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Mills KC, Majumder S. What Is the Latest in Autoimmune Pancreatitis. Gastroenterol Clin North Am 2025; 54:245-258. [PMID: 39880531 DOI: 10.1016/j.gtc.2024.08.021] [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: 01/02/2025]
Abstract
Autoimmune pancreatitis (AIP) is a steroid-responsive fibroinflammatory disorder with 2 clinically distinct subtypes known as type 1 autoimmune and type 2 autoimmune pancreatitis. Type 1 AIP is considered the pancreatic manifestation of immunoglobulin G4-related disease, a systemic disease often presenting with other organ involvement. Advances in understanding the unique clinical presentation, imaging findings, histopathology, and clinical course of this relatively uncommon disease have led to international consensus regarding diagnosis and treatment. While corticosteroids remain the mainstay of treatment, several emerging novel therapies have been explored primarily in the context or relapsing and refractory cases.
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Affiliation(s)
- Krystal C Mills
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA
| | - Shounak Majumder
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA.
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2
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Franchina M, Dell’Oro L, Massironi S. Autoimmune Pancreatitis Mimicking a Pancreatic Neuroendocrine Tumor: A Case Report with a Literature Review. Int J Mol Sci 2025; 26:1536. [PMID: 40004001 PMCID: PMC11855540 DOI: 10.3390/ijms26041536] [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: 12/15/2024] [Revised: 01/29/2025] [Accepted: 02/03/2025] [Indexed: 02/27/2025] Open
Abstract
Autoimmune pancreatitis (AIP) is a rare chronic pancreatitis subtype that often mimics pancreatic cancer due to the overlapping clinical and radiological features, posing significant diagnostic challenges. Similarly, distinguishing AIP from pancreatic neuroendocrine neoplasms (PanNENs), which present with nonspecific symptoms, adds complexity to clinical evaluations. We present the case of a 46-year-old male with recurrent acute idiopathic pancreatitis. Abdominal computed tomography (CT) revealed a 25 mm hypodense mass in the pancreatic tail with mild arterial contrast enhancement. Magnetic resonance imaging (MRI) showed the mass to be hypointense on T2-weighted sequences, with no diffusion restriction and an enhancement pattern akin to normal pancreatic tissue. The endoscopic ultrasound-guided fine needle biopsy (EUS-FNB) was inconclusive. Gallium-68 DOTATATE positron emission tomography-CT (Ga-68 DOTATATE PET-CT) showed an increased tracer uptake, leading to a distal pancreatectomy with a splenectomy. Histopathology demonstrated chronic sclerotic pancreatitis with inflammatory infiltrates. Elevated serum IgG4 levels confirmed the diagnosis of type 1 AIP Differentiating AIP from pancreatic malignancies, including PanNENs, is both critical and complex. This case highlights a misdiagnosis of PanNENs in a patient with focal AIP, where neuroendocrine hyperplasia and islet cell clusters within fibrotic areas mimicked PanNENs, even on Ga-68 PET-CT. The findings emphasize the potential for false positives with Ga-68 DOTATATE PET-CT and the importance of integrating clinical, radiological, and histological data for an accurate diagnosis.
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Affiliation(s)
- Marianna Franchina
- Division of Gastroenterology, ASST Papa Giovanni XXIII, 24127 Bergamo, Italy;
| | - Liliana Dell’Oro
- Department of Medicine and Surgery, University of Milano-Bicocca, 20126 Milan, Italy;
| | - Sara Massironi
- Department of Medicine and Surgery, Vita-Salute San Raffaele University, 20132 Milan, Italy
- Istituti Ospedalieri Bergamaschi, 24040 Zingonia, Italy
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3
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Zhou JF, Chen Z. A rare case of IgG4-related pancreatitis diagnosed preoperatively by endoscopic ultrasound-guided fine needle aspiration. Hepatobiliary Pancreat Dis Int 2024; 23:648-652. [PMID: 38556381 DOI: 10.1016/j.hbpd.2024.03.004] [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: 11/04/2023] [Accepted: 03/20/2024] [Indexed: 04/02/2024]
Affiliation(s)
- Jun-Feng Zhou
- Department of Hepatobiliary Surgery, The 960th Hospital of PLA, Jinan 250031, China
| | - Zhong Chen
- Department of Hepatobiliary Surgery, The 960th Hospital of PLA, Jinan 250031, China.
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Shimada R, Yamada Y, Okamoto K, Murakami K, Motomura M, Takaki H, Fukuzawa K, Asayama Y. Pancreatic volume change using three dimensional-computed tomography volumetry and its relationships with diabetes on long-term follow-up in autoimmune pancreatitis. World J Radiol 2024; 16:644-656. [PMID: 39635311 PMCID: PMC11612800 DOI: 10.4329/wjr.v16.i11.644] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/25/2024] [Revised: 09/07/2024] [Accepted: 09/23/2024] [Indexed: 11/27/2024] Open
Abstract
BACKGROUND Several studies found that early pancreatic atrophy detected by computed tomography (CT) within 6 months was associated with a high incidence of diabetes in patients with type-1 autoimmune pancreatitis (AIP) receiving steroid therapy; however, no long-term follow-up studies have been performed. AIM To investigate pancreatic volume (PV) changes using three dimensional (3D)-CT volumetry and their relationship with IgG4 and diabetes in patients with AIP. METHODS This retrospective study included 33 patients with type-1 AIP receiving steroid therapy. Patients were divided into diffuse (D-type) and mass-forming type (M-type) AIP. PV was determined by semi-automated 3D-CT volumetry, and changes between initial and follow-up values were calculated. The relationship between PV and serum IgG4 levels was analyzed by Spearman's rank correlation. The PV atrophy ratio compared with the presumed normal PV at the time of last follow-up CT and its relationship with diabetes were investigated. RESULTS There were 16 D-type and 17 M-type patients with long-term follow-up (mean, 95.8 months). The regression curve of mean relative PV change reduced exponentially and rapidly during the first 25 months and then more slowly in both groups. The overall cumulative pancreas re-enlargement rates at 1, 3, 5, 7 and 10 years were 6.1%, 12.2%, 29.2%, 47.5% and 55.0%, respectively. There was a moderate-to-very strong positive correlation (ρ ≥ 0.4) between PV and serum IgG4 levels in nine (9/13, 69.2%) patients. All 33 patients showed pancreatic atrophy (mean 59.3%) after long-term follow-up. Patients with D-type AIP had a significantly higher atrophy rate and higher incidence of diabetes than M-type patients (P < 0.05). CONCLUSION PV change initially reduced exponentially and then more slowly and is considered an important factor associated with diabetes. Serum IgG4 levels were positively correlated with PV during follow-up.
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Affiliation(s)
- Ryuichi Shimada
- Department of Radiology, Oita University Faculty of Medicine, Yufu 879-5593, Oita, Japan
| | - Yasunari Yamada
- Department of Radiology, Oita Red Cross Hospital, Oita 870-0033, Oita, Japan
| | - Kazuhisa Okamoto
- Department of Gastroenterology, Oita University Faculty of Medicine, Yufu 879-5593, Oita, Japan
| | - Kazunari Murakami
- Department of Gastroenterology, Oita University Faculty of Medicine, Yufu 879-5593, Oita, Japan
| | - Mitsuteru Motomura
- Department of Hepato-Biliary-Pancreatic Internal Medicine, Oita Red Cross Hospital, Oita 870-0033, Oita, Japan
| | - Hajime Takaki
- Department of Radiology, Oita Red Cross Hospital, Oita 870-0033, Oita, Japan
| | - Kengo Fukuzawa
- Department of Surgery, Oita Red Cross Hospital, Oita 870-0033, Oita, Japan
| | - Yoshiki Asayama
- Department of Radiology, Oita University Faculty of Medicine, Yufu 879-5593, Oita, Japan
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Zhou Y, Sun L, Wang X, Wan D, Xu J, Jiang M, Liu Y, Liu C, Tu Y, Huang H, Jin Z. EUS-guided fine-needle biopsy versus fine-needle aspiration for histopathological evidence for type 1 autoimmune pancreatitis: A single-center retrospective study in China. Endosc Ultrasound 2024; 13:351-360. [PMID: 39802102 PMCID: PMC11723699 DOI: 10.1097/eus.0000000000000095] [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: 04/24/2024] [Accepted: 10/27/2024] [Indexed: 01/16/2025] Open
Abstract
Background and Objectives EUS is recommended for guiding pancreatic tissue acquisition in suspected autoimmune pancreatitis (AIP) cases. However, there is a lack of comparative research on the effectiveness between EUS-guided fine-needle aspiration (EUS-FNA) and EUS-guided fine-needle biopsy (EUS-FNB) for diagnosing AIP in China. This study aimed to evaluate the diagnostic accuracy of EUS-guided tissue acquisition (EUS-TA) specifically for type 1 AIP. Methods Between 2010 and 2023, individuals with AIP who received EUS-TA at Changhai Hospital were included in the study. Results A total of 173 patients diagnosed with AIP who underwent EUS-TA were included in the final analysis. Of these, 104 patients (60.1%) received EUS-FNA, and 69 patients (39.9%) underwent EUS-FNB. Sufficient pancreatic tissue samples (>5 cells/high-power field) were obtained in 164 of 173 patients (94.8%), with success rates of 94.2% for EUS-FNA and 95.7% for EUS-FNB (P > 0.05). EUS-FNB exhibited higher rates of reliable level 1 histopathological findings (40.9% vs. 16.3%, P < 0.001) and reliable level 2 histopathological findings (33.3% vs. 12.2%, P < 0.001) compared with EUS-FNA. Furthermore, a higher occurrence of IgG4-positive plasma cell infiltration (>10 cells/high-power field) was observed with EUS-FNB compared with EUS-FNA (74.2% vs. 27.9%, P < 0.001). The multivariate logistic analysis also revealed that EUS-FNA was less effective in obtaining reliable evidence compared with EUS-FNB, as evident in both level 2 (P = 0.002; odds ratio, 0.21; 95% confidence interval, 0.08-0.56) and level 1 (P = 0.001; odds ratio, 0.19; 95% confidence interval, 0.08-0.49) histopathological evidence. Conclusions EUS-FNB demonstrates higher rates of level 1 and level 2 histopathological findings, as well as more abundant IgG4-positive plasma cell infiltration, compared with EUS-FNA.
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Affiliation(s)
- Yuyan Zhou
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
| | - Liqi Sun
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
| | - Xinyue Wang
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
| | - Dongling Wan
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
| | - Jiaheng Xu
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
| | - Mengruo Jiang
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
| | - Yue Liu
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
| | - Chao Liu
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
| | - Yatao Tu
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
| | - Haojie Huang
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
| | - Zhendong Jin
- Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China
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Ujita W, Kamisawa T, Chiba K, Nakahodo J, Tabata H, Setoguchi K, Igarashi Y, Matsuda T. New insights into predictors of autoimmune pancreatitis relapse after steroid therapy. Scand J Gastroenterol 2024; 59:1202-1208. [PMID: 39257347 DOI: 10.1080/00365521.2024.2398771] [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: 07/04/2024] [Revised: 08/08/2024] [Accepted: 08/27/2024] [Indexed: 09/12/2024]
Abstract
OBJECTIVES While autoimmune pancreatitis (AIP) responds well to steroid therapy, the high relapse rate in type 1 AIP remains a critical problem. The present study examined predictors of relapse of type 1 AIP following steroid therapy. MATERIALS AND METHODS Nine factors potentially predictive of relapse were analyzed in 81 AIP patients receiving steroid therapy with follow-up ≥ 12 months. The rate of serum IgG4 decrease following steroid therapy was calculated by dividing the difference between serum IgG4 values before and at two months after the start of steroid by the IgG4 value before steroid. RESULTS A relapse occurred in 11 patients (13.5%) during a median of 38 months. Multivariate analysis revealed that the presence of IgG4-related retroperitoneal fibrosis (HR: 5.59; 95% CI: 1.42-22.0; p = 0.014) and the low rate of serum IgG4 decrease after steroid therapy (HR: 0.048; 95% CI: 0.005-0.46; p = 0.008) were significant, independent predictors of AIP relapse. The cut-off value based on receiver operating characteristic curve data for the rate of serum IgG4 decrease before and at two months after steroid therapy distinguishing patients with and without a relapse was 0.65. Using this cut-off value, the area under the curve, sensitivity, and specificity were found to be 0.63, 0.73, and 0.60, respectively. CONCLUSION The low rate of serum IgG4 decrease after the start of steroid therapy and the presence of IgG4-related retroperitoneal fibrosis were predictive of type 1 AIP relapse. Cautious, gradual tapering of steroid dosage and longer maintenance therapy are recommended for patients with these factors.
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Affiliation(s)
- Wataru Ujita
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Toho University Omori Medical Center, Tokyo, Japan
| | - Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
- Center of IgG4-Related Disease, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
| | - Kazuro Chiba
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
- Center of IgG4-Related Disease, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
| | - Jun Nakahodo
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
| | - Hiroki Tabata
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
| | - Keigo Setoguchi
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
- Center of IgG4-Related Disease, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
| | - Yoshinori Igarashi
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Toho University Omori Medical Center, Tokyo, Japan
| | - Takahisa Matsuda
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Toho University Omori Medical Center, Tokyo, Japan
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Moqran S, Tahiri El Ousrouti L, Hammas N, El Bouardi N, Chbani L. Uncommon Etiology of Pancreatic Mass: a Case Report. Cureus 2024; 16:e66879. [PMID: 39280381 PMCID: PMC11398729 DOI: 10.7759/cureus.66879] [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: 08/14/2024] [Indexed: 09/18/2024] Open
Abstract
IgG4-related autoimmune pancreatitis (AIP) is a chronic inflammatory disease of the pancreas with a distinct histological feature. Its diagnosis remains challenging as some features overlap with pancreatic cancer. We present a case of IgG4-related AIP mimicking pancreatic cancer. A 70-year-old male patient presented with epigastric pain, radiating to the entire abdomen with an unquantified weight loss. Magnetic resonance cholangiopancreatography (MRCP) showed a mass with a 28 mm long axis, in the head of the pancreas with pancreatic duct dilatation. Thus, it was presumed to be a pancreatic neoplasm and pancreatic resection was undertaken without a definitive preoperative diagnosis. In terms of clinical presentation, imaging characteristics, and laboratory parameters, IgG4-related AIP can resemble pancreatic cancer. Thus, histopathological studies remain the gold standard for a definitive diagnosis that may show a diffuse lymphoplasmacytic infiltrate with storiform fibrosis. On immunohistochemistry, the majority of plasma cells are positive for IgG4 (>50 per high-power field (HPF)). In our case, the histologic diagnosis allowed us to suggest the diagnosis of IgG4-related AIP and the immunohistochemical diagnosis confirmed the diagnosis. It is critical to distinguish pancreatic cancer from IgG4-related AIP due to its completely different prognosis and therapy. Steroids are the first-line treatment that allow a reduction of risk of relapse; therefore, a misdiagnosis as a malignancy leads to inappropriate surgical interventions. In this case, a biopsy is recommended.
