzigotti A. Porto-sinusoidal vascular disorder. J Hepatol 2022; 77:1124-1135. [PMID: 35690264 DOI: 10.1016/j.jhep.2022.05.033] [Citation(s) in RCA: 75] [Impact Index Per Article: 25.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/06/2022] [Revised: 05/15/2022] [Accepted: 05/23/2022] [Indexed: 12/04/2022]
Abstract
It is well established that portal hypertension can occur in the absence of cirrhosis, as reported in patients with immune disorders, infections and thrombophilia. However, similar histological abnormalities primarily affecting the hepatic sinusoidal and (peri)portal vasculature have also been observed in patients without portal hypertension. Thus, the term porto-sinusoidal vascular disorder (PSVD) has recently been introduced to describe a group of vascular diseases of the liver featuring lesions encompassing the portal venules and sinusoids, irrespective of the presence/absence of portal hypertension. Liver biopsy is fundamental for PSVD diagnosis. Specific histology findings include nodular regenerative hyperplasia, obliterative portal venopathy/portal vein stenosis and incomplete septal fibrosis/cirrhosis. Since other conditions including alcohol-related and non-alcoholic fatty liver disease, or viral hepatitis, or the presence of portal vein thrombosis may occur in patients with PSVD, their relative contribution to liver damage should be carefully assessed. In addition to histology and clinical diagnostic criteria, imaging and non-invasive tests such as liver and spleen stiffness measurements could aid in the diagnostic workup. The introduction of PSVD as a novel clinical entity will facilitate collaborative studies and investigations into the underlying molecular pathomechanisms encompassed by this term.
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Affiliation(s)
- Andrea De Gottardi
- Gastroenterology and Hepatology, Ente Ospedaliero Cantonale, Lugano, Switzerland; Faculty of Biomedical Sciences, Università della Svizzera Italiana, Lugano, Switzerland.
| | - Christine Sempoux
- Service of Clinical Pathology, Institute of Pathology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Annalisa Berzigotti
- Department for Visceral Medicine and Surgery, Inselspital, Bern University Hospital, University of Bern, Switzerland; Department of Biomedical Research, University of Bern, Bern, Switzerland
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30
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Jindal A. Letter to the editor: Non-invasive diagnosis of porto-sinusoidal vascular disease. Hepatology 2022; 76:E54. [PMID: 35340040 DOI: 10.1002/hep.32480] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/01/2022] [Accepted: 02/01/2022] [Indexed: 12/08/2022]
Affiliation(s)
- Ankur Jindal
- Department of Hepatology, Institute of Liver & Biliary Sciences, New Delhi, India
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31
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Tan MJ, Liu H, Ding HG. Pathological and imaging features of idiopathic non-cirrhotic portal hypertension. Shijie Huaren Xiaohua Zazhi 2022; 30:729-734. [DOI: 10.11569/wcjd.v30.i16.729] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
Idiopathic non-cirrhotic portal hypertension (INCPH), a kind of portal sinus vascular disease with unknown etiology, is characterized by the presence of clinical signs and symptoms of portal hypertension (PH) in the absence of liver cirrhosis or known risk factors accountable for PH. It has an extremely high rate of initial misdiagnosis and underdiagnosis. Liver biopsy is the only way to make a definitive diagnosis. Non-invasive modalities, such as CT imaging, are becoming a hot topic of interest in recent years. This article summarizes the pathological and CT/MRI features of INCPH and the key points of differentiation from cirrhosis, to improve clinicians' understanding of INCPH and reduce the rate of initial misdiagnosis and missed diagnoses.
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Affiliation(s)
- Ming-Jie Tan
- Department of Gastrointestinal and Hepatology, Beijing You'An Hospital, Capital Medical University, Beijing 100069, China
| | - Hui Liu
- Department of Pathology, Beijing You'An Hospital, Capital Medical University, Beijing 100069, China
| | - Hui-Guo Ding
- Department of Gastrointestinal and Hepatology, Beijing You'An Hospital, Capital Medical University, Beijing 100069, China
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32
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Valainathan SR, Sartoris R, Elkrief L, Magaz M, Betancourt F, Pellegrino S, Nivolli A, Dioguardi Burgio M, Flattet Y, Terraz S, Drilhon N, Lazareth M, Herrou J, Bruno O, Payance A, Plessier A, Durand F, Ronot M, Valla D, Paradis V, Garcia‐Pagan JC, Vilgrain V, Rautou P. Contrast-enhanced CT and liver surface nodularity for the diagnosis of porto-sinusoidal vascular disorder: A case-control study. Hepatology 2022; 76:418-428. [PMID: 35092315 PMCID: PMC9544289 DOI: 10.1002/hep.32367] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/02/2021] [Revised: 01/10/2022] [Accepted: 01/21/2022] [Indexed: 12/25/2022]
Abstract
BACKGROUND AND AIMS Porto-sinusoidal vascular disorder (PSVD) is a rare and commonly overlooked cause of portal hypertension. The interest of CT analysis, including quantification of liver surface nodularity (LSN) for PSVD diagnosis has not been established. This study aimed at assessing the performance of LSN and CT features for a PSVD diagnosis in patients with signs of portal hypertension. APPROACH AND RESULTS This retrospective case-control study included a learning cohort consisting of 50 patients with histologically proven PSVD, according to VALDIG criteria, and 100 control patients with histologically proven cirrhosis, matched on ascites. All patients and controls had at least one sign of portal hypertension and CT available within 1 year of liver biopsy. Principal component analysis of CT features separated patients with PSVD from patients with cirrhosis. Patients with PSVD had lower median LSN than those with cirrhosis (2.4 vs. 3.1, p < 0.001). Multivariate analysis identified LSN < 2.5 and normal-sized or enlarged segment IV as independently associated with PSVD. Combination of these two features had a specificity of 90% for PSVD and a diagnostic accuracy of 84%. Even better results were obtained in an independent multicenter validation cohort including 53 patients with PSVD and 106 control patients with cirrhosis (specificity 94%, diagnostic accuracy 87%). CONCLUSIONS This study that included a total of 103 patients with PSVD and 206 patients with cirrhosis demonstrates that LSN < 2.5 combined with normal-sized or enlarged segment IV strongly suggests PSVD in patients with signs of portal hypertension.
