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Bagare PC, Borle A, Baluni P, Ekbote GG, Sangale S. Clinical Profile and Outcomes of Patients With Systemic Lupus Erythematosus. Cureus 2024; 16:e68541. [PMID: 39364459 PMCID: PMC11448960 DOI: 10.7759/cureus.68541] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/02/2024] [Indexed: 10/05/2024] Open
Abstract
Background Systemic lupus erythematosus (SLE) is a complex autoimmune disorder characterized by relapsing-remitting immune system activation, affecting multiple organ systems. Despite significant advances in understanding SLE's pathogenesis, there remains a need for comprehensive clinical profiling at the time of diagnosis to improve early detection and management. This study addresses this gap by providing a detailed analysis of the clinical presentation, disease activity, and patient outcomes using the Systemic Lupus International Collaborating Clinics (SLICC) criteria and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) index. Methodology This cross-sectional observational study included 80 patients diagnosed with SLE using the 2012 SLICC criteria. Patients were recruited from the Rheumatology department and other wards of Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospital, Pune, India. All participants provided informed consent and institutional ethical approval was obtained. Data were collected through detailed clinical history, physical examinations, and standard tests such as chest X-rays, CBC, RFT, LFT, urine microscopy, creatine phosphokinase, ANA, AntiDsDNA, complement consumption, and Coombs' tests, with 2D echocardiography performed as needed. Follow-ups every three months over 1.5 years assessed disease activity using SLEDAI criteria. Patients aged 12 and above who met the SLICC criteria were included and those with other connective tissue disorders were excluded. Associations between clinical symptoms and organ involvement were analyzed using the chi-square test with a p-value of <0.05 considered significant. Results The study evaluated 80 patients with SLE, revealing a predominantly female cohort (80%) with a mean age of 29.4 years and a standard deviation of 8.3 years, skewed towards younger age groups. Clinical manifestations were diverse; the most common symptoms were (83.75%), oral ulcers (98.75%), and alopecia (95%). Anemia (66.25%) was the most prevalent abnormality, followed by albuminuria and renal abnormalities. Organ involvement was highest in the renal system (50%) and mucocutaneous features, with lower incidences in cardiac, gastrointestinal, and vascular systems. Gender-specific analyses indicated significant differences in SLE nephritis (p=0.048) and autoimmune hemolytic anemia (p=0.046). Autoantibody profiles showed high positivity for ANA (98.8%) and DsDNA (61.3%). Clinical outcomes demonstrated that 68.8% of patients achieved remission and 16.3% experienced organ damage. The SLEDAI scores significantly improved over time, with substantial reductions from baseline to nine months (p<0.001). Conclusion In conclusion, this study provides a detailed examination of SLE, revealing that it predominantly affects young adults and is characterized by diverse manifestations including mucocutaneous symptoms, significant renal involvement, and notable autoantibody profiles. The high prevalence of anti-nucleosome and anti-dsDNA antibodies underscores their diagnostic and prognostic value. Clinically, the findings highlight the necessity for early detection and targeted management of SLE, particularly in addressing renal and mucocutaneous symptoms. Future research should focus on longitudinal studies to track disease progression, explore genetic and environmental influences, and investigate regional variations to enhance treatment strategies and patient outcomes.
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Affiliation(s)
- Prasad C Bagare
- Internal Medicine, Dr. DY Patil Medical College, Hospital and Research Centre, Pune, IND
| | - Akshata Borle
- Internal Medicine, Dr. DY Patil Medical College, Hospital and Research Centre, Pune, IND
| | - Priya Baluni
- Internal Medicine, Dr. DY Patil Medical College, Hospital and Research Centre, Pune, IND
| | | | - Shashikala Sangale
- Internal Medicine, Byramjee Jeejeebhoy Government Medical College and Hospital, Pune, IND
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2
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Amoura Z, Bader-Meunier B, Antignac M, Bardin N, Belizna C, Belot A, Bonnotte B, Bouaziz JD, Chasset F, Chiche L, Cohen F, Costedoat-Chalumeau N, Daugas E, Devilliers H, Diot E, Elefant E, Faguer S, Ferreira N, Hachulla E, Hanslik T, Hie M, Jourde-Chiche N, Le Guern V, Martin T, Mathian A, Michel M, Miyara M, Papo T, Richez C, Scherlinger M, Sibilia J, Uzunhan Y, Wahl D, Wojtasik G, Yelnik C. French protocol for the diagnosis and management of systemic lupus erythematosus. Rev Med Interne 2024; 45:559-599. [PMID: 39191627 DOI: 10.1016/j.revmed.2024.07.006] [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: 06/25/2024] [Accepted: 07/21/2024] [Indexed: 08/29/2024]
Abstract
Because Systemic Lupus Erythematosus (SLE) is a rare disease, and due to the significant prognostic impact of early management, a diagnosis confirmed by a physician with experience in SLE is recommended, for example from an expert center. Once the diagnosis is confirmed, existing manifestations should be identified in particular, renal involvement by an assessment of proteinuria, disease activity and severity should be determined, potential complications anticipated, associated diseases searched for, and the patient's socioprofessional and family context noted. Therapeutic management of SLE includes patient education on recognizing symptoms, understanding disease progression as well as when they should seek medical advice. Patients are informed about routine checkups, treatment side effects, and the need for regular vaccinations, especially if they are receiving immunosuppressive treatment. They are also advised on lifestyle factors such as the risks of smoking, sun exposure, and dietary adjustments, especially when they are receiving corticosteroids. The importance of contraception, particularly when teratogenic medications are being used, and regular cancer screening are emphasized. Support networks can help relieve a patient's isolation. The first-line medical treatment of SLE is hydroxychloroquine (HCQ), possibly combined with an immunosuppressant and/or low-dose corticosteroid therapy. The treatment of flares depends on their severity, and typically involves HCQ and NSAIDs, but may be escalated to corticosteroid therapy with immunosuppressants or biologic therapies in moderate to severe cases. Because there is no curative treatment, the goals of therapy are patient comfort, preventing progression and flares, and preserving overall long-term health and fertility. The frequency of follow-up visits depends on disease severity and any new symptoms. Regular specialized assessments are necessary, especially when treatment changes, but a frequency of every 3 to 6 months is recommended during periods of remission and monthly during active or severe disease, especially in children. These assessments include both clinical and laboratory tests to monitor complications and disease activity, with specific attention to proteinuria.