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Affiliation(s)
- Sanae Moqran
- Laboratory Medicine, Faculty of Medicine, Pharmacy and Dental Medicine, Fez, MAR
- Pathology, Hassan II University Hospital, Fez, MAR
| | | | - Nawal Hammas
- Pathology, Hassan II University Hospital, Fez, MAR
| | | | - Laila Chbani
- Pathology, Hassan II University Hospital, Fez, MAR
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Metelli F, Manfredi G, Pagano N, Buscarini E, Crinò SF, Armellini E. The Role of Endoscopic Ultrasound and Ancillary Techniques in the Diagnosis of Autoimmune Pancreatitis: A Comprehensive Review. Diagnostics (Basel) 2024; 14:1233. [PMID: 38928649 PMCID: PMC11202526 DOI: 10.3390/diagnostics14121233] [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: 04/09/2024] [Revised: 06/04/2024] [Accepted: 06/07/2024] [Indexed: 06/28/2024] Open
Abstract
Autoimmune pancreatitis (AIP) is a unique form of chronic pancreatitis with a multifactorial pathogenesis. Historically, it has been classified as type 1 and type 2, according to its clinical and histological features. The diagnosis of AIP is challenging and relies on a combination of clinical, histopathologic, serologic, and imaging characteristics. In the available guidelines, the imaging hallmarks of AIP are based on cross-sectional imaging and cholangiopancreatography retrograde endoscopic findings. Endoscopic ultrasound (EUS) is generally used for pancreatic tissue acquisition to rule out pancreatic cancer and diagnose AIP with limited accuracy. Several papers reported the reliability of EUS for providing informative morphologic features of AIP. Nowadays, the improvement in the resolution of EUS conventional images and the development of new ancillary technologies have further increased the diagnostic yield of EUS: contrast-enhanced EUS and EUS elastography are non-invasive and real-time techniques that strongly support the diagnosis and management of pancreatic diseases. In this review article, we will present the role of conventional EUS and ancillary diagnostic techniques in the diagnosis of AIP to support clinicians and endosonographers in managing this condition.
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Affiliation(s)
- Flavio Metelli
- Gastroenterology and Endoscopy Department, ASST Maggiore Hospital Crema, 26013 Crema, Italy; (F.M.); (G.M.); (E.B.)
| | - Guido Manfredi
- Gastroenterology and Endoscopy Department, ASST Maggiore Hospital Crema, 26013 Crema, Italy; (F.M.); (G.M.); (E.B.)
| | - Nico Pagano
- Gastroenterology Unit, Department of Oncological and Specialty Medicine, University Hospital Maggiore della Carità, 28100 Novara, Italy;
| | - Elisabetta Buscarini
- Gastroenterology and Endoscopy Department, ASST Maggiore Hospital Crema, 26013 Crema, Italy; (F.M.); (G.M.); (E.B.)
| | - Stefano Francesco Crinò
- Diagnostic and Interventional Endoscopy of Pancreas, Pancreas Institute, University of Verona, 37134 Verona, Italy;
| | - Elia Armellini
- Gastroenterology and Endoscopy Unit, ASST-Bergamoest, 24068 Seriate, Italy
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Takikawa T, Kikuta K, Sano T, Ikeura T, Fujimori N, Umemura T, Naitoh I, Nakase H, Isayama H, Kanno A, Kamata K, Kodama Y, Inoue D, Ido A, Ueki T, Seno H, Yasuda H, Iwasaki E, Nishino T, Kubota K, Arizumi T, Tanaka A, Uchida K, Matsumoto R, Hamada S, Nakamura S, Okazaki K, Takeyama Y, Masamune A. Maintenance steroid therapy is associated with decreased risk of malignancy and better prognosis of patients with autoimmune pancreatitis: A multicenter cohort study in Japan. Pancreatology 2024; 24:335-342. [PMID: 38336506 DOI: 10.1016/j.pan.2024.01.008] [Citation(s) in RCA: 3] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/01/2023] [Revised: 01/15/2024] [Accepted: 01/17/2024] [Indexed: 02/12/2024]
Abstract
BACKGROUND/OBJECTIVES The association between autoimmune pancreatitis (AIP) and pancreatic cancer (PC) remains controversial. This study aimed to clarify the long-term prognosis and risk of malignancies in AIP patients in Japan. METHODS We conducted a multicenter retrospective cohort study on 1364 patients with type 1 AIP from 20 institutions in Japan. We calculated the standardized incidence ratio (SIR) for malignancies compared to that in the general population. We analyzed factors associated with overall survival, pancreatic exocrine insufficiency, diabetes mellitus, and osteoporosis. RESULTS The SIR for all malignancies was increased (1.21 [95 % confidence interval: 1.05-1.41]) in patients with AIP. Among all malignancies, the SIR was highest for PC (3.22 [1.99-5.13]) and increased within 2 years and after 5 years of AIP diagnosis. Steroid use for ≥6 months and ≥50 months increased the risk of subsequent development of diabetes mellitus and osteoporosis, respectively. Age ≥65 years at AIP diagnosis (hazard ratio [HR] = 3.73) and the development of malignancies (HR = 2.63), including PC (HR = 7.81), were associated with a poor prognosis, whereas maintenance steroid therapy was associated with a better prognosis (HR = 0.35) in the multivariate analysis. Maintenance steroid therapy was associated with a better prognosis even after propensity score matching for age and sex. CONCLUSIONS Patients with AIP are at increased risk of developing malignancy, especially PC. PC is a critical prognostic factor for patients with AIP. Although maintenance steroid therapy negatively impacts diabetes mellitus and osteoporosis, it is associated with decreased cancer risk and improved overall survival.
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Affiliation(s)
- Tetsuya Takikawa
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Japan
| | - Kazuhiro Kikuta
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Japan
| | - Takanori Sano
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Japan
| | - Tsukasa Ikeura
- Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Nao Fujimori
- Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Takeji Umemura
- Department of Medicine, Division of Gastroenterology and Hepatology, Shinshu University School of Medicine, Matsumoto, Japan
| | - Itaru Naitoh
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Hiroshi Nakase
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Hiroyuki Isayama
- Department of Gastroenterology, Graduate School of Medicine, Juntendo University, Tokyo, Japan
| | - Atsushi Kanno
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan
| | - Ken Kamata
- Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Osaka, Japan
| | - Yuzo Kodama
- Division of Gastroenterology, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe, Japan
| | - Dai Inoue
- Department of Radiology, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan
| | - Akio Ido
- Digestive and Lifestyle Diseases, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
| | - Toshiharu Ueki
- Department of Gastroenterology, Fukuoka University Chikushi Hospital, Fukuoka, Japan
| | - Hiroshi Seno
- Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, Kyoto, Japan
| | - Hiroaki Yasuda
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Eisuke Iwasaki
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Takayoshi Nishino
- Department of Gastroenterology Tokyo Womens' Medical University Yachiyo Medical Center, Yachiyo, Japan
| | - Kensuke Kubota
- Division of Gastroenterology, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Toshihiko Arizumi
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
| | - Atsushi Tanaka
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
| | - Kazushige Uchida
- Department of Gastroenterology and Hepatology, Kochi Medical School, Kochi University, Kochi, Japan
| | - Ryotaro Matsumoto
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Japan
| | - Shin Hamada
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Japan
| | - Seiji Nakamura
- Faculty of Dental Science, Kyushu University, Fukuoka, Japan
| | - Kazuichi Okazaki
- Department of Internal Medicine, Kansai Medical University, Kori Hospital, Neyagawa, Japan
| | - Yoshifumi Takeyama
- Department of Surgery, Kindai University Faculty of Medicine, Osaka, Japan
| | - Atsushi Masamune
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Japan.
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10
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Yang X, Zhou H, Wang W, Yan C, Ji G. Recent advances in IgG4-related autoimmune pancreatitis. Pathol Res Pract 2024; 257:155331. [PMID: 38678849 DOI: 10.1016/j.prp.2024.155331] [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: 03/14/2024] [Revised: 04/16/2024] [Accepted: 04/24/2024] [Indexed: 05/01/2024]
Abstract
The incidence of IgG4-related autoimmune pancreatitis (IgG4-AIP) is high in Asia and other countries, and unnecessary treatment is often undertaken due to both missed diagnosis and misdiagnosis in clinical practice. Although IgG4-AIP has attracted increasing attention, the details of IgG4-AIP pathogenesis and systemic immune response, including its relationship to tumor pathogenesis, are still unclear. In recent years, research on serum immunological detection, pathological features, clinical manifestations, diagnosis and treatment measures for IgG4-AIP has gradually increased. It is of great importance to summarize and discuss the latest progress regarding IgG4-AIP disease.
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Affiliation(s)
- Xisheng Yang
- Department of Gastrointestinal Surgery, Xijing Hospital, Air Force Medical University, Xi'an, China
| | - Haikun Zhou
- Department of Gastrointestinal Surgery, Xijing Hospital, Air Force Medical University, Xi'an, China
| | - Weidong Wang
- Department of Gastrointestinal Surgery, Xijing Hospital, Air Force Medical University, Xi'an, China
| | - Chunyu Yan
- Department of Metabolism, Digestion and Reproduction, Faculty of Medicine, Imperial College London, London, UK
| | - Gang Ji
- Department of Gastrointestinal Surgery, Xijing Hospital, Air Force Medical University, Xi'an, China.
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Elbanna KY, Kowa JY, Mirajkar N, Khalili K, Kim TK. Radiology domain in the diagnosis of IgG4-RD according to the 2019 American College of Rheumatology and European League Against Rheumatism classification. Insights Imaging 2024; 15:94. [PMID: 38530483 DOI: 10.1186/s13244-024-01638-3] [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: 12/06/2023] [Accepted: 01/21/2024] [Indexed: 03/28/2024] Open
Abstract
OBJECTIVES To evaluate the performance of radiology-related inclusion criteria of the 2019 ACR-EULAR classification system in the diagnosis of IgG4-related disease (IgG4-RD). METHODS This retrospective single-institution study included patients who received a diagnosis of IgG4-RD between January 2010 and December 2020. Two abdominal radiologists independently reviewed baseline imaging studies and scored radiology findings according to the 2019 ACR-EULAR classification criteria. Additional scores were assigned based on serological, histopathological, and immunostaining features. RESULTS Seventy-four patients (58 males and 16 females) with a mean age of 59.3 ± 13.9 years diagnosed with IgG4-RD were included. 51/74 (68.9%) were classified as having IgG4-RD according to the 2019 ACR-EULAR classification criteria. To reach a score ≥ 20 in these 51 patients, the radiology domain was sufficient in 20/51 (39.2%) and adding the serology domain was required for another 20/51 (39.2%). The remaining 11/51 patients (21.6%) required the histopathology and immunostaining domains. Radiological involvement of two or more organs at presentation was significantly associated with a score of ≥ 20 and seen in 43/51 (84.3%) compared to 5/23 (21.7%) of the non-classified group (p < 0.001). The group classified as having IgG4-RD showed a significantly higher proportion of elevated IgG4 levels (39/51, 76.5%) than the non-classified group (8/23, 34.8%) (< 0.001). CONCLUSION The study findings support the effectiveness of the radiology-related inclusion criteria of the 2019 ACR-EULAR classification system in diagnosing IgG4-RD. Combining radiology and serology domains achieved the cut-off in 80% of IgG-RD patients, enabling non-invasive diagnosis. The classification of IgG4-RD was significantly associated with multi-organ involvement, particularly affecting the pancreas and biliary system. CRITICAL RELEVANCE STATEMENT This study is the first to evaluate the diagnostic performance of the radiology domain in the 2019 ACR-EULAR classification criteria. The study results confirm its utility and potential to enable non-invasive diagnosis when combined with serological testing in a significant proportion of patients. KEY POINTS • A significant proportion of patients can be diagnosed with IgG4-RD using the radiology and serology domains exclusively. • Multi-organ involvement is significantly associated with classifying patients as IgG4-RD, with the pancreas and biliary system most frequently affected. • A high level of inter-reader agreement in the scoring of the radiology domain supports its reliability.