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Affiliation(s)
- Shantha Ram Valainathan
- Service d'HépatologieDMU DIGESTCentre de Référence des Maladies Vasculaires du FoieFILFOIEERN RARE‐LIVERCentre de Recherche sur l’inflammationInsermUMR 1149Université de ParisAP‐HPHôpital BeaujonParisFrance
| | - Riccardo Sartoris
- Centre de Recherche sur l’inflammationInsermUMR 1149Université de ParisParisFrance,Department of RadiologyAP‐HP NordHôpital BeaujonClichyFrance
| | - Laure Elkrief
- Service d’Hépato‐gastroentérologieHôpitaux Universitaires de GenèveGenevaSwitzerland,Service d’Hépato‐GastroentérologieHôpital TrousseauCHRU de ToursToursFrance
| | - Marta Magaz
- Barcelona Hepatic Hemodynamic LaboratoryLiver UnitHospital Clínic de BarcelonaIDIBAPSCIBERehdEuropean Reference Network for Rare Vascular Liver DiseasesUniversitat de BarcelonaBarcelonaSpain
| | - Fabian Betancourt
- Barcelona Hepatic Hemodynamic LaboratoryLiver UnitHospital Clínic de BarcelonaIDIBAPSCIBERehdEuropean Reference Network for Rare Vascular Liver DiseasesUniversitat de BarcelonaBarcelonaSpain
| | - Silvia Pellegrino
- Centre de Recherche sur l’inflammationInsermUMR 1149Université de ParisParisFrance,Department of RadiologyAP‐HP NordHôpital BeaujonClichyFrance
| | - Arianna Nivolli
- Centre de Recherche sur l’inflammationInsermUMR 1149Université de ParisParisFrance,Department of RadiologyAP‐HP NordHôpital BeaujonClichyFrance
| | - Marco Dioguardi Burgio
- Centre de Recherche sur l’inflammationInsermUMR 1149Université de ParisParisFrance,Department of RadiologyAP‐HP NordHôpital BeaujonClichyFrance
| | - Yves Flattet
- Service d’Hépato‐gastroentérologieHôpitaux Universitaires de GenèveGenevaSwitzerland
| | - Sylvain Terraz
- Department of RadiologyUniversity Hospitals of GenevaGenevaSwitzerland
| | - Nicolas Drilhon
- Service d'HépatologieDMU DIGESTCentre de Référence des Maladies Vasculaires du FoieFILFOIEERN RARE‐LIVERCentre de Recherche sur l’inflammationInsermUMR 1149Université de ParisAP‐HPHôpital BeaujonParisFrance
| | - Marie Lazareth
- Service d'HépatologieDMU DIGESTCentre de Référence des Maladies Vasculaires du FoieFILFOIEERN RARE‐LIVERCentre de Recherche sur l’inflammationInsermUMR 1149Université de ParisAP‐HPHôpital BeaujonParisFrance
| | - Julia Herrou
- Department of RhumatologyHôpital CochinAssistance Publique‐Hôpitaux de ParisParisFrance
| | - Onorina Bruno
- Centre de Recherche sur l’inflammationInsermUMR 1149Université de ParisParisFrance,Department of RadiologyAP‐HP NordHôpital BeaujonClichyFrance
| | - Audrey Payance
- Service d'HépatologieDMU DIGESTCentre de Référence des Maladies Vasculaires du FoieFILFOIEERN RARE‐LIVERCentre de Recherche sur l’inflammationInsermUMR 1149Université de ParisAP‐HPHôpital BeaujonParisFrance
| | - Aurélie Plessier
- Service d'HépatologieDMU DIGESTCentre de Référence des Maladies Vasculaires du FoieFILFOIEERN RARE‐LIVERCentre de Recherche sur l’inflammationInsermUMR 1149Université de ParisAP‐HPHôpital BeaujonParisFrance
| | - François Durand
- Service d'HépatologieDMU DIGESTCentre de Référence des Maladies Vasculaires du FoieFILFOIEERN RARE‐LIVERCentre de Recherche sur l’inflammationInsermUMR 1149Université de ParisAP‐HPHôpital BeaujonParisFrance
| | - Maxime Ronot
- Centre de Recherche sur l’inflammationInsermUMR 1149Université de ParisParisFrance,Department of RadiologyAP‐HP NordHôpital BeaujonClichyFrance
| | - Dominique‐Charles Valla
- Service d'HépatologieDMU DIGESTCentre de Référence des Maladies Vasculaires du FoieFILFOIEERN RARE‐LIVERCentre de Recherche sur l’inflammationInsermUMR 1149Université de ParisAP‐HPHôpital BeaujonParisFrance
| | - Valérie Paradis
- Department of PathologyUniversité de ParisAP‐HP, Hôpital BeaujonBeaujon HospitalAssistance Publique‐Hôpitaux de ParisClichyFrance
| | - Juan Carlos Garcia‐Pagan
- Barcelona Hepatic Hemodynamic LaboratoryLiver UnitHospital Clínic de BarcelonaIDIBAPSCIBERehdEuropean Reference Network for Rare Vascular Liver DiseasesUniversitat de BarcelonaBarcelonaSpain
| | - Valérie Vilgrain
- Centre de Recherche sur l’inflammationInsermUMR 1149Université de ParisParisFrance,Department of RadiologyAP‐HP NordHôpital BeaujonClichyFrance
| | - Pierre‐Emmanuel Rautou
- Service d'HépatologieDMU DIGESTCentre de Référence des Maladies Vasculaires du FoieFILFOIEERN RARE‐LIVERCentre de Recherche sur l’inflammationInsermUMR 1149Université de ParisAP‐HPHôpital BeaujonParisFrance
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Wöran K, Semmler G, Jachs M, Simbrunner B, Bauer DJM, Binter T, Pomej K, Stättermayer AF, Schwabl P, Bucsics T, Paternostro R, Lampichler K, Pinter M, Trauner M, Mandorfer M, Stift J, Reiberger T, Scheiner B. Clinical Course of Porto-Sinusoidal Vascular Disease Is Distinct From Idiopathic Noncirrhotic Portal Hypertension. Clin Gastroenterol Hepatol 2022; 20:e251-e266. [PMID: 33279774 DOI: 10.1016/j.cgh.2020.11.039] [Citation(s) in RCA: 29] [Impact Index Per Article: 9.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/20/2020] [Revised: 11/08/2020] [Accepted: 11/25/2020] [Indexed: 12/13/2022]
Abstract
BACKGROUND & AIMS Porto-sinusoidal vascular disease (PSVD) was recently proposed as novel clinical entity characterized by typical histological changes with or without portal hypertension (PH) in the absence of cirrhosis. Thus, we aimed to describe clinical characteristics and the outcome of PSVD patients and to compare these to patients meeting traditional idiopathic non-cirrhotic portal hypertension (INCPH) criteria. METHODS Patients undergoing liver biopsy (baseline) ±hepatic venous pressure gradient (HVPG) measurement at the Vienna General Hospital between 2000-2019 were screened for PSVD and INCPH criteria. RESULTS 91 patients were diagnosed with PSVD of which 28 (30.8%) also fulfilled INCPH criteria (INCPH+/PSVD+). Specific histological and specific clinical PH signs were found in 72 (79.1%) and 54 (59.3%) patients, respectively. INCPH+/PSVD+ showed higher Child-Pugh-scores (7±2 vs 6±1 points; P = .002) and a higher prevalence of decompensation (57.1% vs 28.6%; P = .009) than INCPH-/PSVD+ patients. Importantly, hepatic decompensation after three years (3Y) occurred in 11.2% of PSVD patients with specific clinical signs of PH, while no decompensation occurred in patients with only specific histological or with unspecific clinical/histological signs (P = .002). When categorizing by INCPH definition, 3Y decompensation was 13.4% in INCPH+/PSVD+ and 3.8% in INCPH-/PSVD+ (P = .120). While overall mortality was similar in INCPH+/PSVD+ (n = 6; 21.4%) and INCPH-/PSVD+ (n = 10; 15.9%) patients (P = .558), liver-related mortality tended to be higher in INCPH+/PSVD+ (6.9%) than in INCPH-/PSVD+ (0%; P = .078). CONCLUSION Novel PSVD criteria facilitate diagnosis. Compared to INCPH, clinical course of PSVD patients is more favorable. Importantly, specific signs of PH including varices and collaterals are associated with hepatic decompensation and mortality.