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Affiliation(s)
- Zahir Amoura
- Department of Internal Medicine, Institute E3M, CIMI-Paris, Faculty of medicine, National Reference Centre of Systemic Lupus, Antiphospholipid Syndrome, and Other Autoimmune Diseases, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, boulevard de l'Hôpital, 75013 Paris, France.
| | - Brigitte Bader-Meunier
- Paediatric Immunology and Rhumatologie, Hospital Necker for Sick Children, AP-HP, Paris, France.
| | - Marie Antignac
- Department of Pharmacy, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, Paris, France
| | - Nathalie Bardin
- Department of Immunology, Biogénopôle, Timone Hospital, AP-HM, Marseille, France
| | - Cristina Belizna
- Department of Internal Medicine, Department Clinique of L'Anjou, Angers, France
| | - Alexandre Belot
- Department of Paediatric Nephrology, Rheumatology, Dermatology, Reference Centre for Rheumatic, Autoimmune and Systemic Diseases in Children (RAISE), Femme Mère Enfant Hospital, Lyon University Hospital, Bron, France
| | - Bernard Bonnotte
- Department of Internal Medicine, Dijon University Hospital, Dijon, France
| | | | - François Chasset
- Department of Dermatology and Allergology, Faculty of Medicine, Tenon Hospital, Sorbonne University, Paris, France
| | - Laurent Chiche
- Department of Internal Medicine, Marseille Public University Hospital System, Marseille, France
| | - Fleur Cohen
- Department of Internal Medicine, Institute E3M, CIMI-Paris, Faculty of medicine, National Reference Centre of Systemic Lupus, Antiphospholipid Syndrome, and Other Autoimmune Diseases, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, boulevard de l'Hôpital, 75013 Paris, France
| | - Nathalie Costedoat-Chalumeau
- Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases of Île-de-France, Centre for Epidemiology and Statistics, institut national de la santé et de la recherche médicale, French National Institute for Agricultural Research, Cochin Hospital, AP-HP, University Paris Cité, Paris, France
| | - Eric Daugas
- Department of Nephrology, Bichat-Claude Hospital, AP-HP, Nord University of Paris, Paris, France
| | - Hervé Devilliers
- Department of Internal Medicine, Dijon University Hospital, Dijon, France
| | - Elisabeth Diot
- Department of Internal Medicine, Tours University Hospital, Tours, France
| | - Elisabeth Elefant
- Department of Public Health, Teratogens Reference Centre (CRAT), Trousseau Hospital, Sorbonne University, Paris, France
| | - Stanislas Faguer
- Department of Nephrology and Organ Transplantation, Transplantation, Immunity and Environment (TImE) Research Group, Reference Centre of Rare Renal Diseases, University Hospital of Toulouse, Toulouse, France
| | - Nicole Ferreira
- Department of Internal Medicine, Tours University Hospital, Tours, France
| | - Eric Hachulla
- Department of Internal Medicine and Clinical immunology, Reference Centre of Autoimmune Systemic Rare Diseases of North and North-West of France (CeRAINO), Lille University, Inserm, University Hospital of Lille, Lille, France
| | - Thomas Hanslik
- Department of Internal Medicine, Ambroise-Paré Hospital, AP-HP, Paris, France
| | - Miguel Hie
- Department of Internal Medicine, Institute E3M, CIMI-Paris, Faculty of medicine, National Reference Centre of Systemic Lupus, Antiphospholipid Syndrome, and Other Autoimmune Diseases, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, boulevard de l'Hôpital, 75013 Paris, France
| | | | - Véronique Le Guern
- Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases of Île-de-France, Centre for Epidemiology and Statistics, institut national de la santé et de la recherche médicale, French National Institute for Agricultural Research, Cochin Hospital, AP-HP, University Paris Cité, Paris, France
| | - Thierry Martin
- Department of Internal Medicine and Clinical Immunology, Strasbourg University Hospital, Strasbourg, France
| | - Alexis Mathian
- Department of Internal Medicine, Institute E3M, CIMI-Paris, Faculty of medicine, National Reference Centre of Systemic Lupus, Antiphospholipid Syndrome, and Other Autoimmune Diseases, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, boulevard de l'Hôpital, 75013 Paris, France
| | - Marc Michel
- Department of Internal Medicine, National Referral Centre for Autoimmune Cytopenias, Créteil University Hospital, Créteil, France
| | - Makoto Miyara
- Department of Immunology, Pitié-Salpêtrière Hospital, AP-HP, University of Sorbonne, Paris, France
| | - Thomas Papo
- Department of Internal Medicine, Bichat Hospital, AP-HP, Paris, France
| | - Christophe Richez
- Department of Rhumatologie, Bordeaux University Hospital, Bordeaux, France
| | - Marc Scherlinger
- Department of Rhumatologie, Strasbourg University Hospital of Hautepierre, Strasbourg, France
| | - Jean Sibilia
- Department of Rhumatologie, Strasbourg University Hospital of Hautepierre, Strasbourg, France
| | - Yurdagul Uzunhan
- Department of Pneumology, Centre of Reference for Rare Lung Diseases, Avicenne Hospital, Sorbonne Paris North University, Bobigny, France
| | - Denis Wahl
- Department of Vascular Medicine and National Referral Centre for Rare Vascular and Systemic Autoimmune Diseases, University Hospital of Nancy, Nancy, France
| | - Géraldine Wojtasik
- Department of Internal Medicine and Clinical immunology, Reference Centre of Autoimmune Systemic Rare Diseases of North and North-West of France (CeRAINO), Lille University, Inserm, University Hospital of Lille, Lille, France
| | - Cécile Yelnik
- Department of Internal Medicine and Clinical immunology, Reference Centre of Autoimmune Systemic Rare Diseases of North and North-West of France (CeRAINO), Lille University, Inserm, University Hospital of Lille, Lille, France
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Lin W, Zheng Q, Wang X, Lin X, Ni X, Pan J, Zippi M, Fiorino S, Hong W. The causality between use of glucocorticoids and risk of pancreatitis: a Mendelian randomization study. Front Immunol 2024; 15:1420840. [PMID: 39221257 PMCID: PMC11363070 DOI: 10.3389/fimmu.2024.1420840] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/21/2024] [Accepted: 07/29/2024] [Indexed: 09/04/2024] Open
Abstract
Background and aim To date, the association between glucocorticoid use and the risk of pancreatitis remains controversial. The aim of this study was the investigation of this possible relationship. Methods We carried out a two-sample Mendelian randomization (MR) analysis using GWAS data from European ancestry, East Asian descendants and the FinnGen Biobank Consortium to evaluate this potential causal relationship. Genetic variants associated with glucocorticoid use were selected based on genome-wide significance (p < 5×10-8). Results Our MR analysis of European ancestry data revealed no significant causal relationship between glucocorticoid use and AP (IVW: OR=1.084, 95% CI= 0.945-1.242, P=0.249; MR-Egger: OR=1.049, 95% CI= 0.686-1.603, P=0.828; weighted median: OR=1.026, 95% CI= 0.863-1.219, P=0.775) or CP (IVW: OR=1.027, 95% CI= 0.850-1.240, P=0.785; MR-Egger: OR= 1.625, 95% CI= 0.913-2.890, P= 0.111; weighted median: OR= 1.176, 95% CI= 0.909-1.523, P= 0.218). Sensitivity analyses, including MR-Egger and MR-PRESSO, indicated no evidence of pleiotropy or heterogeneity, confirming the robustness of our findings. Multivariable MR analysis adjusted for alcohol consumption, BMI, cholelithiasis and C-reactive protein levels supported these findings. Replicated analysis was performed on datasets from the FinnGen Biobank Consortium and East Asian descendants, and similar results were obtained. Conclusions This MR analysis suggests that there is no causal association between glucocorticoid use and the risk of pancreatitis.