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Affiliation(s)
- Khaled Y Elbanna
- University Medical Imaging Toronto, University Health Network, Toronto, ON, Canada.
| | - Jie-Ying Kowa
- University Medical Imaging Toronto, University Health Network, Toronto, ON, Canada
| | - Nikhil Mirajkar
- University Medical Imaging Toronto, University Health Network, Toronto, ON, Canada
| | - Korosh Khalili
- University Medical Imaging Toronto, University Health Network, Toronto, ON, Canada
| | - Tae Kyoung Kim
- University Medical Imaging Toronto, University Health Network, Toronto, ON, Canada
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12
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Czarnywojtek A, Agaimy A, Pietrończyk K, Nixon IJ, Vander Poorten V, Mäkitie AA, Zafereo M, Florek E, Sawicka-Gutaj N, Ruchała M, Ferlito A. IgG4-related disease: an update on pathology and diagnostic criteria with a focus on salivary gland manifestations. Virchows Arch 2024; 484:381-399. [PMID: 38316669 DOI: 10.1007/s00428-024-03757-0] [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: 12/01/2023] [Revised: 01/12/2024] [Accepted: 01/29/2024] [Indexed: 02/07/2024]
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ disorder characterized by a highly variable clinical presentation depending on the affected organ/s, extent of tumefactive fibroinflammatory lesions, and associated functional impairment. The disease pursues a chronic, relapsing, often asymptomatic course and hence may pose a significant diagnostic challenge. Diagnostic delay can lead to progressive fibrosis and irreversible organ damage resulting into significant morbidity and even mortality. Given its broad clinical spectrum, physicians of all specialties may be the first clinicians facing this diagnostic challenge. Outside the pancreatobiliary system, the head and neck represents the major site of IgG4-RD with variable organ-specific diffuse or mass-forming lesions. In up to 75% of cases, elevated serum IgG4 levels are observed, but this figure possibly underestimates the fraction of seronegative cases, as the disease manifestations may present metachronously with significant intervals. Together with negative serology, this can lead to misdiagnosis of seronegative cases. A standardized nomenclature and diagnostic criteria for IgG4-RD were established in 2012 and revised in 2020 facilitating scientific research and expanding the range of diseases associated with IgG4 abnormalities. In addition to orbital pseudotumor, dacryoadenitis, Riedel thyroiditis, sinonasal manifestations, and rare miscellaneous conditions, IgG4-related sialadenitis is one of the most frequent presentations in the head and neck region. However, controversy still exists regarding the relationship between sialadenitis and IgG4-RD. This review focuses on the clinicopathological features of IgG4-related sialadenitis and its contemporary diagnostic criteria.
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Affiliation(s)
- Agata Czarnywojtek
- Department of Pharmacology, Poznan University of Medical Sciences, 60-806, Poznan, Poland
- Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355, Poznan, Poland
| | - Abbas Agaimy
- Institute of Pathology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg (FAU), 91054, Erlangen, Germany
| | | | - Iain J Nixon
- Department of Otorhinolaryngology Head and Neck Surgery, NHS Lothian, Edinburgh, EH8 9YL, UK
| | - Vincent Vander Poorten
- Otorhinolaryngology-Head and Neck Surgery, KU Leuven University Hospitals, 3000, Leuven, Belgium
- Department of Oncology, Section Head and Neck Oncology, KU Leuven, 3000, Leuven, Belgium
| | - Antti A Mäkitie
- Department of Otorhinolaryngology-Head and Neck Surgery, University of Helsinki and Helsinki University Hospital, and the Research Program in Systems Oncology, Faculty of Medicine, University of Helsinki, 00014, Helsinki, Finland
| | - Mark Zafereo
- Department of Head & Neck Surgery, MD Anderson Cancer Center, Houston, TX, 77005, USA
| | - Ewa Florek
- Laboratory of Environmental Research, Department of Toxicology, Poznan University of Medical Sciences, 60-806, Poznan, Poland.
| | - Nadia Sawicka-Gutaj
- Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355, Poznan, Poland
| | - Marek Ruchała
- Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355, Poznan, Poland
| | - Alfio Ferlito
- International Head and Neck Scientific Group, 35100, Padua, Italy
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13
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Khan F, Shahid J, Saleem A, Khawaja UA, Memon WA, Zafar U, Hameed TA, Abbasher Hussien Mohamed Ahmed K. IgG4-related inflammatory pancreatic head pseudotumor mirrors pancreatic head tumor: A novel case series with a review of the literature. Clin Case Rep 2024; 12:e8467. [PMID: 38317667 PMCID: PMC10839121 DOI: 10.1002/ccr3.8467] [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: 04/04/2023] [Revised: 01/12/2024] [Accepted: 01/19/2024] [Indexed: 02/07/2024] Open
Abstract
Key Clinical Message In this noteworthy case series regarding pancreatic pseudo tumors, we intend to spread knowledge among physicians for the diagnostic and therapeutic approach and eventual disease prognosis. Abstract Inflammatory pseudotumor of pancreatic head greatly mimics pancreatic head tumor. One of them is IgG4-related pancreatic disease, which is commonly mistaken as neoplastic disease on imaging. In our novel case series, we report three cases of IgG4-related pancreatic head pseudotumor with patients ranging from 35 to 72 years of age. Patients presented with jaundice and abdominal pain. Alongside initial laboratory workup, abdominal CTs and serum IgG4 levels were also obtained. Imaging features in conjunction with IgG4 levels confirmed the diagnosis of IgG4-related autoimmune pancreatitis. Pancreatic pseudotumors are notorious for being often reported as real tumors. Through our noteworthy case series, we intend to highlight the imaging features and laboratory markers that are crucial in such cases to avoid invasive procedures.
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Affiliation(s)
- Faheemullah Khan
- Department of RadiologyAga Khan University HospitalKarachiPakistan
| | - Jehanzeb Shahid
- Department of RadiologyAga Khan University HospitalKarachiPakistan
| | - Amna Saleem
- Jinnah Medical and Dental CollegeKarachiPakistan
| | | | | | - Uffan Zafar
- Department of RadiologyAga Khan University HospitalKarachiPakistan
| | - Tariq Abdul Hameed
- Department of RadiologyIndiana University School of MedicineIndianapolisIndianaUSA
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14
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Zhang X, Zeng Z, Tian H, Wang N, Wang Y, Tong J, Chang B, Jin X, Huang D, Wang Y, Cui H, Guan L, Li Y. Clinical features and relapse risks factors of IgG4 related disease: a single-center retrospective study. Clin Exp Med 2023; 23:3527-3538. [PMID: 37392248 DOI: 10.1007/s10238-023-01123-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/07/2023] [Accepted: 06/20/2023] [Indexed: 07/03/2023]
Abstract
OBJECTIVE The aim of this study was to observe the demographic and clinical characteristics of immunoglobulin (Ig) G4-related disease (IgG4-RD). We aimed to compare different treatment methods and to identify the risk factors for non-response and relapse after treatment. METHODS We performed a retrospective study of 201 IgG4-RD patients initially diagnosed and treated at the First Affiliated Hospital of China Medical University from January 2016 to December 2020. Patients' sex, age, clinical manifestations, baseline biochemical values, the number of organs involved, and the type of organ involvement were recorded. All patients received glucocorticoid (GC) monotherapy or GC + immunosuppressant combination therapy. The serum IgG4 concentration as well as the details of clinical response, relapse, and side effects were recorded at 1, 3, 6, and 12 months after treatment. RESULTS The incidence of IgG4-RD was primarily centered in the age group of 50-70 years old, and the proportion of affected male patients increased with age. The most common clinical symptom was swollen glands or eyes (42.79%). The rates of single- and double-organ involvement were 34.83% and 46.27%, respectively. The pancreas (45.77%) was the most frequently involved organ in cases of single-organ involvement, and the pancreas and biliary tract (45.12%) was the most common organ combination in cases of double-organ involvement. Correlation analysis showed that the number of organs involved was positively related to the serum IgG4 concentration (r = 0.161). The effective rate of GC monotherapy was 91.82%, the recurrence rate was 31.46%, and the incidence of adverse reactions was 36.77%. Meanwhile, the effective rate of GC + immunosuppressant combination therapy was 88.52%, the recurrence rate was 19.61%, and the adverse reaction rate was 41.00%. There were no statistically significant differences in response, recurrence, and adverse reactions. The overall response rate within 12 months was 90.64%. Age (< 50 years old) and aorta involvement were significantly associated with non-response. The overall recurrence rate within 12 months was 26.90%. Age (< 50 years old), low serum C4 concentration, a high number of involved organs, and lymph node involvement were significantly associated with recurrence. CONCLUSION The clinical features vary among different age groups and according to gender. The number of organs involved in IgG4-RD is related to the serum IgG4 concentration. Age (< 50 years old), low serum C4 concentration, a high number of involved organs, and lymph node involvement are risk factors for recurrence.
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Affiliation(s)
- Xinhe Zhang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China
| | - Zilu Zeng
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China
| | - Haoyu Tian
- The 3rd Clinical Department, China Medical University, No.77 Puhe Road, Shenyang North New Area, Shenyang, 110122, Liaoning, China
| | - Ningning Wang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China
| | - Ying Wang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China
| | - Jing Tong
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China
| | - Bing Chang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China
| | - Xiuli Jin
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China
| | - Die Huang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China
| | - Yanmeng Wang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China
| | - Huipeng Cui
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China
| | - Lin Guan
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China.
| | - Yiling Li
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, No.155 North Nanjing Street, Heping District, Shenyang, 110001, Liaoning, China.
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15
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Agboola AA, Mohamed KH, Syed M, Shiwlani S, Butt R, Reza RR, Haseeb M, Nasir H. Type 1 Autoimmune Pancreatitis Masquerading as Pancreatic Head Carcinoma. Cureus 2023; 15:e47471. [PMID: 38022068 PMCID: PMC10662655 DOI: 10.7759/cureus.47471] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/22/2023] [Indexed: 12/01/2023] Open
Abstract
Obstructive jaundice is a joint clinical presentation with many etiologies, including pancreatic cancer and autoimmune pancreatitis (AIP). Differentiating between these two conditions is pivotal due to the divergent management approaches and prognoses. In this case report, we present a case of a 49-year-old female patient who presented with weight loss, intermittent chronic abdominal pain, and jaundice. She was initially suspected of having pancreatic cancer because of clinical presentation and imaging findings. However, she was ultimately diagnosed with Type 1 AIP due to histopathology findings and elevated immunoglobulin G4. This case highlights the complexities in diagnosis, the role of advanced imaging techniques and tissue sampling, and the lessons learned regarding managing this challenging clinical scenario.
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Affiliation(s)
| | - Khalid H Mohamed
- Neurology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, GBR
| | - Maria Syed
- Surgery, Aga Khan University Hospital, Karachi, PAK
| | | | - Rowaida Butt
- Family Medicine, Avalon University School of Medicine, Ohio, USA
| | | | - Muhammad Haseeb
- Internal Medicine, Allama Iqbal Medical College, Lahore, PAK
- Internal Medicine, Bahria International Hospital, Lahore, PAK
| | - Hira Nasir
- Internal Medicine, Mayo Hospital, Lahore, PAK
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16
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Gong L, Shu B, Yu F, Zhang X, Chen J, Peng J. Main Diagnostic Criteria Usually Does Not Work for Autoimmune Pancreatitis Wrongly Presuming Malignancy. Gastroenterol Res Pract 2023; 2023:6652881. [PMID: 39291275 PMCID: PMC11407881 DOI: 10.1155/2023/6652881] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/28/2023] [Revised: 07/21/2023] [Accepted: 08/16/2023] [Indexed: 09/19/2024] Open
Abstract
Background Autoimmune pancreatitis (AIP) usually responds dramatically to steroid therapy. Occasionally, however, misdiagnosed patients have undergone pancreaticoduodenectomy. This study is aimed at providing useful information to improve the accuracy of diagnosis before surgery and thus avoid unnecessary resections in patients with AIP. Methods From January 2015 to February 2020, a series of patients were enrolled, having undergone pancreaticoduodenectomy for presumed malignancy. AIP diagnoses were confirmed by postoperative pathology. The demographic and clinical data of the AIP patients were evaluated. The main diagnostic criteria (HISORt, Asian, and ICDC) for AIP were applied to assess whether and how unnecessary surgery could have been avoided. Results A total of 124 cases of pancreaticoduodenectomy were performed for presumed malignancy. Six patients were diagnosed with benign disease and five with AIP. The prevalences of benign disease and AIP were 4.8% and 4%, respectively. Four patients were female and 1 male, with a mean age of 60.0 years old. Jaundice, pain, and weight loss were observed in 100%, 20%, and 40% of AIP patients, respectively. The radiologic features of the AIP patients were a diffusely enlarged gland (40.0%), a focally enlarged gland (40.0%), pancreatic ductal dilatation (60.0%), upstream parenchymal atrophy (20.0%), bile duct thickening (66.0%), and bile duct stricture (40.0%). Based on the diagnostic criteria for AIP, surgery could have been avoided in two cases. Conclusions IgG4 measurement and integrated use of major diagnostic criteria should be emphasized in every patient eligible for pancreaticoduodenectomies.
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Affiliation(s)
- Lei Gong
- Department of Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Bin Shu
- Center of Hepatopancreatobiliary Diseases, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing 102218, China
| | - Fei Yu
- Center of Hepatopancreatobiliary Diseases, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing 102218, China
| | - Xinjing Zhang
- Department of Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Jianfei Chen
- Department of Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Jirun Peng
- Department of Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
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17
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Álvarez García M, Álvarez Torices JC, Díez Liébana MJ, de la Fuente Olmos MR. Thoracoabdominal manifestations of immunoglobulin G4-related disease. RADIOLOGIA 2023; 65:165-175. [PMID: 37059581 DOI: 10.1016/j.rxeng.2022.06.005] [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: 02/16/2022] [Accepted: 06/02/2022] [Indexed: 04/16/2023]
Abstract
Immunoglobulin G4-related disease (IgG4-RD) comprises a group of immune-mediated diseases that, until recently, were considered separate entities. These entities have a similar clinical presentation, serological findings, and pathogenesis, so they are currently considered a single multisystemic disease. The common characteristic is the infiltration of involved tissues by plasma cells and lymphocytes that are positive for IgG4. Three major criteria have been proposed for the diagnosis of IgG4-RD: clinical, laboratory, and histological. The pancreas is the organ that is most affected by IgG4-RD, which can simulate a tumor. In this respect, a series of signs could help us suspect that the pancreatic findings do not reflect a tumor (halo sign, duct-penetrating sign, absence of vascular invasion, etc.). The differential diagnosis is important to avoid unnecessary surgical interventions.