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Affiliation(s)
- Katharina Wöran
- Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria
| | - Georg Semmler
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network RARE-LIVER, Medical University of Vienna, Vienna, Austria; Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria
| | - Mathias Jachs
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network RARE-LIVER, Medical University of Vienna, Vienna, Austria; Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria
| | - Benedikt Simbrunner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network RARE-LIVER, Medical University of Vienna, Vienna, Austria; Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria; CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
| | - David Josef Maria Bauer
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria; Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria
| | - Teresa Binter
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
| | - Katharina Pomej
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
| | - Albert Friedrich Stättermayer
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Philipp Schwabl
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria; Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria
| | - Theresa Bucsics
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
| | - Rafael Paternostro
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
| | - Katharina Lampichler
- Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria
| | - Matthias Pinter
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Michael Trauner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network RARE-LIVER, Medical University of Vienna, Vienna, Austria
| | - Mattias Mandorfer
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network RARE-LIVER, Medical University of Vienna, Vienna, Austria; Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria
| | - Judith Stift
- Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria
| | - Thomas Reiberger
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network RARE-LIVER, Medical University of Vienna, Vienna, Austria; Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria; CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
| | - Bernhard Scheiner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria; Rare Liver Disease Center of the European Reference Network RARE-LIVER, Medical University of Vienna, Vienna, Austria; Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria
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Rautou PE, Elkrief L, Decraecker M, Ollivier-Hourmand I, Plessier A, Ronot M, Vilgrain V, Bourlière M, Ganne-Carrié N, de Lédinghen V, Bureau C. Non-invasive diagnosis and follow-up of vascular liver diseases. Clin Res Hepatol Gastroenterol 2022; 46:101764. [PMID: 34332130 DOI: 10.1016/j.clinre.2021.101764] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/11/2021] [Accepted: 07/23/2021] [Indexed: 02/04/2023]
Abstract
Vascular disorders of the liver are rare diseases, some of which are diagnosed mainly with non-invasive tests and others by liver biopsy. Non-invasive methods can be used to diagnose and monitor these diseases. However, their evaluation needs to be performed by expert centers. Liver biopsy is needed each time there is an unexplained abnormality.
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Affiliation(s)
- Pierre-Emmanuel Rautou
- Service d'hépato-gastroentérologie et d'oncologie digestive, Hôpital Haut-Lévêque, CHU Bordeaux, Pessac & INSERM U1053, Université de Bordeaux, Bordeaux, France.
| | - Laure Elkrief
- Unité d'hépatologie HC - UMUH 1, CHRU Tours, Tours, France
| | - Marie Decraecker
- Service d'hépato-gastroentérologie et d'oncologie digestive, Hôpital Haut-Lévêque, CHU Bordeaux, Pessac & INSERM U1053, Université de Bordeaux, Bordeaux, France
| | | | - Aurélie Plessier
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149 Paris, France
| | - Maxime Ronot
- Service de radiologie, Hôpital Beaujon, Université de Paris, APHP, Clichy, France
| | - Valérie Vilgrain
- Service de radiologie, Hôpital Beaujon, Université de Paris, APHP, Clichy, France
| | - Marc Bourlière
- Service d'hépato-gastroentérologie, Hôpital Saint Joseph & INSERM UMR 1252 IRD SESSTIM, Aix Marseille Université, Marseille, France
| | - Nathalie Ganne-Carrié
- Service d'hépatologie, Hôpital Avicenne, APHP, Université Sorbonne Paris Nord, Bobigny & INSERM UMR 1138, Centre de Recherche des Cordeliers, Université de Paris, France
| | - Victor de Lédinghen
- Service d'hépato-gastroentérologie et d'oncologie digestive, Hôpital Haut-Lévêque, CHU Bordeaux, Pessac & INSERM U1053, Université de Bordeaux, Bordeaux, France
| | - Christophe Bureau
- Service d'hépatologie, Hôpital Rangueil, CHU Toulouse, Toulouse, France
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35
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Nagai K, Ogawa Y, Kobayashi T, Iwaki M, Nogami A, Honda Y, Kessoku T, Saigusa Y, Imajo K, Yoneda M, Kirikoshi H, Komatsu T, Saito S, Nakajima A. Gastroesophageal varices evaluation using spleen‐dedicated stiffness measurement by vibration‐controlled transient elastography. JGH Open 2021; 6:11-19. [PMID: 35071783 PMCID: PMC8762624 DOI: 10.1002/jgh3.12689] [Citation(s) in RCA: 16] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/09/2021] [Revised: 11/22/2021] [Accepted: 11/28/2021] [Indexed: 12/12/2022]
Abstract
Background and Aim Methods Results Conclusions
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Affiliation(s)
- Koki Nagai
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
- Department of Gastroenterology Shin‐yurigaoka General Hospital Kawasaki Japan
| | - Yuji Ogawa
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
- Department of Gastroenterology National Hospital Organization Yokohama Medical Center Yokohama Japan
| | - Takashi Kobayashi
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
| | - Michihiro Iwaki
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
| | - Asako Nogami
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
| | - Yasushi Honda
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
| | - Takaomi Kessoku
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
| | - Yusuke Saigusa
- Department of Biostatistics Yokohama City University School of Medicine Yokohama Japan
| | - Kento Imajo
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
| | - Masato Yoneda
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
| | - Hiroyuki Kirikoshi
- Department of Clinical Laboratory Yokohama City University Hospital Yokohama Japan
| | - Tatsuji Komatsu
- Department of Gastroenterology National Hospital Organization Yokohama Medical Center Yokohama Japan
| | - Satoru Saito
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
| | - Atsushi Nakajima
- Department of Gastroenterology and Hepatology Yokohama City University Graduate School of Medicine Yokohama Japan
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36