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Affiliation(s)
- Wenfeng Lin
- Department of Gastroenterology and Hepatology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Qiqi Zheng
- Department of Infection and Liver Diseases, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Xiaorong Wang
- Department of Intensive Care Unit, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Xiaolu Lin
- Department of Digestive Endoscopy Center, Fujian Provincial Hospital, Shengli Clinical Medical College of Fujian Medical University, Fuzhou, Fujian, China
| | - Xixi Ni
- Department of Gastroenterology and Hepatology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Jingye Pan
- Department of Intensive Care Unit, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Maddalena Zippi
- Unit of Gastroenterology and Digestive Endoscopy, Sandro Pertini Hospital, Rome, Italy
| | - Sirio Fiorino
- Unit of Internal Medicine, Budrio Hospital, Local Health Unit of Bologna, Bologna, Italy
| | - Wandong Hong
- Department of Gastroenterology and Hepatology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
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4
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Sun HW, Zhang X, Shen CC. The shared circulating diagnostic biomarkers and molecular mechanisms of systemic lupus erythematosus and inflammatory bowel disease. Front Immunol 2024; 15:1354348. [PMID: 38774864 PMCID: PMC11106441 DOI: 10.3389/fimmu.2024.1354348] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/12/2023] [Accepted: 04/22/2024] [Indexed: 05/24/2024] Open
Abstract
Background Systemic lupus erythematosus (SLE) is a multi-organ chronic autoimmune disease. Inflammatory bowel disease (IBD) is a common chronic inflammatory disease of the gastrointestinal tract. Previous studies have shown that SLE and IBD share common pathogenic pathways and genetic susceptibility, but the specific pathogenic mechanisms remain unclear. Methods The datasets of SLE and IBD were downloaded from the Gene Expression Omnibus (GEO). Differentially expressed genes (DEGs) were identified using the Limma package. Weighted gene coexpression network analysis (WGCNA) was used to determine co-expression modules related to SLE and IBD. Pathway enrichment was performed using Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis for co-driver genes. Using the Least AbsoluteShrinkage and Selection Operator (Lasso) regressionand Support Vector Machine-Recursive Feature Elimination (SVM-RFE), common diagnostic markers for both diseases were further evaluated. Then, we utilizedthe CIBERSORT method to assess the abundance of immune cell infiltration. Finally,we used the single-cell analysis to obtain the location of common diagnostic markers. Results 71 common driver genes were identified in the SLE and IBD cohorts based on the DEGs and module genes. KEGG and GO enrichment results showed that these genes were closely associated with positive regulation of programmed cell death and inflammatory responses. By using LASSO regression and SVM, five hub genes (KLRF1, GZMK, KLRB1, CD40LG, and IL-7R) were ultimately determined as common diagnostic markers for SLE and IBD. ROC curve analysis also showed good diagnostic performance. The outcomes of immune cell infiltration demonstrated that SLE and IBD shared almost identical immune infiltration patterns. Furthermore, the majority of the hub genes were commonly expressed in NK cells by single-cell analysis. Conclusion This study demonstrates that SLE and IBD share common diagnostic markers and pathogenic pathways. In addition, SLE and IBD show similar immune cellinfiltration microenvironments which provides newperspectives for future treatment.
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Affiliation(s)
- Hao-Wen Sun
- Department of Gastroenterology, Affiliated Hospital of Nantong University, Medical School of Nantong University, Nantong, China
| | - Xin Zhang
- Department of Dermatology, Affiliated Hospital of Nantong University, Medical School of Nantong University, Nantong, China
| | - Cong-Cong Shen
- Department of Dermatology, Affiliated Hospital of Nantong University, Medical School of Nantong University, Nantong, China
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Muñoz-Urbano M, Sangle S, D'Cruz DP. Lupus enteritis: a narrative review. Rheumatology (Oxford) 2024; 63:1494-1501. [PMID: 38216993 DOI: 10.1093/rheumatology/kead689] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2023] [Revised: 10/20/2023] [Accepted: 12/02/2023] [Indexed: 01/14/2024] Open
Abstract
Lupus enteritis (LE) is a rare manifestation of systemic lupus erythematosus. The pathophysiology of LE has not been fully elucidated, although inflammatory and thrombotic processes are likely important factors. The underlying pathophysiological mechanisms may depend on which portion of the intestine is affected. Over half of the patients with LE also present with renal or haematological complications. The diagnosis of LE is based on clinical, histopathological and imaging findings; abdominal computed tomography (CT) is the gold standard in diagnosis. Abdominal CT can also identify factors that predict complications and could potentially guide pharmacological and nutritional management. Timely identification and prompt treatment initiation are paramount to avoid life and organ threatening complications. Glucocorticoids are often the first-line treatment. Additional therapy including immunosuppressive therapy is utilised on a case-by-case basis as there are no clinical trials to define the optimal therapeutic approach. Surgical intervention may be needed especially if there is bowel perforation or peritonitis. In general, the prognosis of LE is good.
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Affiliation(s)
| | - Shirish Sangle
- Louise Coote Lupus Unit, Guy's Hospital, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK
| | - David P D'Cruz
- Louise Coote Lupus Unit, Guy's Hospital, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK
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Huang H, Li P, Zhang D, Zhang MX, Yu K. Acute flare of systemic lupus erythematosus with extensive gastrointestinal involvement: A case report and review of literature. World J Gastrointest Surg 2023; 15:2074-2082. [PMID: 37901723 PMCID: PMC10600777 DOI: 10.4240/wjgs.v15.i9.2074] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/24/2023] [Revised: 07/09/2023] [Accepted: 07/28/2023] [Indexed: 09/21/2023] Open
Abstract
BACKGROUND Lupus mesenteric vasculitis (LMV) is a serious condition that may occur as an acute manifestation of gastrointestinal (GI) involvement and is not easily diagnosed by physicians. Delayed diagnosis and treatment of LMV may lead to rapid disease progression and can be life threatening. CASE SUMMARY A previously healthy 27-year-old woman presented with abdominal pain following a history of fatigue and consumption of cold water. Laboratory investigations, physical examinations, and enhanced abdominal computed tomography (CT) suggested systemic lupus erythematosus complicated by LMV. She received treatments, such as GI decompression, somatostatin, glucocorticoids, and immunosuppressants, and was evaluated using color ultrasonography. Twenty days later, the patient reported no stomach discomfort and was able to consume semi-liquid food. Laboratory investigations showed that inflammatory factors decreased to normal levels and complement levels increased slightly. One year after discharged, she recovered with methylprednisolone being tapered to 4 mg per day, mycophenolate mofetil to 0.75 g bid, and hydroxychloroquine to 0.2 g bid; however, only C3 complement level was slightly below the normal level. CONCLUSION Early diagnosis of LMV is essential for successful treatment; this depends on a combination of clinical manifestations, laboratory investigations, and imaging findings. Enhanced CT is preferred, but ultrasonography can be used for prompt screening and follow-up.