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Affiliation(s)
- M Álvarez García
- Servicio de Radiodiagnóstico del Hospital Universitario Río Hortega, Valladolid, Spain.
| | - J C Álvarez Torices
- Servicio de Atención Primaria, Centro de Salud Antonio Gutiérrez, León, Spain
| | - M J Díez Liébana
- Departamento de Ciencias Biomédicas, Universidad de León, León, Spain
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18
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Zhang XD, Zhang Y, Zhao YZ, Zhou CH, Zou DW. Autoimmune pancreatitis: A bibliometric analysis from 2002 to 2022. Front Immunol 2023; 14:1135096. [PMID: 36911675 PMCID: PMC9992966 DOI: 10.3389/fimmu.2023.1135096] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2022] [Accepted: 02/09/2023] [Indexed: 02/24/2023] Open
Abstract
Background/Objectives Autoimmune pancreatitis (AIP) is a distinct form of pancreatic inflammatory disease that responds well to glucocorticoid therapy. Knowledge on AIP has rapidly evolved over the past two decades. Based on bibliometric analysis, this study aimed to assess the research status of AIP over the past two decades and determine the research focus and emerging topics. Methods AIP-related publications published between January 1, 2002, and June 6, 2022, were retrieved from the Web of Science Core Collection. Bibliometric data were analyzed using HisCite, VOSviewer, CiteSpace, and bibliometrix package. Annual output, leading countries/regions, active institutions and authors, core journals and references, and keywords of AIP were evaluated. Results Overall, 1,772 publications were retrieved from 501 journals by 6,767 authors from 63 countries/regions. Japan published articles on AIP the most (n=728, 41.1%), followed by the United States (n=336, 19%), Germany (n=147, 8.3%), China (n=127, 7%), and Italy (n=107, 6%). The top three most prolific authors were Terumi Kamisawa from Tokyo Metropolitan Komagome Hospital (n=117), Kazuichi Okazaki from Kansai Medical University (n=103), and Shigeyuki Kawa from Matsumoto Dental University (n=94). Pancreas was the most productive journal regarding AIP research (n=95), followed by the Journal of Gastroenterology (n=67), Internal Medicine (n=66), Pancreatology (n=63), and World Journal of Gastroenterology (n=62). "Diagnosis" was the most mentioned keyword. "Risk," "malignancy," "outcome," "22-gauge needle," and "fine-needle aspiration" were recognized as emerging topics. Conclusion Japan was the leading country in AIP research. Research papers were mainly published in specialized journals. Diagnosis was the research focus. Long-term outcomes and pancreatic tissue acquisition were recognized as research frontiers for AIP.
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Affiliation(s)
- Xian-Da Zhang
- Department of Gastroenterology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Yao Zhang
- Department of Gastroenterology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Yi-Zhou Zhao
- Department of Gastroenterology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Chun-Hua Zhou
- Department of Gastroenterology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Duo-Wu Zou
- Department of Gastroenterology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
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19
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Mack S, Flattet Y, Bichard P, Frossard JL. Recent advances in the management of autoimmune pancreatitis in the era of artificial intelligence. World J Gastroenterol 2022; 28:6867-6874. [PMID: 36632320 PMCID: PMC9827582 DOI: 10.3748/wjg.v28.i48.6867] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/18/2022] [Revised: 10/31/2022] [Accepted: 11/16/2022] [Indexed: 12/26/2022] Open
Abstract
Autoimmune pancreatitis (AIP) is a type of immune-mediated pancreatitis subdivided into two subtypes, type 1 and type 2 AIP. Furthermore, type 1 AIP is considered to be the pancreatic manifestation of the immunoglobulin G4 (IgG4)-related disease. Nowadays, AIP is increasingly researched and recognized, although its diagnosis represents a challenge for several reasons: False positive ultrasound-guided cytological samples for a neoplastic process, difficult to interpret levels of IgG4, the absence of biological markers to diagnose type 2 AIP, and the challenging clinical identification of atypical forms. Furthermore, 60% and 78% of type 1 and type 2 AIP, respectively, are retrospectively diagnosed on surgical specimens of resected pancreas for suspected cancer. As distinguishing AIP from pancreatic ductal adenocarcinoma can be challenging, obtaining a definitive diagnosis can therefore prove difficult, since endoscopic ultrasound fine-needle aspiration or biopsy of the pancreas are suboptimal. This paper focuses on recent innovations in the management of AIP with regard to the use of artificial intelligence, new serum markers, and new therapeutic approaches, while it also outlines the current management recommendations. A better knowledge of AIP can reduce the recourse to surgery and avoid its overuse, although such an approach requires close collaboration between gastroenterologists, surgeons and radiologists. Better knowledge on AIP and IgG4-related disease remains necessary to diagnose and manage patients.
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Affiliation(s)
- Sahar Mack
- Division of Gastroenterology, Department of Medical Specialties, University Hospital of Geneva, Geneva 1205, Switzerland
| | - Yves Flattet
- Division of Gastroenterology, Department of Medical Specialties, University Hospital of Geneva, Geneva 1205, Switzerland
| | - Philippe Bichard
- Division of Gastroenterology, Department of Medical Specialties, University Hospital of Geneva, Geneva 1205, Switzerland
| | - Jean Louis Frossard
- Division of Gastroenterology, Department of Medical Specialties, University Hospital of Geneva, Geneva 1205, Switzerland
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20
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Imai S, Tahara N, Igata S, Tahara A, Bekki M, Sugiyama Y, Maeda-Ogata S, Honda A, Otsuka H, Ushijima T, Okabe Y, Kaida H, Abe T, Tanaka H, Fukumoto Y, Tayama E. Vascular/perivascular inflammation in IgG4-related disease. J Nucl Cardiol 2022; 29:2920-2933. [PMID: 34704218 DOI: 10.1007/s12350-021-02812-6] [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: 01/14/2021] [Accepted: 09/12/2021] [Indexed: 01/29/2023]
Abstract
BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is characterized by the infiltration of IgG4-positive plasma cells and fibrosclerotic inflammation in multiple organs. Although vascular complications are present in some patients with IgG4-RD, vascular and/or perivascular inflammatory activity compared to control subjects remains unknown. This study sought to investigate vascular/perivascular inflammation in IgG4-RD patients compared to control subjects using 18F-fluorodeoxyglucose-positron emission tomography combined with computed tomography (FDG-PET/CT). METHODS We examined 37 consecutive patients diagnosed as IgG4-RD (29 males, mean age of 64.3 ± 8.3 years old), who underwent FDG-PET/CT. Thirty-seven age- and gender-matched subjects without IgG4-RD were employed as controls. Vascular/perivascular inflammation was quantified by blood-normalized standardized uptake value, known as a target-to-background ratio (TBR). RESULTS All IgG4-RD patients presented with multiple region involvements. Twelve (32.4%) of the IgG4-RD patients had vascular complications, all of which appeared in the abdominal aorta. IgG4-RD patients had significantly higher TBR values in the descending aorta, abdominal aorta, and common iliac artery than control subjects. Also, IgG4-RD patients with vascular complication exhibited higher TBR values in the infra-renal aorta and common iliac artery than those without vascular complication. CONCLUSIONS We found that vascular FDG activity is significantly elevated in IgG4-RD patients regardless of vascular complication than control subjects. FDG-PET/CT is a useful modality for assessing vascular/perivascular inflammation, which may contribute vascular complication in IgG4-RD patients.
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Affiliation(s)
- Shinichi Imai
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Nobuhiro Tahara
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan.
| | - Sachiyo Igata
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Atsuko Tahara
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Munehisa Bekki
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Yoichi Sugiyama
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Shoko Maeda-Ogata
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Akihiro Honda
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Hiroyuki Otsuka
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Tomoyuki Ushijima
- Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Kurume, Japan
| | - Yoshinobu Okabe
- Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Kurume, Japan
| | - Hayato Kaida
- Department of Radiology, Kindai University Faculty of Medicine, Osakasayama, Japan
| | - Toshi Abe
- Department of Radiology, Kurume University School of Medicine, Kurume, Japan
| | - Hiroyuki Tanaka
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Yoshihiro Fukumoto
- Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Eiki Tayama
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
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21
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Nista EC, De Lucia SS, Manilla V, Schepis T, Pellegrino A, Ojetti V, Pignataro G, Zileri dal Verme L, Franceschi F, Gasbarrini A, Candelli M. Autoimmune Pancreatitis: From Pathogenesis to Treatment. Int J Mol Sci 2022; 23:ijms232012667. [PMID: 36293522 PMCID: PMC9604056 DOI: 10.3390/ijms232012667] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2022] [Revised: 10/14/2022] [Accepted: 10/18/2022] [Indexed: 11/05/2022] Open
Abstract
Autoimmune pancreatitis (AIP) is a rare disease. The diagnosis of AIP is difficult and should be made by a comprehensive evaluation of clinical, radiological, serological, and pathological findings. Two different types of AIP have been identified: autoimmune pancreatitis type 1 (AIP-1), which is considered a pancreatic manifestation of multiorgan disease related to IgG4, and autoimmune pancreatitis type 2 (AIP-2), which is considered a pancreas-specific disease not related to IgG4. Although the pathophysiological conditions seem to differ between type 1 and type 2 pancreatitis, both respond well to steroid medications. In this review, we focused on the pathogenesis of the disease to develop a tool that could facilitate diagnosis and lead to the discovery of new therapeutic strategies to combat autoimmune pancreatitis and its relapses. The standard therapy for AIP is oral administration of corticosteroids. Rituximab (RTX) has also been proposed for induction of remission and maintenance therapy in relapsing AIP-1. In selected patients, immunomodulators such as azathioprine are used to maintain remission. The strength of this review, compared with previous studies, is that it focuses on the clear difference between the two types of autoimmune pancreatitis with a clearly delineated and separate pathogenesis. In addition, the review also considers various therapeutic options, including biologic drugs, such as anti-tumor necrosis factor (TNF) therapy, a well-tolerated and effective second-line therapy for AIP type 2 relapses or steroid dependence. Other biologic therapies are also being explored that could provide a useful therapeutic alternative to corticosteroids and immunosuppressants, which are poorly tolerated due to significant side effects.
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Affiliation(s)
- Enrico Celestino Nista
- Department of Medical and Surgical Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
| | - Sara Sofia De Lucia
- Department of Medical and Surgical Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
| | - Vittoria Manilla
- Department of Medical and Surgical Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
| | - Tommaso Schepis
- Department of Medical and Surgical Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
| | - Antonio Pellegrino
- Department of Medical and Surgical Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
| | - Veronica Ojetti
- Department of Emergency, Anesthesiological, and Reanimation Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
| | - Giulia Pignataro
- Department of Emergency, Anesthesiological, and Reanimation Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
| | - Lorenzo Zileri dal Verme
- Department of Medical and Surgical Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
| | - Francesco Franceschi
- Department of Emergency, Anesthesiological, and Reanimation Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
| | - Antonio Gasbarrini
- Department of Medical and Surgical Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
| | - Marcello Candelli
- Department of Emergency, Anesthesiological, and Reanimation Sciences, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy
- Correspondence:
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22
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Moon SH, Kim MH. Autoimmune Pancreatitis and Immunoglobulin G4-related Sclerosing Cholangitis: Past, Present, and Future. THE KOREAN JOURNAL OF GASTROENTEROLOGY = TAEHAN SOHWAGI HAKHOE CHI 2022; 80:107-114. [PMID: 36156034 DOI: 10.4166/kjg.2022.102] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/20/2022] [Revised: 08/15/2022] [Accepted: 08/16/2022] [Indexed: 06/16/2023]
Abstract
The emergence of glucocorticoid-responsive autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC), a new disease entity, has attracted considerable interest within the international gastroenterology community. The typical manifestations of AIP/IgG4-SC are obstructive jaundice and pancreatic enlargement in the elderly, which may mimic the presentations of pancreatobiliary malignancies. The timely diagnosis of AIP/IgG4-SC can lead to adequate glucocorticoid treatment, whereas a misdiagnosis can result in unnecessary major surgery. The diagnostic criteria used to diagnose AIP include several cardinal features of AIP that can be detected via pancreatic parenchymal imaging, ductal imaging, serum IgG4 levels, histopathology, other organ involvement, and response to glucocorticoid therapy. The differential diagnosis of AIP/IgG4-SC may include pancreatobiliary malignancies and primary sclerosing cholangitis. Although most patients with AIP/IgG4-SC respond well to glucocorticoid therapy, there is a frequent relapse of the disease in the long term. This review describes the evolution of the concept of AIP and IgG4-related disease, including the development of diagnostic criteria, discusses the current practice for diagnosis and treatment, and suggests prospects for research.
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Affiliation(s)
- Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Anyang, Korea
| | - Myung-Hwan Kim
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
- Department of Internal Medicine, Hanyang University Changwon Hanmaeum Hospital, Changwon, Korea
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23
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Manifestaciones toracoabdominales de las enfermedades relacionadas con la inmunoglobulina G4. RADIOLOGIA 2022. [DOI: 10.1016/j.rx.2022.06.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
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24
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Correlation of Autoimmune Pancreatitis and Malignancy: Systematic Review and Meta-Analysis. Dig Dis Sci 2022; 67:3252-3264. [PMID: 34297267 DOI: 10.1007/s10620-021-07179-9] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/22/2021] [Accepted: 07/14/2021] [Indexed: 12/19/2022]
Abstract
BACKGROUND There is conflicting evidence regarding autoimmune pancreatitis (AIP) association with pancreatic and non-pancreatic cancers. Literature lacks data on overall prevalence of malignancies in autoimmune pancreatitis. AIM Given the lack of definite evidence, we aimed to pool and summarize data from available literature regarding prevalence of different malignancies in AIP. METHODS We conducted a systematic search of MEDLINE, EMBASE, Cochrane Register of Controlled Trials, and Web of Science through February 16, 2021, to include observational studies assessing the incidence of cancer in AIP. We used the DerSimonian-Laird method with random effects for meta-analysis. Pooled prevalence, 95% confidence interval (CI), and I2 statistic are reported. RESULTS A total of 17 studies with 2746 patients were included assessing the prevalence of cancer in AIP. The overall prevalence of cancer in AIP was 9.6% [95% confidence interval (CI), 5.7-13.5%]. The cancers with the highest prevalence in AIP population were gastric and colorectal cancer, with prevalence of 1.3% (95% CI, 0.5-2.1%) and 1.2% (95% CI, 0.6-1.8%), respectively. CONCLUSION We demonstrate the prevalence of different cancers in AIP. Inflammatory surge in AIP and subsequent carcinogenesis is one explanation for this association. Moreover, AIP can be a paraneoplastic syndrome manifestation of malignancies.