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Ferreira-Silva J, Gaspar R, Liberal R, Cardoso H, Macedo G. Transient splenic elastography predicts high-risk esophageal varices in patients with non-cirrhotic portal hypertension. Scand J Gastroenterol 2021; 56:1462-1466. [PMID: 34428123 DOI: 10.1080/00365521.2021.1968485] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
BACKGROUND AND AIMS Non-cirrhotic portal hypertension (NCPH) comprise a group of diseases that cause portal hypertension without cirrhosis, leading to a high risk of hemorrhage from esophageal varices. There are no non-invasive predictors of high-risk varices (HRV) described in the literature for NCPH. This study aimed to evaluate whether transient splenic elastography (TSE) can predict HRV in patients with NCPH. METHODS Prospective study of patients with NCPH who underwent a single timepoint evaluation with transient liver and spleen elastography, ultrasonography, upper endoscopy, and laboratory tests. The study was performed from January to September 2020. Patients were divided into two groups based on the presence of HRV. The relation between TSE, transient liver elastography (TLE), spleen size, and platelet count to the presence of HRV was evaluated. RESULTS Of 42 patients with NCPH, 50% (21/42) presented HRV. In univariate analysis, TSE (median, 58.4 vs. 28.3, p = 0.009) and spleen size (median, 17.5 vs. 14.5 cm, p = 0.013) were associated with HRV. No statistically significant relationship was found between the presence of HRV and platelet count or TLE. In multivariate analysis, TSE was the only variable related to HRV (OR 1.21, 95% CI 1.02-1.38). TSE had a good performance in predicting HRV in our population (AUROC 0.878; 95% CI 0.751-1000). TSE > 35.4 kPa presents 93.3% sensitivity, 60.0% specificity, and 90.9% negative predictive value. CONCLUSION In our population of patients with NCPH, TSE is useful in predicting HRV. TLE, spleen size, and platelet count were not related to HRV.
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Affiliation(s)
- Joel Ferreira-Silva
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal
| | - Rui Gaspar
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal.,Faculty of Medicine, University of Porto, Porto, Portugal
| | - Rodrigo Liberal
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal.,Faculty of Medicine, University of Porto, Porto, Portugal
| | - Hélder Cardoso
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal.,Faculty of Medicine, University of Porto, Porto, Portugal
| | - Guilherme Macedo
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal.,Faculty of Medicine, University of Porto, Porto, Portugal
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37
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Gioia S, Riggio O, Nardelli S, d'Amati G, Ridola L. Identifying Patients at High Risk of Developing Non-Cirrhotic Portal Hypertension. Hepat Med 2021; 13:105-111. [PMID: 34764704 PMCID: PMC8572743 DOI: 10.2147/hmer.s282674] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/04/2021] [Accepted: 10/27/2021] [Indexed: 12/23/2022] Open
Abstract
The term porto-sinusoidal vascular disease (PSVD) has been recently proposed to replace the term idiopathic non-cirrhotic portal hypertension (INCPH) to describe patients with or without signs of portal hypertension and typical histological lesions involving the portal venules or sinusoids in the absence of cirrhosis. According to the new definition, the presence of known causes of liver disease as well as of portal vein thrombosis does not rule out PSVD. Therefore, the patients in whom the diagnosis of PSVD is possible are much more than the patients strictly fulfilling the diagnostic criteria for INCPH. In this setting, the clinical challenge for the hepatologist is to identify patients at risk of developing PSVD and to indicate liver biopsy to confirm the diagnosis. We describe some possible scenarios in which PSVD should always be suspected, and we provide some tools useful to reach the diagnosis of PSVD.
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Affiliation(s)
- Stefania Gioia
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
| | - Oliviero Riggio
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
| | - Silvia Nardelli
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
| | - Giulia d'Amati
- Department of Radiological, Oncological, and Pathological Sciences, Sapienza University of Rome, Rome, Italy
| | - Lorenzo Ridola
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
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38
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Elkrief L, Lazareth M, Chevret S, Paradis V, Magaz M, Blaise L, Rubbia-Brandt L, Moga L, Durand F, Payancé A, Plessier A, Chaffaut C, Valla D, Malphettes M, Diaz A, Nault JC, Nahon P, Audureau E, Ratziu V, Castera L, Garcia Pagan JC, Ganne-Carrie N, Rautou PE. Liver Stiffness by Transient Elastography to Detect Porto-Sinusoidal Vascular Liver Disease With Portal Hypertension. Hepatology 2021; 74:364-378. [PMID: 33345307 DOI: 10.1002/hep.31688] [Citation(s) in RCA: 37] [Impact Index Per Article: 9.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2020] [Revised: 11/19/2020] [Accepted: 12/04/2020] [Indexed: 12/15/2022]
Abstract
BACKGROUND AND AIMS Porto-sinusoidal vascular liver disease (PSVD) is a rare cause of portal hypertension. PSVD is still often misdiagnosed as cirrhosis, emphasizing the need to improve PSVD diagnosis strategies. Data on liver stiffness measurement using transient elastography (TE-LSM) in PSVD are limited. The aim of this study was to evaluate the accuracy of TE-LSM to discriminate PSVD from cirrhosis in patients with signs of portal hypertension. APPROACH AND RESULTS Retrospective multicenter study comparing TE-LSM in patients with PSVD, according to Vascular Liver Disease Interest Group criteria, with patients with compensated biopsy-proven cirrhosis associated with alcohol (n = 117), HCV infection (n = 110), or NAFLD (n = 46). All patients had at least one sign of portal hypertension among gastroesophageal varices, splenomegaly, portosystemic collaterals, history of ascites, or platelet count < 150 × 109 /L. The 77 patients with PSVD included in the test cohort had lower median TE-LSM (7.9 kPa) than the patients with alcohol-associated, HCV-related, and NAFLD-related cirrhosis (33.8, 18.2, and 33.6 kPa, respectively; P < 0.001). When compared with cirrhosis, a cutoff value of 10 kPa had a specificity of 97% for the diagnosis of PSVD with a 85% positive predictive value. A cutoff value of 20 kPa had a sensitivity of 94% for ruling out PSVD with a 97% negative predictive value. Of the patients, 94% were well-classified. Even better results were obtained in a validation cohort including 78 patients with PSVD. CONCLUSIONS This study including a total of 155 patients with PSVD and 273 patients with cirrhosis demonstrates that TE-LSM < 10 kPa strongly suggests PSVD in patients with signs of portal hypertension. Conversely, when TE-LSM is >20 kPa, PSVD is highly unlikely.