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Affiliation(s)
- Hua Huang
- Department of Rheumatology and Immunology, General Hospital of Northern Theater Command, Shenyang 110000, Liaoning Province, China
| | - Ping Li
- Department of Rheumatology and Immunology, General Hospital of Northern Theater Command, Shenyang 110000, Liaoning Province, China
| | - Dan Zhang
- Department of Nutrition, General Hospital of Northern Theater Command, Shenyang 110000, Liaoning Province, China
| | - Ming-Xuan Zhang
- Department of Rheumatology and Immunology, General Hospital of Northern Theater Command, Shenyang 110000, Liaoning Province, China
| | - Kai Yu
- Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang 110000, Liaoning Province, China
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Guru S, Behera A, Barik S, Sahu A. Lithium Toxicity - A Chameleon to Gastrointestinal Vasculitis as an Initial Presenter of Systemic Lupus Erythematosus. Int J Appl Basic Med Res 2023; 13:53-55. [PMID: 37266532 PMCID: PMC10230520 DOI: 10.4103/ijabmr.ijabmr_516_22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2022] [Revised: 02/09/2023] [Accepted: 02/15/2023] [Indexed: 04/01/2023] Open
Abstract
Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease. Gastrointestinal manifesting as nausea, vomiting, and pain abdomen are not so uncommon in SLE flare. However, gastrointestinal intestinal vasculitis as an initial presenter of SLE is very rare. This case report narrated gastrointestinal vasculitis as an initial presentation of systemic lupus erythematous, which mimicked lithium toxicity in a patient of preexisting bipolar disorder who was on long-term lithium therapy. A 26-year-old female presented with abdominal pain and persistent vomiting for 2 months. On further workup, she was antinuclear, anti-Smith, and anti-ds-DNA antibody positive. The serum lithium level was found to be normal computed tomography angiogram of the abdomen suggestive of vasculitis. A final diagnosis of SLE with gastrointestinal vasculitis as an initial presenter was made. She was treated with high-dose corticosteroid, cyclophosphamide, and other supportive care. She improved dramatically and was discharged with an oral corticosteroid, hydroxychloroquine, and ramipril.
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Affiliation(s)
- Satyabrata Guru
- Department of Trauma and Emergency, AIIMS, Bhubaneswar, Odisha, India
| | - Anupama Behera
- Department of General Medicine, AIIMS, Bhubaneswar, Odisha, India
| | - Sadananda Barik
- Department of Trauma and Emergency, AIIMS, Bhubaneswar, Odisha, India
| | - Ajitesh Sahu
- Department of Trauma and Emergency, AIIMS, Bhubaneswar, Odisha, India
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Khatiwada A, Wolf BJ, Yilmaz AS, Ramos PS, Pietrzak M, Lawson A, Hunt KJ, Kim HJ, Chung D. GPA-Tree: statistical approach for functional-annotation-tree-guided prioritization of GWAS results. Bioinformatics 2022; 38:1067-1074. [PMID: 34849578 PMCID: PMC10060690 DOI: 10.1093/bioinformatics/btab802] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/01/2021] [Revised: 10/09/2021] [Accepted: 11/23/2021] [Indexed: 02/03/2023] Open
Abstract
MOTIVATION In spite of great success of genome-wide association studies (GWAS), multiple challenges still remain. First, complex traits are often associated with many single nucleotide polymorphisms (SNPs), each with small or moderate effect sizes. Second, our understanding of the functional mechanisms through which genetic variants are associated with complex traits is still limited. To address these challenges, we propose GPA-Tree and it simultaneously implements association mapping and identifies key combinations of functional annotations related to risk-associated SNPs by combining a decision tree algorithm with a hierarchical modeling framework. RESULTS First, we implemented simulation studies to evaluate the proposed GPA-Tree method and compared its performance with existing statistical approaches. The results indicate that GPA-Tree outperforms existing statistical approaches in detecting risk-associated SNPs and identifying the true combinations of functional annotations with high accuracy. Second, we applied GPA-Tree to a systemic lupus erythematosus (SLE) GWAS and functional annotation data including GenoSkyline and GenoSkylinePlus. The results from GPA-Tree highlight the dysregulation of blood immune cells, including but not limited to primary B, memory helper T, regulatory T, neutrophils and CD8+ memory T cells in SLE. These results demonstrate that GPA-Tree can be a powerful tool that improves association mapping while facilitating understanding of the underlying genetic architecture of complex traits and potential mechanisms linking risk-associated SNPs with complex traits. AVAILABILITY AND IMPLEMENTATION The GPATree software is available at https://dongjunchung.github.io/GPATree/. SUPPLEMENTARY INFORMATION Supplementary data are available at Bioinformatics online.