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25
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Sumimoto K, Uchida K, Ikeura T, Hirano K, Yamamoto M, Takahashi H, Nishino T, Mizushima I, Kawano M, Kamisawa T, Saeki T, Maguchi H, Ushijima T, Shiokawa M, Seno H, Goto H, Nakamura S, Okazaki K. Nationwide epidemiological survey of immunoglobulin G4-related disease with malignancy in Japan. J Gastroenterol Hepatol 2022; 37:1022-1033. [PMID: 35229347 DOI: 10.1111/jgh.15809] [Citation(s) in RCA: 12] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/22/2021] [Revised: 01/24/2022] [Accepted: 02/08/2022] [Indexed: 01/11/2023]
Abstract
BACKGROUND AND AIM To clarify the clinicoepidemiological characteristics of immunoglobulin G4 (IgG4)-related disease (IgG4-RD) with malignancy, a nationwide epidemiological survey was conducted. METHODS Immunoglobulin G4-related disease patients with malignancy who had visited selected hospitals in Japan were surveyed. The study consisted of two stages: the number of IgG4-RD patients with malignancy was estimated by the first questionnaire and their clinicoepidemiological characteristics were assessed by the second questionnaire. RESULTS The frequencies of autoimmune pancreatitis (AIP), IgG4-related sialadenitis, IgG4-related eye disease, IgG4-related kidney disease, and IgG4-related retroperitoneal fibrosis were 44.7%, 20.8%, 14.0%, 5.16%, and 5.12%, respectively. The overall prevalence of malignant disease in IgG4-RD cases was estimated to be 10 900 per 100 000 cases, which was significantly higher than that of malignant disease in the general population. The prevalence of malignant lymphoma in IgG4-RD cases was the highest and was estimated to be 1985 per 100 000 cases. IgG4-related kidney disease had the highest frequency of malignant disease (17.1%). In data from 200 patients, 61 (30.5%) cases of cancer were found 2 years or more before the IgG4-RD diagnosis, 92 cases (46%) during the 1 year preceding or following IgG4-RD diagnosis, and 62 cases of cancer (31%) 2 or more years following IgG4-RD diagnosis. CONCLUSIONS The nationwide survey for IgG4-RD with malignancy in Japan showed that IgG4-RD may be related with malignant diseases.
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Affiliation(s)
- Kimi Sumimoto
- The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
| | - Kazushige Uchida
- Department of Gastroenterology and Hepatology, Kochi Medical School, Kochi University, Nankoku, Japan
| | - Tsukasa Ikeura
- The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
| | - Kenji Hirano
- Department of Gastroenterology, Tokyo Takanawa Hospital, Tokyo, Japan
| | - Motohisa Yamamoto
- The Institute of Medical Science, The University of Tokyo, Tokyo, Japan
| | - Hiroki Takahashi
- Department of Rheumatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Takayoshi Nishino
- Department of Gastroenterology, Tokyo Women's Medical University Yachiyo Medical Center, Tokyo, Japan
| | - Ichiro Mizushima
- Division of Rheumatology, Department of Internal Medicine, Kanazawa University Hospital, Kanazawa, Japan
| | - Mitsuhiro Kawano
- Division of Rheumatology, Department of Internal Medicine, Kanazawa University Hospital, Kanazawa, Japan
| | | | - Takako Saeki
- Department of Internal Medicine, Nagaoka Red Cross Hospital, Niigata, Japan
| | - Hiroyuki Maguchi
- Education and Research Center, Teine-Keijinkai Hospital, Sapporo, Hokkaido, Japan
| | - Tomoyuki Ushijima
- Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Fukuoka, Japan
| | - Masahiro Shiokawa
- Division of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, Kyoto, Japan
| | - Hiroshi Seno
- Division of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, Kyoto, Japan
| | - Hiroshi Goto
- Department of Ophthalmology, Tokyo Medical University, Tokyo, Japan
| | - Seiji Nakamura
- Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, Fukuoka, Japan
| | - Kazuichi Okazaki
- The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
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26
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Lanzillotta M, Tacelli M, Falconi M, Arcidiacono PG, Capurso G, Della-Torre E. Incidence of endocrine and exocrine insufficiency in patients with autoimmune pancreatitis at diagnosis and after treatment: a systematic review and meta-analysis. Eur J Intern Med 2022; 100:83-93. [PMID: 35367110 DOI: 10.1016/j.ejim.2022.03.014] [Citation(s) in RCA: 15] [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: 01/14/2022] [Revised: 03/09/2022] [Accepted: 03/11/2022] [Indexed: 11/03/2022]
Abstract
BACKGROUND Autoimmune pancreatitis (AIP) is a rare form of pancreatitis that may lead to endocrine and exocrine insufficiency if left untreated. AIP clinically responds to glucocorticoids (GCs) therapy, but multiple GCs courses are often required to maintain remission with detrimental effects on glycaemic control. OBJECTIVE In this systematic review and meta-analysis, we aimed to assess the rate of endocrine and of exocrine insufficiency at diagnosis and at follow up in patients with AIP as well as the impact of GC therapy on pancreatic function in the long-term. METHODS The MEDLINE, SCOPUS, and EMBASE databases were searched from inception to August 2021 to identify studies reporting data on endocrine and exocrine insufficiency in patients with AIP. Pooled events were calculated using a random-effect model and expressed in terms of pooled prevalence rates. RESULTS A total of 6522 AIP patients and sixty-two studies were included in the analysis. The pooled estimate rate for the overall prevalence of diabetes in AIP at baseline was 37% (95% CI 32-42, I2 96%). The pooled prevalence rate of exocrine insufficiency was 45% (95%CI 32.9-57.4; I2 97%). The pooled estimate rate of diabetes at follow-up was 44% (95%CI 26.1-62.4) in studies where GCs were given to 100% of patients and 42% (95%CI 30.6-52.9) in studies where GCs were given to less than 100% of patients. CONCLUSION A large proportion of patients with AIP displays concomitant exocrine and endocrine insufficiency at the time of diagnosis. The incidence of diabetes at the longest available follow up tends to increase in patients treated with GCs.
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Affiliation(s)
- Marco Lanzillotta
- Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy.
| | - Matteo Tacelli
- Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy; Pancreato-Biliary Endoscopy and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Massimo Falconi
- Division of Pancreatic Surgery and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Paolo Giorgio Arcidiacono
- Pancreato-Biliary Endoscopy and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Gabriele Capurso
- Pancreato-Biliary Endoscopy and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Emanuel Della-Torre
- Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy
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27
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Beyer G, Hoffmeister A, Michl P, Gress TM, Huber W, Algül H, Neesse A, Meining A, Seufferlein TW, Rosendahl J, Kahl S, Keller J, Werner J, Friess H, Bufler P, Löhr MJ, Schneider A, Lynen Jansen P, Esposito I, Grenacher L, Mössner J, Lerch MM, Mayerle J. S3-Leitlinie Pankreatitis – Leitlinie der Deutschen Gesellschaft für Gastroenterologie, Verdauungs- und Stoffwechselkrankheiten (DGVS) – September 2021 – AWMF Registernummer 021-003. ZEITSCHRIFT FUR GASTROENTEROLOGIE 2022; 60:419-521. [PMID: 35263785 DOI: 10.1055/a-1735-3864] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Affiliation(s)
- Georg Beyer
- Medizinische Klinik und Poliklinik II, LMU Klinikum, Ludwig-Maximilians-Universität München, Deutschland
| | - Albrecht Hoffmeister
- Bereich Gastroenterologie, Klinik und Poliklinik für Onkologie, Gastroenterologie, Hepatologie Pneumologie und Infektiologie, Universitätsklinikum Leipzig, Deutschland
| | - Patrick Michl
- Universitätsklinik u. Poliklinik Innere Medizin I mit Schwerpunkt Gastroenterologie, Universitätsklinikum Halle, Deutschland
| | - Thomas Mathias Gress
- Klinik für Gastroenterologie und Endokrinologie, Universitätsklinikum Gießen und Marburg, Deutschland
| | - Wolfgang Huber
- Comprehensive Cancer Center München TUM, II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technische Universität München, München, Deutschland
| | - Hana Algül
- Comprehensive Cancer Center München TUM, II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technische Universität München, München, Deutschland
| | - Albrecht Neesse
- Klinik für Gastroenterologie, gastrointestinale Onkologie und Endokrinologie, Universitätsmedizin Göttingen, Deutschland
| | - Alexander Meining
- Medizinische Klinik und Poliklinik II Gastroenterologie und Hepatologie, Universitätsklinikum Würzburg, Deutschland
| | | | - Jonas Rosendahl
- Universitätsklinik u. Poliklinik Innere Medizin I mit Schwerpunkt Gastroenterologie, Universitätsklinikum Halle, Deutschland
| | - Stefan Kahl
- Klinik für Innere Medizin m. Schwerpkt. Gastro./Hämat./Onko./Nephro., DRK Kliniken Berlin Köpenick, Deutschland
| | - Jutta Keller
- Medizinische Klinik, Israelitisches Krankenhaus, Hamburg, Deutschland
| | - Jens Werner
- Klinik für Allgemeine, Viszeral-, Transplantations-, Gefäß- und Thoraxchirurgie, Universitätsklinikum München, Deutschland
| | - Helmut Friess
- Klinik und Poliklinik für Chirurgie, Klinikum rechts der Isar, München, Deutschland
| | - Philip Bufler
- Klinik für Pädiatrie m. S. Gastroenterologie, Nephrologie und Stoffwechselmedizin, Charité Campus Virchow-Klinikum - Universitätsmedizin Berlin, Deutschland
| | - Matthias J Löhr
- Department of Gastroenterology, Karolinska, Universitetssjukhuset, Stockholm, Schweden
| | - Alexander Schneider
- Klinik für Gastroenterologie und Hepatologie, Klinikum Bad Hersfeld, Deutschland
| | - Petra Lynen Jansen
- Deutsche Gesellschaft für Gastroenterologie, Verdauungs- und Stoffwechselkrankheiten (DGVS), Berlin, Deutschland
| | - Irene Esposito
- Pathologisches Institut, Heinrich-Heine-Universität und Universitätsklinikum Duesseldorf, Duesseldorf, Deutschland
| | - Lars Grenacher
- Conradia Radiologie München Schwabing, München, Deutschland
| | - Joachim Mössner
- Bereich Gastroenterologie, Klinik und Poliklinik für Onkologie, Gastroenterologie, Hepatologie Pneumologie und Infektiologie, Universitätsklinikum Leipzig, Deutschland
| | - Markus M Lerch
- Klinik für Innere Medizin A, Universitätsmedizin Greifswald, Deutschland.,Klinikum der Ludwig-Maximilians-Universität (LMU) München, Deutschland
| | - Julia Mayerle
- Medizinische Klinik und Poliklinik II, LMU Klinikum, Ludwig-Maximilians-Universität München, Deutschland
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28
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Kanno A, Miwata T, Nagai H, Ikeda E, Ando K, Kawasaki Y, Tada Y, Yokoyama K, Tamada K, Fukushima N, Kawarai Lefor A, Yamamoto H. Endoscopic ultrasound-guided pancreatic sampling for the histopathological diagnosis of autoimmune pancreatitis. Dig Endosc 2022; 34:420-427. [PMID: 34233051 DOI: 10.1111/den.14076] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/30/2021] [Revised: 06/21/2021] [Accepted: 07/06/2021] [Indexed: 12/25/2022]
Abstract
Autoimmune pancreatitis (AIP), which is characterized by pancreatic enlargement and irregular narrowing of the main pancreatic duct, is difficult to differentiate from malignancy. The irregular narrowing of the pancreatic duct, which can be detected via endoscopic retrograde cholangiopancreatography, is a characteristic feature of AIP; however, distinguishing between localized AIP and pancreatic cancer based on pancreatic duct imaging is difficult. This study overviews the efficacy of endoscopic ultrasound (EUS)-guided pancreatic sampling for the histopathological diagnosis of AIP. Recent enhancements in needle biopsy methodologies and technologies have contributed to improvement in the diagnostic efficacy of this technique. The guidance provided in this study for the histological diagnosis of AIP is anticipated to further advance in the histopathological diagnosis of AIP using EUS-guided pancreatic sampling.
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Affiliation(s)
- Atsushi Kanno
- Department of, Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan
| | - Tetsurou Miwata
- Department of, Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan
| | - Hiroki Nagai
- Department of, Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan
| | - Eriko Ikeda
- Department of, Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan.,Pathology, Jichi Medical University, Tochigi, Japan
| | - Kozue Ando
- Department of, Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan.,Pathology, Jichi Medical University, Tochigi, Japan
| | - Yuki Kawasaki
- Department of, Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan
| | - Yamato Tada
- Department of, Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan
| | - Kensuke Yokoyama
- Department of, Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan
| | - Kiichi Tamada
- Department of, Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan
| | | | | | - Hironori Yamamoto
- Department of, Medicine, Division of Gastroenterology, Jichi Medical University, Tochigi, Japan
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29
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Agarwal KK, Jassal R, Browne A, Hossain M, Akhtar R. Autoimmune Pancreatitis Masquerading as Pancreatic Cancer: A Case Report and Literature Review. Cureus 2022; 14:e21900. [PMID: 35265424 PMCID: PMC8898479 DOI: 10.7759/cureus.21900] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/03/2022] [Indexed: 11/05/2022] Open
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30
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Maslinska M, Dmowska-Chalaba J, Jakubaszek M. The Role of IgG4 in Autoimmunity and Rheumatic Diseases. Front Immunol 2022; 12:787422. [PMID: 35145508 PMCID: PMC8821096 DOI: 10.3389/fimmu.2021.787422] [Citation(s) in RCA: 17] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2021] [Accepted: 12/23/2021] [Indexed: 12/15/2022] Open
Abstract
The distinguishing of the IgG4-related disease (IgG4-RD) from among other rheumatic diseases has brought attention to the IgG4 subclass of immunoglobulins. It is the least numerous subclass among immunoglobulins G. In general, IgG4 is considered to be non-inflammatory and tolerance inducing, due to its unique structure. However, in IgG4-RD this antibody plays a pathogenic role in activation of the fibrinogenesis and of the inflammatory process; there are also suggestions that it may be a marker of an abnormal inflammatory response. The importance of IgG4 for the pathogenesis of allergic diseases, with a vital role of its ratio to immunoglobulin E (IgE/IgG4 ratio), has been known for years. The role of IgG4 in the course and pathogenesis of rheumatic diseases is still being researched and is not yet fully understood. Increased IgG4 levels have been revealed in rheumatoid arthritis, although no clear link between this phenomenon and disease activity has been demonstrated. There are articles on the potential importance of IgG4 concentration (of both elevated and decreased serum levels) in Sjogren’s syndrome. Additionally, anti-nuclear IgG4 antibody significant titers have been detected in SLE patients, and it has been suggested that the effect of these antibodies on complement consumption and the production of proinflammatory cytokines may play a role in inhibiting the progression of SLE. IgG4 plays a role in autoimmune diseases other than rheumatic diseases, such as pemphigus, bullous pemphigoid, idiopathic membranous glomerulonephritis, or myasthenia gravis, but also in helmints infections. Research shows the importance of IgG4 in malignancy of neoplasms. Melanoma cells are known to stimulate IgG4 production through a modified Th2-based inflammatory response. The role of this immunoglobulin in cholangiocarcinoma is also considered as possible. The aim of this review article is to discuss the current knowledge of IgG4 not only from the perspective of the IgG4-RD but also from a point of view of other autoimmune diseases with particular emphasis on rheumatic diseases.