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Affiliation(s)
- Laure Elkrief
- Service de Transplantation et Hépato-gastroentérologie, Hôpitaux Universitaires de Genève, Switzerland.,Service d'Hépato-Gastroentérologie, CHU de Tours, France.,Université de Paris, Centre de recherche sur l'inflammation, Inserm, U1149, CNRS, ERL8252, Paris, France
| | - Marie Lazareth
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Sylvie Chevret
- Service de Biostatistiques et Information médicale, Hôpital Saint-Louis, APHP and Inserm, UMR-1153, ECSTRA Team, Paris, France
| | - Valérie Paradis
- Université de Paris, Centre de recherche sur l'inflammation, Inserm, U1149, CNRS, ERL8252, Paris, France.,Service d'Anatomopathologie, DHU Unity, DMU Digest, Hôpital Beaujon, AP-HP, Clichy, France
| | - Marta Magaz
- Hepatic Hemodynamic Laboratory, European Reference Network for Rare Liver Disorders, Liver Unit, Hospital Clinic, IDIBAPS and CIBERehd, Barcelona, Spain
| | | | - Laura Rubbia-Brandt
- Service d'anatomie et cytologie pathologiques, Hôpitaux Universitaires de Genève, Switzerland
| | - Lucile Moga
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - François Durand
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Audrey Payancé
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Aurélie Plessier
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Cendrine Chaffaut
- Service de Biostatistiques et Information médicale, Hôpital Saint-Louis, APHP and Inserm, UMR-1153, ECSTRA Team, Paris, France
| | - Dominique Valla
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Marion Malphettes
- Service d'Immunopathologie clinique, Hôpital Saint-Louis, AP-HP, Paris, France
| | - Alba Diaz
- Pathology Unit, Hospital Clinic, IDIBAPS and CIBERehd, Barcelona, Spain
| | - Jean-Charles Nault
- AP-HP, Hôpital Jean Verdier, Liver Unit, Bondy, France.,University Paris 13, Sorbonne Paris Cité, "équipe labellisée Ligue Contre le Cancer", Bobigny, France.,Inserm, UMR-1162 «Functional Genomics of Solid Tumors», Paris, France
| | - Pierre Nahon
- AP-HP, Hôpital Jean Verdier, Liver Unit, Bondy, France.,University Paris 13, Sorbonne Paris Cité, "équipe labellisée Ligue Contre le Cancer", Bobigny, France.,Inserm, UMR-1162 «Functional Genomics of Solid Tumors», Paris, France
| | - Etienne Audureau
- AP-HP, Hôpital Henri Mondor, Département de Santé Publique, and Université Paris-Est, A-TVB DHU, CEpiA (Clinical Epidemiology and Aging) Unit EA4393, UPEC, Créteil, France
| | - Vlad Ratziu
- Service d'Hépato-gastroentérologie, Hôpital Pitié-Salpêtrière, AP-HP, Paris, France
| | - Laurent Castera
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
| | - Juan-Carlos Garcia Pagan
- Hepatic Hemodynamic Laboratory, European Reference Network for Rare Liver Disorders, Liver Unit, Hospital Clinic, IDIBAPS and CIBERehd, Barcelona, Spain
| | - Nathalie Ganne-Carrie
- AP-HP, Hôpital Jean Verdier, Liver Unit, Bondy, France.,University Paris 13, Sorbonne Paris Cité, "équipe labellisée Ligue Contre le Cancer", Bobigny, France.,Inserm, UMR-1162 «Functional Genomics of Solid Tumors», Paris, France
| | - Pierre-Emmanuel Rautou
- Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France
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39
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Vuille-Lessard É, Rodrigues SG, Berzigotti A. Noninvasive Detection of Clinically Significant Portal Hypertension in Compensated Advanced Chronic Liver Disease. Clin Liver Dis 2021; 25:253-289. [PMID: 33838850 DOI: 10.1016/j.cld.2021.01.005] [Citation(s) in RCA: 29] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Patients with compensated advanced chronic liver disease have different prognoses depending on the presence of portal hypertension. Current non-invasive diagnostic methods allow identification of clinically significant portal hypertension. Portosystemic collaterals on imaging or liver stiffness of more than 20 to 25 kPa by using transient elastography identifies patients with clinically significant portal hypertension. Patients with liver stiffness of less than 20 kPa and platelet count of greater than 150 g/L can avoid endoscopy. This rule could be expanded using spleen stiffness. Methods to risk stratify for portal hypertension in compensated advanced chronic liver disease and successfully treated chronic hepatitis C and B are subject of research.
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Affiliation(s)
- Élise Vuille-Lessard
- Hepatology, University Clinic for Visceral Surgery and Medicine (UVCM), Inselspital, University Hospital of Bern, Freiburgstrasse, 3010 Bern, Switzerland; Department of Biomedical Research, University of Bern, Switzerland
| | - Susana G Rodrigues
- Hepatology, University Clinic for Visceral Surgery and Medicine (UVCM), Inselspital, University Hospital of Bern, Freiburgstrasse, 3010 Bern, Switzerland; Department of Biomedical Research, University of Bern, Switzerland
| | - Annalisa Berzigotti
- Hepatology, University Clinic for Visceral Surgery and Medicine (UVCM), Inselspital, University Hospital of Bern, Freiburgstrasse, 3010 Bern, Switzerland; Department of Biomedical Research, University of Bern, Switzerland.
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40
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Veldhuijzen van Zanten D, Buganza E, Abraldes JG. The Role of Hepatic Venous Pressure Gradient in the Management of Cirrhosis. Clin Liver Dis 2021; 25:327-343. [PMID: 33838853 DOI: 10.1016/j.cld.2021.01.002] [Citation(s) in RCA: 16] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Quantifying the degree of portal hypertension provides useful information to estimate prognosis and to evaluate new therapies for portal hypertension. This quantification is done in clinical practice with the measurement of the hepatic venous pressure gradient. This article addresses the applications of measuring portal pressure in cirrhosis, including the differential diagnosis of portal hypertension; estimation of prognosis in cirrhosis, including preoperative evaluation before hepatic and extrahepatic surgery; assessment of the response to drug therapy (mainly in the context of drug development); and assessing the regression of portal hypertension syndrome.