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Affiliation(s)
- Aastha Khatiwada
- Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC 29425, USA
- Division of Biostatistics and Bioinformatics, National Jewish Health, Denver, CO 80206, USA
| | - Bethany J Wolf
- Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC 29425, USA
| | - Ayse Selen Yilmaz
- Department of Biomedical Informatics, The Ohio State University, Columbus, OH 43210, USA
| | - Paula S Ramos
- Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC 29425, USA
- Department of Medicine, Medical University of South Carolina, Charleston, SC 29425, USA
| | - Maciej Pietrzak
- Department of Biomedical Informatics, The Ohio State University, Columbus, OH 43210, USA
| | - Andrew Lawson
- Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC 29425, USA
| | - Kelly J Hunt
- Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC 29425, USA
| | - Hang J Kim
- Division of Statistics and Data Science, University of Cincinnati, Cincinnati, OH 45221, USA
| | - Dongjun Chung
- Department of Biomedical Informatics, The Ohio State University, Columbus, OH 43210, USA
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Bandhan IH, Islam MN, Ahmad HI, Ahmedullah AK. Outcome of low-dose prednisolone use for the induction of remission in lupus nephritis patients. Int J Rheum Dis 2021; 25:121-130. [PMID: 34894070 DOI: 10.1111/1756-185x.14265] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2021] [Revised: 11/27/2021] [Accepted: 12/03/2021] [Indexed: 12/19/2022]
Abstract
OBJECTIVES This study aimed to compare the efficacy of low-dose prednisolone with conventional high-dose regimen in proliferative lupus nephritis (LN) for remission. METHODS This open-label randomized clinical trial was conducted in the Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. A total of 32 LN patients were randomized into low-dose (experimental) and high-dose (control) groups. All subjects received standard dose of intravenous (I/V) methylprednisolone and pulse I/V cyclophosphamide. Oral prednisolone, 0.5 mg/kg/d and 1 mg/kg/d were given to experimental and control groups respectively for initially 4 weeks then tapered. The patients were followed for 24 weeks. The rates of renal remission (complete and partial) were assessed at 24 weeks. The disease activity, biochemical markers, and quality of life were evaluated at baseline and at 24 weeks. RESULTS Complete renal remission was achieved by 66.7% of patients in each group (P = .99). Renal remission (partial/complete) was achieved by 86.7% and 83.3% of patients in the prednisolone low-dose group and high-dose group respectively (P = .99). In between groups, no significant difference was observed in the improvement of active urinary sediments, serum creatinine level, anti-double-stranded DNA level, complements level, disease activity and Short Form-12 score. The prednisolone dose-related adverse events like cushingoid facies, abdominal stria, infections and serious adverse events like death occurred more in the high-dose prednisolone group. CONCLUSIONS It has been observed that low-dose prednisolone regimen may be effective in LN. Steroid dose-related side effects and rate of infections were lower in this group.
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Affiliation(s)
- Iftekhar Hussain Bandhan
- Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.,Directorate General of Health Services, Dhaka, Bangladesh
| | - Md Nazrul Islam
- Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
| | - Habib Imtiaz Ahmad
- Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.,Department of Rheumatology, Enam Medical College & Hospital, Savar, Dhaka, Bangladesh
| | - Abul Khair Ahmedullah
- Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
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10
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Potera J, Palomera Tejeda E, Arora S, Manadan AM. Lupus Enteritis: An Uncommon Presentation of Lupus Flare. Cureus 2021; 13:e18030. [PMID: 34671520 PMCID: PMC8520498 DOI: 10.7759/cureus.18030] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/15/2021] [Indexed: 11/23/2022] Open
Abstract
Gastrointestinal (GI) symptoms are common in systemic lupus erythematosus (SLE) but are usually attributable to medication side effects, infections, or other underlying conditions. In rare cases, they are caused by the autoimmune process itself. In this report, we present two cases of lupus enteritis as the sole manifestation of lupus flare. We also provide a comprehensive review of available literature on this topic with a specific focus on clinical symptoms, complications, laboratory findings, histology, imaging findings, and therapies. Lupus enteritis is an uncommon manifestation of SLE. CT scan of the abdomen is the diagnostic modality of choice. The three major CT findings are target sign, comb sign, and increased mesenteric fat attenuation. Ascites is also commonly present. Corticosteroids and second-line immunosuppressants have been successfully employed in the treatment of lupus enteritis. Our cases highlight this unusual manifestation as the only symptom of active SLE. A high index of suspicion should be maintained when evaluating SLE patients presenting with GI symptoms to prevent diagnosis and treatment delays that could lead to serious complications such as bowel necrosis, perforation, and even death.
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Affiliation(s)
- Joanna Potera
- Internal Medicine, John H. Stroger, Jr. Hospital of Cook County, Chicago, USA
| | | | - Shilpa Arora
- Rheumatology, Rush University Medical Center, Chicago, USA
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11
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Frittoli RB, Vivaldo JF, Costallat LTL, Appenzeller S. Gastrointestinal involvement in systemic lupus erythematosus: A systematic review. J Transl Autoimmun 2021; 4:100106. [PMID: 34179742 PMCID: PMC8214088 DOI: 10.1016/j.jtauto.2021.100106] [Citation(s) in RCA: 24] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2021] [Accepted: 05/21/2021] [Indexed: 12/11/2022] Open
Abstract
INTRODUCTION Gastrointestinal involvement is a common complain observed in 40-60% of systemic lupus erythematosus (SLE) patients. We performed a systematic review of clinically severe and potential life-threatening gastrointestinal manifestations and discuss clinical presentation, pathogenesis and treatment. METHODS We performed a literature search in English literature using PubMed and Embase from 2000 to December 2020. The following MeSH terms: systemic lupus erythematosus, protein-losing enteropathy, ascites, pancreatitis, vasculitis, intestinal vasculitis, enteritis and diarrhea published in the English literature. RESULTS We identified 141 studies (case reports, case series and cohort studies). The most frequent presenting symptoms are acute abdominal pain, nausea, and vomiting. Many of the manifestations were associated with disease activity. Histological features are rarely available, but both vasculitis and thrombosis have been described. There is no treatment guideline. The majority of patients were treated with corticosteroids and the most common immunososupressant were azathioprine, cyclophosphamide and mycophenolate. CONCLUSION Vasculitis and thrombosis may be responsible for severe life-threatening manifestations such as pancreatitis, protein loosing gastroenteritis, acalculous cholecistyitis and enteritis.
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Affiliation(s)
- Renan Bazuco Frittoli
- Post-Graduation in Medical Pathophysiology, School of Medical Science, University of Campinas, Brazil
| | - Jéssica Fernandes Vivaldo
- Graduate Program in Child and Adolescent Health, School of Medical Science, University of Campinas, Brazil
| | - Lilian Tereza Lavras Costallat
- Department of Orthopedics, Rheumatology and Traumatology, School of Medical Science, University of Campinas, Campinas, São Paulo, 13083881, Brazil
| | - Simone Appenzeller
- Department of Medicine, School of Medical Science - State University of Campinas, Campinas, São Paulo, 13083881, Brazil
- Department of Orthopedics, Rheumatology and Traumatology, School of Medical Science, University of Campinas, Campinas, São Paulo, 13083881, Brazil
- Post-Graduation in Medical Pathophysiology, School of Medical Science, University of Campinas, Brazil
- Graduate Program in Child and Adolescent Health, School of Medical Science, University of Campinas, Brazil
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12
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Özdemir-Şimşek Ö, Kasap-Demir B, Erfidan G, Arslansoyu-Çamlar S, Soyaltın E, Alaygut D, Mutlubaş F. Severe abdominal pain in a case with lupus nephritis: Answers. Pediatr Nephrol 2021; 36:875-879. [PMID: 32749540 DOI: 10.1007/s00467-020-04675-4] [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: 06/05/2020] [Accepted: 06/11/2020] [Indexed: 11/27/2022]
Affiliation(s)
- Özgür Özdemir-Şimşek
- Department of Pediatrics, Division of Nephrology, Izmir Tepecik Training and Research Hospital, University of Health Sciences, Izmir, Turkey
| | - Belde Kasap-Demir
- Department of Pediatrics, Division of Nephrology, Izmir Tepecik Training and Research Hospital, University of Health Sciences, Izmir, Turkey.