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31
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Amendment of the Japanese consensus guidelines for autoimmune pancreatitis, 2020. J Gastroenterol 2022; 57:225-245. [PMID: 35192048 PMCID: PMC8938398 DOI: 10.1007/s00535-022-01857-9] [Citation(s) in RCA: 47] [Impact Index Per Article: 15.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/03/2021] [Accepted: 01/28/2022] [Indexed: 02/04/2023]
Abstract
In response to the latest knowledge and the amendment of the Japanese diagnostic criteria for autoimmune pancreatitis (AIP) in 2018, the Japanese consensus guidelines for managing AIP in 2013 were required to be revised. Three committees [the professional committee for developing clinical questions (CQs) and statements by Japanese specialists; the expert panelist committee for rating statements by the modified Delphi method; and the evaluating committee of moderators] were organized. Twenty specialists in AIP extracted the specific clinical statements from a total of 5218 articles (1963-2019) from a search in PubMed and the Cochrane Library. The professional committee made 14, 9, 5, and 11 CQs and statements for the current concept and diagnosis, extra-pancreatic lesions, differential diagnosis, and treatment, respectively. The expert panelists regarded the statements as valid after a two-round modified Delphi approach with individually rating these clinical statements, in which a clinical statement receiving a median score greater than 7 on a 9-point scale from the panel was regarded as valid. After evaluation by the moderators, the amendment of the Japanese consensus guidelines for AIP has been proposed in 2020.
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IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease. Can J Gastroenterol Hepatol 2021; 2021:1959832. [PMID: 34970512 PMCID: PMC8714375 DOI: 10.1155/2021/1959832] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/10/2021] [Revised: 11/08/2021] [Accepted: 12/01/2021] [Indexed: 11/30/2022] Open
Abstract
IgG4-related sclerosing cholangitis, a biliary manifestation of an IgG4-related disease, belongs to the spectrum of sclerosing cholangiopathies which result in biliary stenosis. It presents with signs of cholestasis and during differential diagnosis it should be distinguished from cholangiocarcinoma or from other forms of sclerosing cholangitis (primary and secondary sclerosing cholangitis). Despite increasing information and recently established diagnostic criteria, IgG4-related sclerosing cholangitis remains underdiagnosed in routine clinical practice. The diagnosis is based on a combination of the clinical picture, laboratory parameters, histological findings, and a cholangiogram. Increased serum IgG4 levels are nonspecific but are indeed a part of the diagnostic criteria proposed by the Japan Biliary Association and the HISORt criteria for IgG4-SC. High serum IgG4 retains clinical utility depending on the magnitude of elevation. Approximately 90% of patients have concomitant autoimmune pancreatitis, while 10% present with isolated biliary involvement only. About 26% of patients have other organ involvement, such as IgG4-related dacryoadenitis/sialadenitis, IgG4-related retroperitoneal fibrosis, or IgG4-related renal lesions. A full-blown histological finding characterized by IgG4-enriched lymphoplasmacytic infiltrates, obliterative phlebitis, and storiform fibrosis is difficult to capture in practice because of its subepithelial localization. However, the histological yield is increased by immunohistochemistry, with evidence of IgG4-positive plasma cells. Based on a cholangiogram, IgG-4 related sclerosing cholangitis is classified into four subtypes according to the localization of stenoses. The first-line treatment is corticosteroids. The aim of the initial treatment is to induce clinical and laboratory remission and cholangiogram normalization. Even though 30% of patients have a recurrent course, in the literature data, there is no consensus on chronic immunosuppressive maintenance therapy. The disease has a good prognosis when diagnosed early.
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Yoon SB, Moon SH, Song TJ, Kim JH, Kim MH. Endoscopic ultrasound-guided fine needle aspiration versus biopsy for diagnosis of autoimmune pancreatitis: Systematic review and comparative meta-analysis. Dig Endosc 2021; 33:1024-1033. [PMID: 33030283 DOI: 10.1111/den.13866] [Citation(s) in RCA: 26] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/22/2020] [Accepted: 10/01/2020] [Indexed: 12/17/2022]
Abstract
BACKGROUND Endoscopic ultrasound (EUS) is recommended for guiding the acquisition of pancreatic tissue in patients with suspected autoimmune pancreatitis (AIP). Data comparing EUS-guided fine needle aspiration (FNA) and fine needle biopsy (FNB) sampling in the diagnosis of AIP are limited. METHODS A comprehensive literature search of the PubMed, EMBASE, and Ovid MEDLINE databases was conducted until April 2020. The pooled rates of diagnostic yield for the histologic criteria of AIP, histologic tissue procurement, and adverse events were compared between FNA and FNB. Diagnostic yields were also compared between 19 gauge (G) and 22G needles. RESULTS This meta-analysis included nine studies comprising 309 patients with AIP who underwent FNA and seven studies comprising 131 patients who underwent FNB. The pooled diagnostic yields for level 1 or 2 histology criteria of AIP were 55.8% (95% confidence interval (CI) 37.0-73.9%, I2 = 91.1) for FNA and 87.2% (95% CI 68.8-98.1%, I2 = 69.4) for FNB (P = 0.030). The pooled histologic procurement rates for FNA and FNB were 91.3% (95% CI, 84.9-97.6%, I2 = 82.9) and 87.0% (95% CI, 77.8-96.1%, I2 = 40.0), respectively (P = 0.501). Adverse events were comparable between two groups. When analyzed by needle size, the diagnostic yield was better with a 19G needle than with a 22G needle (88.9% vs. 60.6%, P = 0.023). CONCLUSIONS The diagnostic yield may be better with FNB needles than with FNA needles for the diagnosis of AIP, despite the similar rate of histologic tissue procurement. A quantitative definition for the histologic sample adequacy for AIP may be warranted.
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Affiliation(s)
- Seung Bae Yoon
- Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, South Korea
| | - Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea.,Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea
| | - Tae Jun Song
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
| | - Jong Hyeok Kim
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea.,Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea
| | - Myung-Hwan Kim
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
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Kanno A, Tamada K, Fukushima N, Lefor AK, Yamamoto H. Endoscopic ultrasound-guided tissue acquisition for the histopathological diagnosis of autoimmune pancreatitis. J Med Ultrason (2001) 2021; 48:555-563. [PMID: 34669069 DOI: 10.1007/s10396-021-01144-9] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/14/2021] [Accepted: 07/28/2021] [Indexed: 01/22/2023]
Abstract
Autoimmune pancreatitis (AIP) is a disease concept that originated in Japan. It is characterized by diffuse pancreatic enlargement and irregular narrowing of the main pancreatic duct. Although the usefulness of the histological diagnosis of AIP using endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) and EUS-guided fine-needle biopsy (FNB) has been reported, enhanced diagnostic performance is expected with improvements in tissue collection methods and fine-needle techniques. Guidance for establishing the tissue diagnosis of AIP has been developed and is useful for histological evaluation. Histopathological diagnosis by EUS-FNA/FNB is expected to play a central role in AIP diagnosis in the future.
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Affiliation(s)
- Atsushi Kanno
- Division of Gastroenterology, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan.
| | - Kiichi Tamada
- Division of Gastroenterology, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan
| | | | | | - Hironori Yamamoto
- Division of Gastroenterology, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan
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Endoscopic retrograde cholangiopancreatography and intraductal ultrasonography in the diagnosis of autoimmune pancreatitis and IgG4-related sclerosing cholangitis. J Med Ultrason (2001) 2021; 48:573-580. [PMID: 34331625 DOI: 10.1007/s10396-021-01114-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2021] [Accepted: 06/10/2021] [Indexed: 12/17/2022]
Abstract
Endoscopic retrograde cholangiopancreatography is used to evaluate the narrowing of the main pancreatic duct in autoimmune pancreatitis (AIP) and biliary stricture in IgG4-related sclerosing cholangitis (IgG4-SC). Intraductal ultrasonography enables detailed visualization of the thickening of the bile duct wall in IgG4-SC. Pancreatic cancer, cholangiocarcinoma, and primary sclerosing cholangitis are important mimicking conditions of AIP and IgG4-SC. Diffuse or segmental stricture without marked upstream dilatation is a typical pancreatographic finding in AIP. By contrast, a single, short stricture with marked upstream dilatation is a typical finding in pancreatic cancer. The cholangiogram of IgG4-SC is classified into four types based on biliary stricture location, and this cholangiogram classification is useful for the differential diagnosis of IgG4-SC. Endoscopic retrograde cholangiography can be used to distinguish between IgG4-SC and primary sclerosing cholangitis. A segmental/long and intrapancreatic stricture is a characteristic finding of IgG4-SC, whereas band-like strictures, a beaded or pruned-tree appearance, and diverticulum-like outpouching are characteristic of primary sclerosing cholangitis. The characteristic intraductal ultrasonographic findings of circular-symmetrical wall thickening, smooth outer and inner margins, and homogeneous internal echo at the biliary stricture site are useful for diagnosis of IgG4-SC. Thickening of the bile duct wall at non-stricture sites is also a typical intraductal ultrasonographic finding of IgG4-SC and can be used for differential diagnosis from cholangiocarcinoma. Transpapillary bile duct and duodenal papilla biopsy during endoscopic retrograde cholangiopancreatography are also useful in the diagnosis of IgG4-SC.
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Marya NB, Powers PD, Chari ST, Gleeson FC, Leggett CL, Abu Dayyeh BK, Chandrasekhara V, Iyer PG, Majumder S, Pearson RK, Petersen BT, Rajan E, Sawas T, Storm AC, Vege SS, Chen S, Long Z, Hough DM, Mara K, Levy MJ. Utilisation of artificial intelligence for the development of an EUS-convolutional neural network model trained to enhance the diagnosis of autoimmune pancreatitis. Gut 2021; 70:1335-1344. [PMID: 33028668 DOI: 10.1136/gutjnl-2020-322821] [Citation(s) in RCA: 71] [Impact Index Per Article: 17.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/13/2020] [Revised: 09/10/2020] [Accepted: 09/15/2020] [Indexed: 02/06/2023]
Abstract
OBJECTIVE The diagnosis of autoimmune pancreatitis (AIP) is challenging. Sonographic and cross-sectional imaging findings of AIP closely mimic pancreatic ductal adenocarcinoma (PDAC) and techniques for tissue sampling of AIP are suboptimal. These limitations often result in delayed or failed diagnosis, which negatively impact patient management and outcomes. This study aimed to create an endoscopic ultrasound (EUS)-based convolutional neural network (CNN) model trained to differentiate AIP from PDAC, chronic pancreatitis (CP) and normal pancreas (NP), with sufficient performance to analyse EUS video in real time. DESIGN A database of still image and video data obtained from EUS examinations of cases of AIP, PDAC, CP and NP was used to develop a CNN. Occlusion heatmap analysis was used to identify sonographic features the CNN valued when differentiating AIP from PDAC. RESULTS From 583 patients (146 AIP, 292 PDAC, 72 CP and 73 NP), a total of 1 174 461 unique EUS images were extracted. For video data, the CNN processed 955 EUS frames per second and was: 99% sensitive, 98% specific for distinguishing AIP from NP; 94% sensitive, 71% specific for distinguishing AIP from CP; 90% sensitive, 93% specific for distinguishing AIP from PDAC; and 90% sensitive, 85% specific for distinguishing AIP from all studied conditions (ie, PDAC, CP and NP). CONCLUSION The developed EUS-CNN model accurately differentiated AIP from PDAC and benign pancreatic conditions, thereby offering the capability of earlier and more accurate diagnosis. Use of this model offers the potential for more timely and appropriate patient care and improved outcome.