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Affiliation(s)
- Daniel Veldhuijzen van Zanten
- Department of Medicine, University of Alberta, 13-103 Clinical Sciences Building, 11350-83 Avenue, Edmonton, Alberta T6G 2G3, Canada
| | - Elizabeth Buganza
- Division of Gastroenterology, Zeidler Ledcor Centre, University of Alberta, 8540 112 St NW, Edmonton, Alberta T6G 2X8, Canada
| | - Juan G Abraldes
- Division of Gastroenterology, University of Alberta, 8540 112 St NW, 1-38 Zeidler Ledcor Centre, Edmonton, Alberta T6G 2X8, Canada.
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41
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Kmeid M, Liu X, Ballentine S, Lee H. Idiopathic Non-Cirrhotic Portal Hypertension and Porto-Sinusoidal Vascular Disease: Review of Current Data. Gastroenterology Res 2021; 14:49-65. [PMID: 34007347 PMCID: PMC8110235 DOI: 10.14740/gr1376] [Citation(s) in RCA: 29] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/07/2021] [Accepted: 03/30/2021] [Indexed: 12/29/2022] Open
Abstract
Idiopathic non-cirrhotic portal hypertension (INCPH) is a clinicopathologic disease entity characterized by the presence of clinical signs and symptoms of portal hypertension (PH) in the absence of liver cirrhosis or known risk factors accountable for PH. Multiple hematologic, immune-related, infectious, hereditary and metabolic risk factors have been associated with this disorder. Still, the exact etiopathogenesis is largely unknown. The recently proposed porto-sinusoidal vascular disease (PSVD) scheme broadens the spectrum of the disease by also including patients without clinical PH who are found to have similar histopathologic findings on core liver biopsies. Three histomorphologic lesions have been identified as specific for PSVD to include obliterative portal venopathy, nodular regenerative hyperplasia and incomplete septal cirrhosis/fibrosis. However, these findings are often subtle, under-recognized and subjective with low interobserver agreement among pathologists. Additionally, the natural history of the subclinical forms of the disease remains unexplored. The clinical course is more favorable compared to cirrhosis patients, especially in the absence of clinical PH or liver dysfunction. There are no universally accepted guidelines in regard to diagnosis and treatment of INCPH/PSVD. Hence, this review emphasizes the need to raise awareness of this entity by highlighting its complex pathophysiology and clinicopathologic associations. Lastly, formulation of standardized diagnostic criteria with clinical validation is necessary to avoid misclassifying vascular diseases of the liver and to develop and implement targeted therapeutic strategies.
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Affiliation(s)
- Michel Kmeid
- Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY, USA
| | - Xiuli Liu
- Department of Pathology and Laboratory Medicine, University of Florida at Gainesville, FL, USA
| | - Samuel Ballentine
- Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO, USA
| | - Hwajeong Lee
- Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY, USA
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42
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Bochnakova T. Hepatic Venous Pressure Gradient. Clin Liver Dis (Hoboken) 2021; 17:144-148. [PMID: 33868655 PMCID: PMC8043695 DOI: 10.1002/cld.1031] [Citation(s) in RCA: 18] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/21/2020] [Revised: 08/10/2020] [Accepted: 08/15/2020] [Indexed: 02/06/2023] Open
Affiliation(s)
- Teodora Bochnakova
- Dotter Institute of Interventional RadiologyOregon Health & Science UniversityPortlandOR
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43
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Gao E, Hercun J, Heller T, Vilarinho S. Undiagnosed liver diseases. Transl Gastroenterol Hepatol 2021; 6:28. [PMID: 33824932 DOI: 10.21037/tgh.2020.04.04] [Citation(s) in RCA: 16] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2019] [Accepted: 03/19/2020] [Indexed: 02/06/2023] Open
Abstract
The landscape of chronic liver disease has drastically changed over the past 20 years, largely due to advances in antiviral therapy and the rise of metabolic syndrome and associated non-alcoholic fatty liver disease (NAFLD). Despite advances in the diagnosis and treatment of a variety of liver diseases, the burden of chronic liver disease is increasing worldwide. The first step to addressing any disease is accurate diagnosis. Here, we discuss liver diseases that remain undiagnosed, either because they are difficult to diagnose or due to hepatic manifestations of an unrecognized systemic disease. Additionally, their underlying etiology may remain unknown or they represent previously uncharacterized and therefore novel liver diseases. Our goal is to provide a framework for approaching undiagnosed liver diseases which elude standard hepatic diagnostic work-up and whose patterns of disease are often overlooked.
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Affiliation(s)
- Emily Gao
- Department of Internal Medicine, Section of Digestive Diseases, Yale School of Medicine, New Haven, CT, USA
| | - Julian Hercun
- Translational Hepatology Section, National Institute of Diabetes & Digestive & Kidney Diseases, National Institute of Health, Bethesda, MD, USA
| | - Theo Heller
- Translational Hepatology Section, National Institute of Diabetes & Digestive & Kidney Diseases, National Institute of Health, Bethesda, MD, USA
| | - Sílvia Vilarinho
- Department of Internal Medicine, Section of Digestive Diseases, Yale School of Medicine, New Haven, CT, USA.,Department of Pathology, Yale School of Medicine, New Haven, CT, USA
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44
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Ferrusquía-Acosta J, Bassegoda O, Turco L, Reverter E, Pellone M, Bianchini M, Pérez-Campuzano V, Ripoll E, García-Criado Á, Graupera I, García-Pagán JC, Schepis F, Senzolo M, Hernández-Gea V. Agreement between wedged hepatic venous pressure and portal pressure in non-alcoholic steatohepatitis-related cirrhosis. J Hepatol 2021; 74:811-818. [PMID: 33068638 DOI: 10.1016/j.jhep.2020.10.003] [Citation(s) in RCA: 52] [Impact Index Per Article: 13.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/25/2020] [Revised: 09/29/2020] [Accepted: 10/05/2020] [Indexed: 02/06/2023]
Abstract
BACKGROUND & AIMS Wedge hepatic vein pressure (WHVP) accurately estimates portal pressure (PP) in alcohol- or viral hepatitis-related cirrhosis. Whether this also holds true in cirrhosis caused by non-alcoholic steatohepatitis (NASH) is unknown. We aimed to evaluate the agreement between WHVP and PP in patients with NASH cirrhosis in comparison to patients with alcohol- or HCV-related cirrhosis. METHODS All consecutive patients with NASH cirrhosis treated with a transjugular intrahepatic portosystemic shunt (TIPS) in 3 European centres were included (NASH group; n = 40) and matched with 2 controls (1 with alcohol-related and 1 with HCV-related cirrhosis) treated with TIPS contemporaneously (control group; n = 80). Agreement was assessed by Pearson's correlation (R), intra-class correlation coefficient (ICC), and Bland-Altman method. Disagreement between WHVP and PP occurred when both pressures differed by >10% of PP value. A binary logistic regression analysis was performed to identify factors associated with this disagreement. RESULTS Correlation between WHVP and PP was excellent in the control group (R 0.92; p <0.001; ICC 0.96; p <0.001) and moderate in the NASH group (R 0.61; p <0.001; ICC 0.74; p <0.001). Disagreement between WHVP and PP was more frequent in the NASH group (37.5% vs. 14%; p = 0.003) and was mainly because of PP underestimation. In uni- and multivariate analyses, only NASH aetiology was associated with disagreement between WHVP and PP (odds ratio 4.03; 95% CI 1.60-10.15; p = 0.003). CONCLUSIONS In patients with decompensated NASH cirrhosis, WHVP does not estimate PP as accurately as in patients with alcohol- or HCV-related cirrhosis, mainly because of PP underestimation. Further studies aimed to assess this agreement in patients with compensated NASH cirrhosis are needed. LAY SUMMARY Portal pressure is usually assessed by measuring wedge hepatic vein pressure because of solid evidence demonstrating their excellent agreement in alcohol- and viral hepatitis-related cirrhosis. Our results show that in patients with decompensated cirrhosis caused by non-alcoholic steatohepatitis, wedge hepatic vein pressure estimates portal pressure with less accuracy than in patients with other aetiologies of cirrhosis, mainly because of portal pressure underestimation.