- Department of Pediatrics, Division of Pediatric Nephrology and Rheumatology, İzmir Katip Çelebi University, Izmir, Turkey.
| | - Gökçen Erfidan
- Department of Pediatrics, Division of Nephrology, Izmir Tepecik Training and Research Hospital, University of Health Sciences, Izmir, Turkey
| | - Seçil Arslansoyu-Çamlar
- Department of Pediatrics, Division of Nephrology, Izmir Tepecik Training and Research Hospital, University of Health Sciences, Izmir, Turkey
| | - Eren Soyaltın
- Department of Pediatrics, Division of Nephrology, Izmir Tepecik Training and Research Hospital, University of Health Sciences, Izmir, Turkey
| | - Demet Alaygut
- Department of Pediatrics, Division of Nephrology, Izmir Tepecik Training and Research Hospital, University of Health Sciences, Izmir, Turkey
| | - Fatma Mutlubaş
- Department of Pediatrics, Division of Nephrology, Izmir Tepecik Training and Research Hospital, University of Health Sciences, Izmir, Turkey
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13
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Mathapathi S, Preziosi M. Multiple Hepatic Micro-Hypodensities as a Presenting Sign in Systemic Lupus Erythematosus- A Case Report. Open Rheumatol J 2020. [DOI: 10.2174/1874312902014010022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
Systemic Lupus Erythematosus (SLE) is a chronic multisystemic inflammatory disorder that can present with a wide array of signs and symptoms. Hepatic involvement is commonly limited to a subclinical biochemical transaminitis while clinically significant liver disease is rare. A case of a 22-year-old female who presented with abdominal pain, fevers, arthralgia, and several hepatic hypodense lesions with normal liver function tests is reported in this study. She failed to improve with antibiotics and infectious workup was largely unrevealing. She was found to have a positive ANA, high titers of anti-double-stranded DNA antibody, and was ultimately diagnosed with new-onset SLE with hepatic aseptic micro-abscesses. Her symptoms were self-limiting, and she was later started on a low-dose prednisone taper and hydroxychloroquine. This case demonstrates that hepatic involvement, despite normal liver function tests, should be considered in SLE patients presenting with abdominal pain.
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14
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González-Regueiro JA, Cruz-Contreras M, Merayo-Chalico J, Barrera-Vargas A, Ruiz-Margáin A, Campos-Murguía A, Espin-Nasser M, Martínez-Benítez B, Méndez-Cano VH, Macías-Rodríguez RU. Hepatic manifestations in systemic lupus erythematosus. Lupus 2020; 29:813-824. [PMID: 32390496 DOI: 10.1177/0961203320923398] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
Systemic lupus erythematosus (SLE) is a multi-organic autoimmune disease with a wide variety of clinical manifestations. However, hepatic dysfunction is not included in the diagnostic criteria for the disease and has not been recognized properly. The spectrum of hepatic involvement described in these patients ranges from abnormalities in liver function tests (LFTs) to fulminant hepatic failure. Usually, abnormalities in LFTs are only mild and transient, have a hepatocellular pattern and are not related to SLE but rather are mostly drug related. The most frequent finding on liver biopsy is steatosis (non-alcoholic fatty liver disease). Patients do not frequently progress to advanced chronic liver disease, and their outcome is favourable. Those who develop cirrhosis have traditional risk factors, such as other non-SLE-related conditions. In this work, we aim to review hepatic manifestations in patients with SLE, as well as the diagnostic and therapeutic approaches used for different liver diseases in these patients.
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Affiliation(s)
- José A González-Regueiro
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Mariana Cruz-Contreras
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Javier Merayo-Chalico
- Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Ana Barrera-Vargas
- Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Astrid Ruiz-Margáin
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Alejandro Campos-Murguía
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - May Espin-Nasser
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Braulio Martínez-Benítez
- Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Victor H Méndez-Cano
- Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Ricardo U Macías-Rodríguez
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
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15
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Odama UO, Shih DJ, Korbet SM. Sclerosing Peritonitis and Systemic Lupus Erythematosus: A Report of Two Cases. Perit Dial Int 2020. [DOI: 10.1177/089686089901900215] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
Objective To heighten the awareness of a possible association of sclerosing peritonitis in patients with systemic lupus erythematosus (SLE). Methods and Results Over the course of 17 years (from January 1981 to December 1997), 371 patients were treated with continuous ambulatory peritoneal dialysis (CAPD) at Rush–Presbyterian–St Lukes Medical Center. The patients were followed on CAPD for an average of 25 ± 21 (SD) months with a median of 19 months (range 0.2 – 115 months). During this time only 2 (0.5%) patients were diagnosed with sclerosing peritonitis, and both had SLE with ongoing evidence of active disease while on CAPD. With a total of 26 SLE patients being treated with CAPD during the observation period, the prevalence of sclerosing peritonitis can be said to be as high as 8% in this patient population. Conclusion These cases suggest that autoimmune diseases, such as SLE, that are well known to cause immune-mediated serositis may represent an additional factor predisposing to the development of sclerosing peritonitis in patients treated with CAPD.
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Affiliation(s)
- Uninibile O. Odama
- Section of Nephrology, Department of Internal Medicine, Rush–Presbyterian–St. Lukes Medical Center, Chicago, Illinois, U.S.A
| | - David J. Shih
- Section of Nephrology, Department of Internal Medicine, Rush–Presbyterian–St. Lukes Medical Center, Chicago, Illinois, U.S.A
| | - Stephen M. Korbet
- Section of Nephrology, Department of Internal Medicine, Rush–Presbyterian–St. Lukes Medical Center, Chicago, Illinois, U.S.A
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16
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Panigrahy AK, Srinivasan S. Gastrointestinal Manifestations of Systemic Diseases in Critically Ill. Indian J Crit Care Med 2020; 24:S190-S192. [PMID: 33354040 PMCID: PMC7724940 DOI: 10.5005/jp-journals-10071-23610] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
Abstract
Systemic disorders can have gastrointestinal (GI) manifestations which are characterized by nausea, vomiting, diarrhea, constipation, abdominal pain, jaundice, and abnormal liver function tests. These gastrointestinal symptoms can be signs of various immunologic, infectious, and endocrine diseases. Gastrointestinal manifestations can be the first signs and symptoms for which the patient can be admitted in a critical care unit. In this article, we will discuss the GI manifestations of various topical diseases, endocrine diseases, and immunological diseases which are the major bulk of patients in intensive care unit (ICU). How to cite this article: Panigrahy AK, Srinivasan S. Gastrointestinal Manifestations of Systemic Diseases in Critically Ill. Indian J Crit Care Med 2020;24(Suppl 4):S190–S192.