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Affiliation(s)
- Neil B Marya
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | | | - Suresh T Chari
- Gastroenterology, Hepatology and Nutrition, University of Texas MD Anderson Cancer Center, Houston, Texas, USA
| | - Ferga C Gleeson
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Cadman L Leggett
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | | | | | - Prasad G Iyer
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Shounak Majumder
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Randall K Pearson
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Bret T Petersen
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Elizabeth Rajan
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Tarek Sawas
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Andrew C Storm
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Santhi S Vege
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Shigao Chen
- Diagnostic Radiology, Mayo Clinic, Rochester, Minnesota, USA
| | - Zaiyang Long
- Diagnostic Radiology, Mayo Clinic, Rochester, Minnesota, USA
| | - David M Hough
- Diagnostic Radiology, Mayo Clinic, Rochester, Minnesota, USA
| | - Kristin Mara
- Biomedical Statistics and Informatics, Mayo Clinic Rochester, Rochester, Minnesota, USA
| | - Michael J Levy
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
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Yoon SB, Moon SH, Kim JH, Park JW, Kim SE, Kim MH. Determination of the duration of glucocorticoid therapy in type 1 autoimmune pancreatitis: A systematic review and meta-analysis. Pancreatology 2021; 21:S1424-3903(21)00474-9. [PMID: 34090808 DOI: 10.1016/j.pan.2021.05.303] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/01/2021] [Revised: 05/18/2021] [Accepted: 05/21/2021] [Indexed: 12/11/2022]
Abstract
BACKGROUND The indications for maintenance glucocorticoid therapy (MGT) and its duration after initial remission of type 1 autoimmune pancreatitis (AIP) remain controversial. In contrast to the Japanese treatment protocol, the Mayo protocol does not recommend MGT after initial remission. This study aimed to evaluate the relapse rate in patients with type 1 AIP according to the duration of glucocorticoid therapy. METHODS We conducted a systematic literature review up until November 30, 2020, and identified 40 studies reporting AIP relapse rates. The pooled relapse rates were compared between groups according to the protocol and duration of glucocorticoids (routine vs. no MGT; glucocorticoids ≤6 months vs. 6-12 months vs. 12-36 months vs. ≥ 36 months). The pooled rates of adverse events related to glucocorticoids were also evaluated. RESULTS Meta-analysis indicated calculated pooled relapse rates of 46.6% (95% confidence interval (CI), 38.9-54.3%) with glucocorticoids for ≤ 6 months, 44.3% (95% CI, 38.8-49.8%) for 6-12 months, 34.1% (95% CI, 28.6-39.7%) for 12-36 months, and 27.0% (95% CI, 23.4-30.6%) for ≥ 36 months. The rate of relapse was also significantly lower in patients with routine-use protocol of MGT (31.2%; 95% CI, 27.5-34.8%) than in patients with no MGT protocol (44.1%; 95% CI, 35.8-52.4%). Adverse events were comparable between groups. CONCLUSIONS The rate of relapse tended to decrease with extended durations of glucocorticoid therapy up to 36 months. Clinicians may decide the duration of glucocorticoids according to patient condition, including comorbidities and risk of relapse.
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Affiliation(s)
- Seung Bae Yoon
- Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, South Korea
| | - Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea; Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea.
| | - Jong Hyeok Kim
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea; Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea
| | - Ji Won Park
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea; Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea
| | - Sung Eun Kim
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea; Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea
| | - Myung-Hwan Kim
- Department of Internal Medicine, University of Ulsan, College of Medicine, Asan Medical Center, Seoul, South Korea
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Zhang D, Mao S, Lan S, Zhou C, Liu X. CT Image Changes of Severe Acute Pancreatitis Based on Smart Electronic Medical Augmented Reality in Nursing Practice. JOURNAL OF HEALTHCARE ENGINEERING 2021; 2021:5522492. [PMID: 33995982 PMCID: PMC8096576 DOI: 10.1155/2021/5522492] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/20/2021] [Revised: 04/03/2021] [Accepted: 04/15/2021] [Indexed: 01/11/2023]
Abstract
Severe acute pancreatitis (SAP) is traditionally treated with chemical analysis. Faced with the increasing maturity of CT imaging technology, it is necessary to use more advantageous CT imaging to treat SAP. In this article, 72 SAP patients admitted to the Affiliated Hospital of Southwest Medical University were selected for study, of which 62 were severely ill, 8 were exacerbated, and 2 changed from severe to mild. This article combines the patient's case records and related CT images during treatment from the perspective of nursing and conducts nursing research on the application of CT image changes in severe acute pancreatitis in nursing practice. CT image processing uses CT imaging system workstation (DICOM). The results of the study showed that, in the care of patients, 21 cases had recurrence after internal drainage, and the cure rate was 91.1%. Internal drainage is an effective way to treat SAP. The higher the incidence of pancreatitis, the more likely it is to relapse after SAP internal drainage, which may be related to repeated episodes of pancreatitis and repeated inflammation of the pancreas and pancreatic duct damage. 4 of the relapsed cases in this article are postchronic pancreatitis SAP, and the relapsed cases account for 50% of the chronic pancreatic cases. This may be due to chronic fibrosis of the branched and main pancreatic ducts, continuous abnormal pancreatic juice drainage. Therefore, it is necessary to further explore the prognosis of different causes of SAP. In terms of complication care, the overall complication rate was 16.6%. One patient died of postoperative hemorrhage. Analysis of the causes of cyst recurrence and complications may be closely related to the mechanism of the occurrence and development of SAP. The initiating factor of SAP is that the pancreatic tissue is damaged due to inflammation, trauma, or microcirculation disorder, and then the pancreatic juice leaks out of the pancreas, wrapping the pancreatic juice; it takes a certain time for the capsule of fibrous knot tissue to form and strengthen.
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Affiliation(s)
- Defen Zhang
- Emergency Intensive Care Unit, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan, China
| | - Shifang Mao
- Emergency Intensive Care Unit, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan, China
| | - Siyou Lan
- Department 1 of Respiratory and Critical Diseases, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan, China
| | - Chengli Zhou
- Emergency Intensive Care Unit, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan, China
| | - Xiaoyan Liu
- Emergency Intensive Care Unit, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan, China
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Ergin E, Oruc N, Özütemiz Ö. Autoimmune Pancreatitis after a Seven-Year History of Suspicious Pancreatic Cancer. Case Rep Gastroenterol 2021; 15:195-201. [PMID: 33790705 PMCID: PMC7989778 DOI: 10.1159/000511286] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/09/2020] [Accepted: 08/31/2020] [Indexed: 11/19/2022] Open
Abstract
In this case report, we present a case of autoimmune pancreatitis (AIP) diagnosis in a patient after a 7-year history of suspicious pancreatic cancer. Kim's and Japanese criteria were used to diagnose AIP. Our case avoided undesirable invasive procedures and recovered thanks to the proper diagnosis and timely treatment with prednisone. Early and accurate diagnosis of AIP, in this case, had a significant impact on the treatment and prognosis process.
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Affiliation(s)
- Erhan Ergin
- Gastroenterology Department, Faculty of Medicine, Ege University, Izmir, Turkey
| | - Nevin Oruc
- Gastroenterology Department, Faculty of Medicine, Ege University, Izmir, Turkey
| | - Ömer Özütemiz
- Gastroenterology Department, Faculty of Medicine, Ege University, Izmir, Turkey
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Nasser R, Gilshtein H, Mansour S, Yasin K, Borzellino G, Khuri S. Isolated Type Immunoglobulin G4 Sclerosing Cholangitis: The Misdiagnosed Cholangiocarcinoma. J Clin Med Res 2021; 13:75-81. [PMID: 33747321 PMCID: PMC7935625 DOI: 10.14740/jocmr4428] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2021] [Accepted: 01/21/2021] [Indexed: 12/13/2022] Open
Abstract
Immunoglobulin G4 sclerosing cholangitis (IgG4-SC), firstly described in 2004, is the biliary manifestation of a recently described multisystem immune-mediated disease known as IgG4-related disease. IgG4-SC is a unique and rare type of cholangitis of unknown etiology and its precise prevalence rate is still unclear. It is characterized by bile duct wall thickening and high levels of systemic serum IgG4 plasma cells. Differential diagnoses for IgG4-SC include benign (primary sclerosing cholangitis) as well as malignant (extra-hepatic cholangiocarcinoma) diseases. Discrimination between these entities is very important, due to the fact that they have different biological behaviors and different therapeutic strategies. The rare IgG4-SC subgroup with its puzzling manifestations carries a hefty diagnostic challenge for the treating physicians, and inaccurate diagnosis can lead to unnecessary morbid surgical procedures. With the paucity and relative weakness of available data in the current literature, one needs to carefully review all available parameters. A low threshold of suspicion is required to try and prevent missing IgG4-SC. IgG4-SC is highly responsive to steroid treatment, especially during the early inflammatory phase, while delay in management could lead to fibrosis and organ dysfunction. On the other hand, cholangiocarcinoma is treated by means of surgery and/or chemotherapeutic agents.
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Affiliation(s)
- Roni Nasser
- Gastroenterology and Hepatology Department, Rambam Health Care Campus, Haifa, Israel
| | - Hayim Gilshtein
- Colorectal Surgery Unit, General Surgery Department, Rambam Health Care Campus, Haifa, Israel
| | - Subhi Mansour
- HPB and Surgical Oncology Unit, General Surgery Department, Rambam Health Care Campus, Haifa, Israel
| | - Kamel Yasin
- Gastroenterology and Hepatology Department, Rambam Health Care Campus, Haifa, Israel
| | | | - Safi Khuri
- HPB and Surgical Oncology Unit, General Surgery Department, Rambam Health Care Campus, Haifa, Israel
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Floreani A, Okazaki K, Uchida K, Gershwin ME. IgG4-related disease: Changing epidemiology and new thoughts on a multisystem disease. J Transl Autoimmun 2020; 4:100074. [PMID: 33490938 PMCID: PMC7806798 DOI: 10.1016/j.jtauto.2020.100074] [Citation(s) in RCA: 30] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2020] [Revised: 11/19/2020] [Accepted: 12/10/2020] [Indexed: 02/07/2023] Open
Abstract
IgG4-related disease (IgG4-RD) represents an immune-mediated fibroinflammatory condition with peculiar histopathologic changes that can affect various organs. In 2012 its unified nomenclature was published, which allows to abandon other synonymous names. Up to now, only little is known about its epidemiology around the world. However, although it is generally considered a rare condition, the number of patients with IgG4-RD is increasing enormously. Likewise, the annual number of publications on this subject has increased progressively. The spectrum of clinical manifestations in IgG4-RD is highly variable, depending on the severity of the disease as well as the presence of organ(s) involvement. This review gives an overview on changing epidemiology of IgG4-RD focusing the attention on the large cohorts of patients published in the literature.
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Affiliation(s)
- Annarosa Floreani
- Scientific Consultant IRCCS Negrar, Verona, Italy
- Senior Scholar, University of Padova, Italy
| | - Kazuichi Okazaki
- Department of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
| | - Kazushige Uchida
- Department of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
| | - M. Eric Gershwin
- Division of Rheumatology Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, CA, USA
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Erivwo P, Turashvili G. Pathology of IgG4-related sclerosing mastitis. J Clin Pathol 2020; 74:475-482. [PMID: 33328182 DOI: 10.1136/jclinpath-2020-207029] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2020] [Revised: 11/15/2020] [Accepted: 12/06/2020] [Indexed: 01/11/2023]
Abstract
Immunoglobulin G4-related sclerosing mastitis (IgG4-RM) is a recently recognised member of the IgG4-related disease (IgG4-RD) family, a multisystem fibroinflammatory condition that can affect any organ system. IgG4-RM is rare and predominantly occurs in middle-aged women. It may present with painless palpable mass and/or lymphadenopathy thereby mimicking breast cancer. Although there is an abundance of literature describing the clinicopathological characteristics of IgG4-RD in a variety of organs, data on IgG4-RM are limited due to its rarity. This review describes the manifestation of the disease in the breast based on reported cases, emphasising the clinicopathological features, pathophysiology, differential diagnosis, treatment and prognosis.
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Affiliation(s)
- Polycarp Erivwo
- Department of Pathology, Memorial University of Newfoundland, St. John's, Newfoundland, Canada
| | - Gulisa Turashvili
- Department of Pathology and Laboratory Medicine, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada
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Yoon SB, Moon SH, Kim JH, Song TJ, Kim MH. The use of immunohistochemistry for IgG4 in the diagnosis of autoimmune pancreatitis: A systematic review and meta-analysis. Pancreatology 2020; 20:1611-1619. [PMID: 33060017 DOI: 10.1016/j.pan.2020.10.028] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/22/2020] [Revised: 08/01/2020] [Accepted: 10/08/2020] [Indexed: 02/07/2023]
Abstract
BACKGROUND The diagnosis of autoimmune pancreatitis (AIP) remains challenging, especially when serum IgG4 is normal or imaging features are indeterminate. We performed a systematic review and meta-analysis to evaluate the performance of IgG4 immunostaining of pancreatic, biliary, and ampullary tissues as a diagnostic aid for AIP. METHODS A comprehensive literature search of the PubMed, EMBASE, and Ovid MEDLINE databases was conducted until February 2020. The methodological quality of each study was assessed according to the Quality Assessment of Diagnostic Accuracy Studies checklist. A random-effects model was used to summarize the diagnostic odds ratio and other measures of accuracy. RESULTS The meta-analysis included 20 studies comprising 346 patients with AIP and 590 patients with other pancreatobiliary diseases, including 371 pancreatobiliary malignancies. The summary estimates for tissue IgG4 in discriminating AIP and controls were as follows: diagnostic odds ratio 38.86 (95% confidence interval (CI), 18.70-80.75); sensitivity 0.64 (95% CI, 0.59-0.69); specificity 0.93 (95% CI, 0.91-0.95). The area under the curve was 0.939 for tissue IgG4 in discriminating AIP and controls. Subgroup analysis revealed no significant difference in diagnostic accuracy according to control groups (pancreatobiliary cancer versus other chronic pancreatitis) and sampling site (pancreas versus bile duct/ampulla). CONCLUSIONS Current data demonstrate that IgG4 immunostaining of pancreatic, biliary, and ampullary tissue has a high specificity but moderate sensitivity for diagnosing AIP. IgG4 immunostaining may be useful in supporting a diagnosis of AIP when AIP is clinically suspected, but a combination of imaging and serology does not provide a conclusive diagnosis.