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Affiliation(s)
- José Ferrusquía-Acosta
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver), Barcelona, Spain; Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain
| | - Octavi Bassegoda
- Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain
| | - Laura Turco
- Division of Gastroenterology, Azienda Ospedaliero-Universitaria di Modena and University of Modena and Reggio Emilia, Modena, Italy; PhD Program in Clinical and Experimental Medicine, University of Modena and Reggio Emilia, Modena, Italy
| | - Enric Reverter
- Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain
| | - Monica Pellone
- University Hospital of Padua, Multivisceral Transplant Unit, Gastroenterology, Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver) Padua, Italy
| | - Marcello Bianchini
- Division of Gastroenterology, Azienda Ospedaliero-Universitaria di Modena and University of Modena and Reggio Emilia, Modena, Italy
| | - Valeria Pérez-Campuzano
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver), Barcelona, Spain; Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain
| | - Enric Ripoll
- Centre de Diagnostic per l'Imatge, Hospital Clínic, Barcelona, Spain
| | | | - Isabel Graupera
- Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Centro de Investigación Biomédica Red de Enfermedades Hepáticas y Digestivas, Madrid, Spain
| | - Juan Carlos García-Pagán
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver), Barcelona, Spain; Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Centro de Investigación Biomédica Red de Enfermedades Hepáticas y Digestivas, Madrid, Spain
| | - Filippo Schepis
- Division of Gastroenterology, Azienda Ospedaliero-Universitaria di Modena and University of Modena and Reggio Emilia, Modena, Italy
| | - Marco Senzolo
- University Hospital of Padua, Multivisceral Transplant Unit, Gastroenterology, Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver) Padua, Italy
| | - Virginia Hernández-Gea
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN-Liver), Barcelona, Spain; Liver Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Centro de Investigación Biomédica Red de Enfermedades Hepáticas y Digestivas, Madrid, Spain.
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Abstract
PURPOSE OF THE REVIEW Non-cirrhotic portal hypertension (NCPH) includes a heterogeneous group of conditions. The aim of this paper is to make an overview on the denominations, diagnostical features and management of porto-sinusoidal vascular disease (PSVD) and chronic portal vein thrombosis (PVT) being the main causes of NCPH in the Western world. RECENT FINDINGS The management of NCPH consists in the treatment of associated diseases and of portal hypertension (PH). PH due to PSVD or PVT is managed similarly to PH due to cirrhosis. TIPS placement and liver transplantation are considerable options in patients with refractory variceal bleeding/ascites and with progressive liver failure. Anticoagulation is a cornerstone both in the treatment of thrombosis in PSVD and in the prevention of thrombosis recurrence in patients with portal cavernoma. Physicians should be aware of the existence of PSVD and chronic PVT and actively search them in particular settings. To now, the management of portal hypertension-related complications in NCPH is the same of those of cirrhosis. Large cooperative studies on the natural history of NCPH are necessary to better define its management.
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Giudicelli H, Rautou PE, Paradis V, Bedossa P, Goria O, Lambert V, Hernandez-Gea V, Dutheil D, Plessier A, Bureau C, Valla D. Porto-sinusoidal vascular disease. Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver. Clin Res Hepatol Gastroenterol 2020; 44:447-451. [PMID: 32335045 DOI: 10.1016/j.clinre.2020.03.005] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2020] [Accepted: 03/03/2020] [Indexed: 02/04/2023]
Affiliation(s)
- Héloïse Giudicelli
- Department of hepatology, DHU Unity, Beaujon hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France
| | - Pierre-Emmanuel Rautou
- Department of hepatology, DHU Unity, Beaujon hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France; Inserm, center for research in inflammation, university of Paris, 75018 Paris, France; Reference center of vascular liver diseases European Reference Network (ERN) "Rare-Liver", France; French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine hospital, AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France.