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Affiliation(s)
- Alok K Panigrahy
- Department of Critical Care Medicine, Manipal Hospital, Dwarka, Delhi, India
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17
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Kesar V, Lakhani K, Swaminath A. Unusual cause of lower gastrointestinal bleed and abdominal pain. Frontline Gastroenterol 2019; 11:337-338. [PMID: 32582425 PMCID: PMC7307049 DOI: 10.1136/flgastro-2019-101243] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/09/2019] [Revised: 05/31/2019] [Accepted: 06/04/2019] [Indexed: 02/04/2023] Open
Affiliation(s)
- Varun Kesar
- Gastroenterology, Virginia Tech Carilion School of Medicine, Roanoke, Virginia, USA
| | - Komal Lakhani
- Gastroenterology, Lenox Hill Hospital, New York, New York, USA
| | - Arun Swaminath
- Gastroenterology, Lenox Hill Hospital, New York, New York, USA
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18
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Nango D, Hirose Y, Goto M, Echizen H. Analysis of the Association of Administration of various glucocorticoids with development of acute pancreatitis using US Food and Drug Administration adverse event reporting system (FAERS). J Pharm Health Care Sci 2019; 5:5. [PMID: 30858980 PMCID: PMC6394067 DOI: 10.1186/s40780-019-0134-6] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/25/2018] [Accepted: 01/23/2019] [Indexed: 01/20/2023] Open
Abstract
Background There have been debates about the association between the administration of glucocorticoids and the development of acute pancreatitis, since many anecdotal cases of this adverse event were affected either by concomitant diseases (such as systemic lupus erythematosus, SLE) that may develop acute pancreatitis without glucocorticoid treatment or by co-administered drugs with high risk for the event. The aim of the present study was to explore whether disproportionally elevated signals of developing acute pancreatitis may be detected in patients receiving glucocorticoids as compared those receiving other drugs. Methods We retrieved spontaneously reported cases of acute pancreatitis and clinically related adverse events (target events) from the US Food and Drug Administration Adverse Event Reporting System (FAERS) using 18 preferred terms (PTs). Target drugs studied were cortisol, cortisone, prednisolone, methylprednisolone, triamcinolone, dexamethasone, and betamethasone. After cleaning the data, we calculated reporting odds ratios (RORs) and 95% confidence intervals (CIs) of acute pancreatitis in patients who received one of the glucocorticoids. RORs were calculated for each glucocorticoid using all reported cases irrespective of reporters’ judgement about the contribution of the target drugs to events [i.e., primary suspected medication (PS), secondary suspected medication (SS), concomitant medication (C) and interacting (I)] and using cases with higher certainty of contribution (PS and SS), separately. When the lower limit of 95% CI of a ROR signal exceeded 1.0, the signal was considered statistically significant. Results The RORs (95% CIs) calculated using all reported cases (PS, SS, C, and I) for cortisol (1.68; 1.43–1.98), prednisolone (1.33; 1.19–1.47), methylprednisolone (1.77; 1.55–2.02) were significant, whereas those for other target drugs were insignificant. Using the cases in which target drugs were considered to contribute the events with higher certainty (PS or SS), RORs for prednisolone (1.31; 1.10–1.55), methylprednisolone (1.62; 1.30–2.01), and dexamethasone (1.27; 1.10–1.47) were considered significant, whereas those for others were insignificant. Regarding the performance of PTs for detecting signals (RORs) associated with acute pancreatitis from FAERS database, “pancreatitis acute” gave RORs with higher significance than others, whereas more specific PTs, “haemorrhagic necrotic pancreatitis”, “ischaemic pancreatitis”, “pancreatic necrosis” and “pancreatitis necrotising”, gave RORs with greater magnitude. Conclusion The present study demonstrated that the overrepresentation of signals for acute pancreatitis may be detected for prednisolone, methylprednisolone, and some others in the FAERS database. (372 words)
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Affiliation(s)
- Daisuke Nango
- Departments of Pharmacy, Shin-Yurigaoka General Hospital, 255 Furusawa-tsuko, Asao-ku, Kawasaki, Kanagawa 215-0026 Japan.,3Department of Pharmacotherapy, Meiji Pharmaceutical University, 2-522-1 Noshio, Kiyose, Tokyo, 204-8588 Japan
| | - Yukifumi Hirose
- Departments of Pharmacy, Shin-Yurigaoka General Hospital, 255 Furusawa-tsuko, Asao-ku, Kawasaki, Kanagawa 215-0026 Japan
| | - Makoto Goto
- Kyoto Constella Technologies Co., Ltd., 4th Floor, Kyozome Kaikan, 481 Tourouyama-cho, Nakagyo-ku, Kyoto, 604-8225 Japan
| | - Hirotoshi Echizen
- 3Department of Pharmacotherapy, Meiji Pharmaceutical University, 2-522-1 Noshio, Kiyose, Tokyo, 204-8588 Japan
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19
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Frequency and Type of Hepatic and Gastrointestinal Involvement in Juvenile Systemic Lupus Erythematosus. Autoimmune Dis 2018; 2017:8097273. [PMID: 29318043 PMCID: PMC5727633 DOI: 10.1155/2017/8097273] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/21/2017] [Revised: 11/03/2017] [Accepted: 11/12/2017] [Indexed: 12/15/2022] Open
Abstract
Background Systemic lupus erythematosus (SLE) is a frequent rheumatology disorder among children. Since hepatic involvement is a common systemic manifestation in lupus, the frequency and type of hepatic involvement were determined in pediatric cases of SLE admitted to Children's Medical Hospital from 2005 to 2014. Methods and Patients In this observational case-series study, 138 pediatric cases of SLE were admitted in Children's Medical Center (a pediatric rheumatology referral center in Tehran, Iran) enrolled from 2005 to 2014 and the outcomes, frequency, and type of hepatic involvement were assessed among them. Results Hepatic involvement was reported in 48.55% of total SLE patients. Aspartate aminotransferase (AST), alanine aminotransferase (ALT), and both enzymes higher than normal upper limits were detected in 8.7%, 5%, and 34.7% of lupus patients, respectively. Increased level of liver enzymes was categorized as less than 100, between 100 and 1000, and more than 1000 levels in 23.1%, 23.1%, and 2.1% of cases. The only gastrointestinal involvement in lupus patients contributing to hepatic involvement was gastrointestinal bleeding. Rising in liver enzymes was detected mostly in lupus patients without gastrointestinal bleeding (52.2% without versus 25.8% with gastrointestinal bleeding, P = 0.007). Conclusion Approximately half of the pediatric patients suffering from SLE have hepatic involvement. No significant correlation was observed between various organs involvement and abnormal level of liver enzymes.