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Affiliation(s)
- Seung Bae Yoon
- Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, South Korea
| | - Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea; Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea.
| | - Jong Hyeok Kim
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea; Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea
| | - Tae Jun Song
- Department of Internal Medicine, University of Ulsan, College of Medicine, Asan Medical Center, Seoul, South Korea
| | - Myung-Hwan Kim
- Department of Internal Medicine, University of Ulsan, College of Medicine, Asan Medical Center, Seoul, South Korea
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Kanno A, Ikeda E, Ando K, Nagai H, Miwata T, Kawasaki Y, Tada Y, Yokoyama K, Numao N, Ushio J, Tamada K, Lefor AK, Yamamoto H. The Diagnosis of Autoimmune Pancreatitis Using Endoscopic Ultrasonography. Diagnostics (Basel) 2020; 10:diagnostics10121005. [PMID: 33255660 PMCID: PMC7760882 DOI: 10.3390/diagnostics10121005] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2020] [Revised: 11/18/2020] [Accepted: 11/20/2020] [Indexed: 12/14/2022] Open
Abstract
Autoimmune pancreatitis (AIP) is characterized by enlargement of the pancreas and irregular narrowing of the main pancreatic duct. It is often associated with IgG4-related sclerosing cholangitis (IgG4-SC), in which the bile duct narrows. Although characteristic irregular narrowing of the pancreatic duct caused by endoscopic retrograde cholangiopancreatography is noted in AIP, it is difficult to differentiate between localized AIP and pancreatic carcinoma based on imaging of the pancreatic duct. While stenosis of the bile duct in IgG4-SC is characterized by longer-length stenosis than in cholangiocarcinoma, differentiation based on bile duct imaging alone is challenging. Endoscopic ultrasound (EUS) can characterize hypoechoic enlargement of the pancreas or bile duct wall thickening in AIP and IgG4-SC, and diagnosis using elastography and contrast-enhanced EUS are being evaluated. The utility of EUS-guided fine needle aspiration for the histological diagnosis of AIP has been reported and is expected to improve diagnostic performance for AIP. Findings in the bile duct wall from endoscopic retrograde cholangiopancreatography followed by intraductal ultrasonography are useful in differentiating IgG4-SC from cholangiocarcinoma. Diagnoses based on endoscopic ultrasonography play a central role in the diagnosis of AIP.
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Affiliation(s)
- Atsushi Kanno
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
- Correspondence: ; Tel.: +81-285-58-7348; Fax: 81-285-44-8297
| | - Eriko Ikeda
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Kozue Ando
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Hiroki Nagai
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Tetsuro Miwata
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Yuki Kawasaki
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Yamato Tada
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Kensuke Yokoyama
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Norikatsu Numao
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Jun Ushio
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Kiichi Tamada
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Alan Kawarai Lefor
- Department of Surgery, Jichi Medical University, Shimotsuke 329-0498, Japan;
| | - Hironori Yamamoto
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
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Xiao J, Hu B, Cheng D, Shi H, Xiu Y. Features of IgG4-related lung disease on 18F-FDG PET/computed tomography imaging. Nucl Med Commun 2020; 41:933-941. [PMID: 32796482 DOI: 10.1097/mnm.0000000000001238] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
OBJECTIVES The aim of the study was to summarize the features of immunoglobulin G4-related lung disease (IgG4-RLD) on fluorine 18-fluorodeoxyglucose (F-FDG) PET/computed tomography (CT). METHODS In this retrospective case series, 12 consecutive patients (9 men and 3 women, mean age 55.4 ± 13.7 years) with IgG4-RLD were included. The clinicopathological information and features of F-FDG PET/CT imaging were analyzed. RESULTS Six (50%) patients had pulmonary involvement alone and six (50%) patients had extrapulmonary involvement with intense F-FDG uptake. Pulmonary manifestations included mass (25%, 3/12), solid nodule (solitary 25%, 3/12; multiple 50%, 6/12), multiple ground-glass opacities (GGOs) (50%, 6/12), thickening of alveolar interstitium (50%, 6/12), and thickening of bronchovascular bundle (33.3%, 4/12). The maximum standardized uptake value (SUVmax) of the solid nodules and masses, multiple GGOs, bronchovascular bundle and the thickening of septa was 4.0 ± 2.5, 2.3 ± 1.8, 1.4 ± 0.6, and 0.9 ± 0.5, respectively. The SUVmax statistically significant linear association with the diameter of masses or solid nodules (P value = 0.03), but no significant inverse linear association (P value = 0.06) with the concentration of serum IgG4 concentration. CONCLUSIONS The image patterns of IgG4-RLD on F-FDG PET/CT are varying. Multiple pulmonary manifestations or multiple organ involvement, especially in combination with elevated levels of serum IgG and IgG4, may help to make the diagnosis. A potential major application of PET-CT would be evaluation of response to treatment, and the impact of PET/CT on IgG4-RLD management is worth investigating further in the future.
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Affiliation(s)
- Jie Xiao
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University
- Nuclear Medicine Institute of Fudan University
- Medical Imaging Institute of Shanghai, China
| | - Bingxin Hu
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University
- Nuclear Medicine Institute of Fudan University
- Medical Imaging Institute of Shanghai, China
| | - Dengfeng Cheng
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University
- Nuclear Medicine Institute of Fudan University
- Medical Imaging Institute of Shanghai, China
| | - Hongcheng Shi
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University
- Nuclear Medicine Institute of Fudan University
- Medical Imaging Institute of Shanghai, China
| | - Yan Xiu
- Department of Nuclear Medicine, Zhongshan Hospital, Fudan University
- Nuclear Medicine Institute of Fudan University
- Medical Imaging Institute of Shanghai, China
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Blaho M, Dítě P, Kunovský L, Kianička B. Autoimmune pancreatitis - An ongoing challenge. Adv Med Sci 2020; 65:403-408. [PMID: 32805624 DOI: 10.1016/j.advms.2020.07.002] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/01/2020] [Revised: 04/15/2020] [Accepted: 07/13/2020] [Indexed: 02/07/2023]
Abstract
Autoimmune pancreatitis is a rare form of chronic pancreatitis. The first descriptions of the disease date back to the 1990s. Etiology is multifactorial, with the use of genetic, environmental and complex immunological mechanisms. It is classified into two subtypes. Type 1 is part of a group of diseases called IgG4-related disease. Clinically is autoimmune pancreatitis manifested by icterus and abdominal discomfort. It can rarely present as acute pancreatitis. There is also a completely asymptomatic form of the disease. The diagnosis is based on abnormalities in histology, imaging methods, serology, the involvement of other organs in relation to IgG4-related disease, and a significant positive response to corticosteroid therapy. Differential diagnosis between the focal form of autoimmune pancreatitis and pancreatic cancer can be complicated, with endosonography playing an important role. In the treatment, we use corticosteroids and other immunosuppressants including biological therapy. Patients with the asymptomatic disease should also be treated to prevent late complications and exocrine and endocrine insufficiency. In addition to drug treatment, endoscopic and/or surgical treatment may be necessary. Even after recovery, the disease can relapse. The relationship between autoimmune pancreatitis and malignancies has not been clearly confirmed. The goal of this review is to provide a comprehensive look at autoimmune pancreatitis and translate latest scientific knowledge into clinical practice.
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Affiliation(s)
- Martin Blaho
- Department of Internal Medicine, Department of Gastroenterology, University Hospital Ostrava, Ostrava, Czech Republic; Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic; Department of Internal Medicine II - Gastroenterology and Geriatrics, Faculty of Medicine, Palacký University Olomouc and University Hospital, Olomouc, Czech Republic
| | - Petr Dítě
- Department of Internal Medicine, Department of Gastroenterology, University Hospital Ostrava, Ostrava, Czech Republic; Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic
| | - Lumír Kunovský
- Department of Gastroenterology and Internal Medicine, University Hospital Brno, Brno, Czech Republic; Department of Surgery, University Hospital Brno, Brno, Czech Republic; Faculty of Medicine, Masaryk University, Brno, Czech Republic
| | - Bohuslav Kianička
- Faculty of Medicine, Masaryk University, Brno, Czech Republic; 2nd Department of Internal Medicine, Department of Gastroenterology, St. Anne's University Hospital, Brno, Czech Republic.
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Tanaka A, Mori M, Kubota K, Naitoh I, Nakazawa T, Takikawa H, Unno M, Kamisawa T, Kawa S, Okazaki K. Epidemiological features of immunoglobulin G4-related sclerosing cholangitis in Japan. JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES 2020; 27:598-603. [PMID: 32603554 DOI: 10.1002/jhbp.793] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/01/2020] [Accepted: 06/23/2020] [Indexed: 12/17/2022]
Abstract
BACKGROUND/PURPOSE Epidemiological data on immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are scarce. METHOD We randomly selected healthcare facilities from a list of all facilities in Japan. The selection rate was determined according to a stratification based on the facility characteristics and scale. We sent questionnaires to the selected facilities enquiring about the number and sex of patients with IgG4-SC in 2018. Among responding facilities, we further inquired about the clinical characteristics of the patients with IgG4-SC. RESULTS We selected 1180 departments from health centers across Japan. Of them, 532 (45.1%) responded to the questionnaires. The number of reported patients with IgG4-SC was 1045, and the estimated point prevalence was 2.18 (95% confidence interval, 2.13-2.23) per 100 000 population. In addition, we enrolled 1096 patients with IgG4-SC, combining data from previously and newly registered patients. Male patients comprised 79.5% of the population and patients aged 60-80 years had the highest risk for developing the disease. The follow-up period was 5.1 ± 4.0 years and the 5- and 10-year overall survival rates were 95.3% and 89.0%, respectively. CONCLUSIONS The point prevalence of IgG4-SC was 2.18 per 100 000 population. The epidemiological features were similar to those observed in previous studies.
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Affiliation(s)
- Atsushi Tanaka
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
| | - Mitsuru Mori
- Hokkaido Chitose College of Rehabilitation, Chitose, Japan
| | - Kensuke Kubota
- Department of Endoscopy, Yokohama City University Hospital, Yokohama, Japan
| | - Itaru Naitoh
- Department of Gastroenterology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Takahiro Nakazawa
- Department of Gastroenterology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Hajime Takikawa
- Faculty of Medical Technology, Teikyo University, Tokyo, Japan
| | - Michiaki Unno
- Division of Hepato-Biliary Pancreatic Surgery, Tohoku University Graduate School, of Medicine, Sendai, Japan
| | - Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan, Komagome Hospital, Tokyo, Japan
| | - Shigeyuki Kawa
- Department of Internal Medicine, Matsumoto Dental University, Matsumoto, Japan
| | - Kazuichi Okazaki
- The Third Department of Internal Medicine, Kansai Medical University, Hirakata, Japan
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Li KM, Xu MH, Wu X, He WM. The Expression of IgG and IgG4 in Orbital MALT Lymphoma: The Similarities and Differences of IgG4-Related Diseases. Onco Targets Ther 2020; 13:5755-5761. [PMID: 32606787 PMCID: PMC7306456 DOI: 10.2147/ott.s242852] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2019] [Accepted: 04/09/2020] [Indexed: 02/05/2023] Open
Abstract
Objective This study aims to study the expression and role of IgG and IgG4 in orbital MALT lymphoma and to compare the characteristics of IgG4-related diseases. Patients and Methods Patients with orbital MALT lymphoma, treated in the West China Hospital of Sichuan University from 2012 to 2017, were enrolled in the current study. The immunological examination of the wax blocks of orbital masses was performed again and the expression level of IgG and IgG4 in pathological tissue was analyzed. Results The results presented that the positive rates of IgG and IgG4 in the cases of orbital MALT lymphoma were 90.91% and 61.98% respectively, of which IgG4/IgG >40% accounted for 49.33%. The positive rates of IgG and IgG4 in relapse cases were 94.60% and 70.27% respectively, and IgG4/IgG >40% accounted for 42.31%. There was no significant change in the expression of IgG and IgG4 in cases of lymphoproliferation converting to MALT lymphoma whereas, in cases of MALT lymphoma postoperatively converting to lymphoproliferation, there was an increase in IgG and IgG4 expression, with the change of IgG4 being significant. Conclusion IgG and IgG4 have a high correlation in the pathogenesis of MALT lymphoma and may even play an important role in the transformation of MALT lymphoma into orbital lymphoid hyperplasia. Given the association of IgG4 with inflammation and tumors and as an important diagnostic indicator for IgG4-RD and IgG4-related ophthalmic diseases, IgG4 may play an important role in these diseases.
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Affiliation(s)
- Kai-Ming Li
- Department of Ophthalmology, West China Hospital of Sichuan University, Chengdu 610000, People's Republic of China.,Department of Ophthalmology, Affiliated Hospital of Southwest Medical University, Luzhou 646000, People's Republic of China
| | - Man-Hua Xu
- Department of Ophthalmology, Affiliated Hospital of Southwest Medical University, Luzhou 646000, People's Republic of China
| | - Xia Wu
- Department of Pathology, West China Second Hospital of Sichuan University, Chengdu 610000, People's Republic of China
| | - Wei-Min He
- Department of Ophthalmology, West China Hospital of Sichuan University, Chengdu 610000, People's Republic of China
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Chhoda A, Rustagi T. EUS-guided needle biopsy for autoimmune pancreatitis. Clin J Gastroenterol 2020; 13:669-677. [DOI: 10.1007/s12328-020-01153-0] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2019] [Accepted: 06/02/2020] [Indexed: 12/11/2022]
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Abstract
OBJECTIVE Since the time of inception of autoimmune pancreatitis (AIP), our knowledge of autoimmune pancreatitis has expanded significantly. The aim of this review is to provide an update on clinical manifestations, diagnosis, imaging features, and treatment of AIP. BACKGROUND AND CLINICAL SIGNIFICANCE Type 1 AIP is the pancreatic manifestation of IgG4-related systemic disease, which can be diagnosed using a combination of clinical, histopathological, pancreatic imaging findings in conjunction with manifestation in other organs, as well of responsiveness to steroid treatment. It is vital to differentiate AIP from pancreatic cancer since both can mimic each other clinically and radiologically. Type 2 AIP is a rare but distinct subtype of AIP which occurs mostly in the younger patient. CONCLUSION AIP is steroid-responsive chronic pancreatitis with distinct manifestations on imaging.
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Affiliation(s)
- Ashish Khandelwal
- Department of Radiology, Mayo Clinic, 200 First St, Rochester, MN, 55902, USA
| | - Dai Inoue
- Department of Radiology, Kanazawa Univeristy, 1192 Kakumamachi, Kanazawa, Ishikawa, 920-1192, Japan
| | - Naoki Takahashi
- Department of Radiology, Mayo Clinic, 200 First St, Rochester, MN, 55902, USA.
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