| | - Valérie Paradis
- Department of pathology, Beaujon hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France
| | - Pierre Bedossa
- Inserm, center for research in inflammation, university of Paris, 75018 Paris, France; Department of pathology, Beaujon hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France
| | - Odile Goria
- Department of gastroenterology and hepatology, Charles Nicolles hospital, university hospital of Rouen, 1, rue de Germont, 76031 Rouen cedex, France
| | - Vincent Lambert
- General medicine, 46, avenue Yolande-d'Aragon, 49100 Angers, France
| | - Virginia Hernandez-Gea
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona. Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas (CIBERehd). Health Care Provider of the European Reference Network onRare Liver Disorders (ERN-Liver), Spain; Reference center of vascular liver diseases European Reference Network (ERN) "Rare-Liver", France
| | - Danielle Dutheil
- French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine hospital, AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Association of patients with vascular liver diseases (AMVF), department of hepatology, Beaujon hospital, 100, boulevard du Général-Leclerc, 92118 Clichy, France
| | - Aurélie Plessier
- Department of hepatology, DHU Unity, Beaujon hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France; Reference center of vascular liver diseases European Reference Network (ERN) "Rare-Liver", France; French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine hospital, AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France
| | - Christophe Bureau
- Department of gastroenterology and hepatology, Rangueil hospital, university hospital of Toulouse, 1, avenue du Professeur Jean-Poulhès, 31400 Toulouse, France
| | - Dominique Valla
- Department of hepatology, DHU Unity, Beaujon hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France; Inserm, center for research in inflammation, university of Paris, 75018 Paris, France; Reference center of vascular liver diseases European Reference Network (ERN) "Rare-Liver", France; French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine hospital, AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France
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47
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Extrahepatic portal vein obstruction (EHPVO) in cirrhosis. Clin Res Hepatol Gastroenterol 2020; 44:497-502. [PMID: 32312599 DOI: 10.1016/j.clinre.2020.03.014] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2020] [Accepted: 03/03/2020] [Indexed: 02/04/2023]
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48
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Mandorfer M, Hernández-Gea V, García-Pagán JC, Reiberger T. Noninvasive Diagnostics for Portal Hypertension: A Comprehensive Review. Semin Liver Dis 2020; 40:240-255. [PMID: 32557480 DOI: 10.1055/s-0040-1708806] [Citation(s) in RCA: 40] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Noninvasive diagnostics for portal hypertension include imaging and functional tests, as well as blood-based biomarkers, and capture different features of the portal hypertensive syndrome. Definitive conclusions regarding their clinical utility require assessment of their diagnostic value in specific clinical settings (i.e., diagnosing a particular hemodynamic condition within a well-defined target population). Several noninvasive methods are predictive of clinically significant portal hypertension (CSPH; hepatic venous pressure gradient [HVPG] ≥ 10 mm Hg; the threshold for complications of portal hypertension); however, only a minority of them have been evaluated in compensated advanced chronic liver disease (i.e., the target population). Importantly, most methods correlate only weakly with HVPG at high values (i.e., in patients with CSPH). Nevertheless, selected methods show promise for diagnosing HVPG ≥ 16 mm Hg (the cut-off for increased risks of hepatic decompensation and mortality) and monitoring HVPG changes in response to nonselective beta-blockers or etiological treatments. Finally, we review established and potential future clinical applications of noninvasive methods.
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Affiliation(s)
- Mattias Mandorfer
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria.,Barcelona Hepatic Hemodynamic Lab, Liver Unit, Hospital Clínic, Barcelona, Spain
| | - Virginia Hernández-Gea
- Barcelona Hepatic Hemodynamic Lab, Liver Unit, Hospital Clínic, Barcelona, Spain.,Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.,Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Madrid, Spain
| | - Juan Carlos García-Pagán
- Barcelona Hepatic Hemodynamic Lab, Liver Unit, Hospital Clínic, Barcelona, Spain.,Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.,Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Madrid, Spain
| | - Thomas Reiberger
- Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
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49
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Porto-Sinusoidal Vascular Disease as the Cause of Portal Hypertension in Felty's Syndrome: A Case Report and Literature Review. BIOMED RESEARCH INTERNATIONAL 2020; 2020:2618260. [PMID: 32714976 PMCID: PMC7352150 DOI: 10.1155/2020/2618260] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/09/2020] [Revised: 05/24/2020] [Accepted: 06/01/2020] [Indexed: 11/17/2022]
Abstract
Felty's syndrome (FS) is a disorder wherein patients with rheumatoid arthritis develop splenomegaly, neutropenia, and in some cases, portal hypertension without underlying cirrhosis. Esophageal variceal bleeding is a complication of FS in patients with portal hypertension. In contrast to splenectomy, few reports exist on the management of variceal bleeding with endoscopic therapy. Moreover, the long-term outcome has not been reported. We present a patient with esophageal variceal bleeding due to portal hypertension secondary to Felty's syndrome. The patient was followed up for two years postendoscopy intervention. Literature review was performed and the histological features of portal hypertension in FS are discussed. The patient presented with a typical triad of rheumatoid arthritis (RA), splenomegaly, and neutropenia and was diagnosed as Felty's syndrome in 2012. She was admitted to our hospital in September 2017 for esophageal variceal bleeding. At the time of admission, her liver function test was normal. Abdominal CT showed no signs of cirrhosis and portal vein obstruction. Liver biopsy further excluded diagnosis of cirrhosis and supported the diagnosis of porto-sinusoidal vascular disease (PSVD), which was previously named as noncirrhotic idiopathic portal hypertension (NCIPH). An upper abdominal endoscopy revealed gastric and esophageal varices. A series of endoscopies was performed to ligate the esophageal varices. The patient was followed up for two years and did not show rebleeding. In conclusion, comorbid PSVD might be a cause of portal hypertension in FS patients. The present case had excellent outcome in two years, which supported the use of endoscopic therapy for the management of variceal bleeding in FS patients. Further large prospective study is needed to confirm the findings.
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50
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Nicoară-Farcău O, Rusu I, Stefănescu H, Tanțău M, Badea RI, Procopeț B. Diagnostic challenges in non-cirrhotic portal hypertension - porto sinusoidal vascular disease. World J Gastroenterol 2020; 26:3000-3011. [PMID: 32587444 PMCID: PMC7304099 DOI: 10.3748/wjg.v26.i22.3000] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/30/2019] [Revised: 03/31/2020] [Accepted: 05/27/2020] [Indexed: 02/06/2023] Open
Abstract
Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension, which differ from cirrhosis through histological alterations, hemodynamic characterization and, clinical outcome. Because of the similarities in clinical presentation and imaging signs, frequently these patients, and particularly those with porto-sinusoidal vascular disease (PSVD), are misdiagnosed as having liver cirrhosis and thus raising difficulties in their diagnosis. The most challenging differentiation to be considered is between PSVD and cirrhosis and, although not pathognomonic, liver biopsy is still the standard of diagnosis. Although they still require extended validation before being broadly used, new non-invasive methods for the diagnosis of porto-sinusoidal vascular disease, like transient elastography, contrast-enhanced ultrasound or metabolomic profiling, have shown promising results. Another issue is the differentiation between PSVD and chronic extrahepatic portal vein obstruction, especially now when it is known that 40% of patients suffering from PSVD develop portal vein thrombosis. In this particular case, once the portal vein thrombosis occurred, the diagnosis of PSVD is impossible according to the current guidelines. Moreover, so far, the differentiation between PSVD and sinusoidal obstruction syndrome has not been clear so far in particular circumstances. In this review we highlighted the diagnostic challenges regarding the PSVD, as well as the current techniques used in the evaluation of these patients.
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Affiliation(s)
- Oana Nicoară-Farcău
- University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
- Gastroenterology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
| | - Ioana Rusu
- University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
- Pathology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
| | - Horia Stefănescu
- Gastroenterology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
| | - Marcel Tanțău
- University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
- Gastroenterology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
| | - Radu Ion Badea
- University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
- Imagistic Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
| | - Bogdan Procopeț
- University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
- Gastroenterology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
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