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20
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Intussusception merged with systemic lupus erythematosus: one case report and retrospective analysis. Clin Rheumatol 2017; 37:285-288. [PMID: 29079890 DOI: 10.1007/s10067-017-3879-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/01/2017] [Revised: 09/29/2017] [Accepted: 10/15/2017] [Indexed: 10/18/2022]
Abstract
Intussusception is characterized by one segment of the gastrointestinal tract telescoping into the lumen of the adjacent segment; it is rarely reported in systemic lupus erythematosus (SLE), and the condition can be threatening. Only four cases of intussusception with SLE have been reported in literature. Here, we describe a new case of a patient with ileocecal intussusception merged with SLE, who was diagnosed using abdominal computed tomography and successfully treated with high-dose intravenous immunoglobulin (IVIG) and pulse methylprednisolone.
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21
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Lee HA, Shim HG, Seo YH, Choi SJ, Lee BJ, Lee YH, Ji JD, Kim JH, Song GG. Panenteritis as an Initial Presentation of Systemic Lupus Erythematosus. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2017; 67:107-11. [PMID: 26907488 DOI: 10.4166/kjg.2016.67.2.107] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/14/2023]
Abstract
Lupus enteritis is a rare, severe complication of systemic lupus erythematosus (SLE), needing prompt diagnosis and proper management. However, SLE rarely presents as lupus enteritis at the time of initial diagnosis. Thus, delayed diagnosis and misdiagnosis are common. We report a case of a 25-year-old woman with lupus panenteritis. The patient had multiple hospitalizations for abdominal pain, nausea, and diarrhea, initially without any other symptoms suggestive of SLE, but was later observed to have malar rash and oral ulcers. Laboratory investigations were compatible with SLE, including positive antinuclear antibody (1:320) with speckled pattern. CT revealed diffuse hypodense submucosal thickening of the stomach, the entire small bowel, colon, appendix, and rectum. Treatment with high-dose corticosteroids followed by maintenance therapy with mycophenolate mofetil, hydroxychloroquine, and azathioprine resulted in clinical improvement. Diagnosis of lupus enteritis requires a high index of suspicion given the low incidence and nonspecific clinical findings.
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Affiliation(s)
- Han Ah Lee
- Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
| | - Hye Gi Shim
- Department of Internal Medicine, Montefiore Medical Center, The University Hospital for Albert Einstein College of Medicine, Bronx, NY, USA
| | - Young Ho Seo
- Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
| | - Sung Jae Choi
- Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
| | - Beom Jae Lee
- Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
| | - Young Ho Lee
- Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
| | - Jong Dae Ji
- Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
| | - Jae-Hoon Kim
- Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
| | - Gwan Gyu Song
- Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
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22
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Bert J, Gertner E. Lupus Gastrointestinal Tract Vasculopathy: Lupus "Enteritis" Involving the Entire Gastrointestinal Tract from Esophagus to Rectum. Case Rep Gastroenterol 2017; 11:48-53. [PMID: 28203138 PMCID: PMC5301120 DOI: 10.1159/000455826] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/22/2016] [Accepted: 12/20/2016] [Indexed: 12/15/2022] Open
Abstract
Gastrointestinal symptoms are very common in systemic lupus erythematosus (SLE). Lupus "enteritis" is very responsive to treatment but can have devastating consequences if not detected. Most descriptions of enteritis involve the small and large bowel. This is the first report of lupus "enteritis" involving the entire gastrointestinal tract from the esophagus and stomach to the rectum. Lupus "enteritis" is another cause of upper gastrointestinal involvement in SLE (involving even the esophagus and stomach) in addition to involvement of the lower intestinal tract.
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Affiliation(s)
- Joseph Bert
- Section of Rheumatology, Regions Hospital, St. Paul, MN, USA
- Division of Rheumatology, University of Minnesota Medical School, Minneapolis, MN, USA
| | - Elie Gertner
- Section of Rheumatology, Regions Hospital, St. Paul, MN, USA
- Division of Rheumatology, University of Minnesota Medical School, Minneapolis, MN, USA
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23
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Anquetil C, Stavris C, Chanson N, Lambert M, Hachulla E, Launay D, Hatron PY. [Hemorrhagic shock revealing multiple digestive microaneurysms in a patient with systemic lupus erythematosus: Case report and literature review]. Rev Med Interne 2016; 38:56-60. [PMID: 27036226 DOI: 10.1016/j.revmed.2016.02.020] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/02/2015] [Revised: 02/23/2016] [Accepted: 02/27/2016] [Indexed: 01/17/2023]
Abstract
INTRODUCTION The vascular disorders in systemic lupus erythematosus (SLE) result from various mechanisms and presentations (inflammatory disease or vasculitis, atherosclerosis). CASE REPORT We report on a 34-year-old man with cutaneous, articular, neurological and nephrologic SLE. He presented with catastrophic haemorrhage on microaneurysm rupture of the left hepatic artery. After blood transfusions and immunosuppressive treatments, his condition improves. CONCLUSION Uncommon complication in SLE patients, digestive vasculitis with microaneurysms may occur as in polyarteritis nodosa. In the literature, we identified 10 additional cases of hepatic microaneurysms in SLE patients. The main issue is an earlier diagnosis in order to give appropriate treatment and improve prognosis.
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Affiliation(s)
- C Anquetil
- UFR de médecine, université de Lille, 59000 Lille, France; Service de médecine interne et immunologie clinique, hôpital Claude-Huriez, CHRU de Lille, pôle spécialités médicales et gérontologie, rue Michel-Polonovski, 59037 Lille cedex, France; Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), 59037 Lille cedex, France; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMInENT), 59000 Lille, France
| | - C Stavris
- UFR de médecine, université de Lille, 59000 Lille, France; Service de médecine interne et immunologie clinique, hôpital Claude-Huriez, CHRU de Lille, pôle spécialités médicales et gérontologie, rue Michel-Polonovski, 59037 Lille cedex, France; Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), 59037 Lille cedex, France; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMInENT), 59000 Lille, France
| | - N Chanson
- UFR de médecine, université de Lille, 59000 Lille, France; Service de médecine interne et immunologie clinique, hôpital Claude-Huriez, CHRU de Lille, pôle spécialités médicales et gérontologie, rue Michel-Polonovski, 59037 Lille cedex, France; Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), 59037 Lille cedex, France; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMInENT), 59000 Lille, France
| | - M Lambert
- UFR de médecine, université de Lille, 59000 Lille, France; Service de médecine interne et immunologie clinique, hôpital Claude-Huriez, CHRU de Lille, pôle spécialités médicales et gérontologie, rue Michel-Polonovski, 59037 Lille cedex, France; Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), 59037 Lille cedex, France; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMInENT), 59000 Lille, France
| | - E Hachulla
- UFR de médecine, université de Lille, 59000 Lille, France; Service de médecine interne et immunologie clinique, hôpital Claude-Huriez, CHRU de Lille, pôle spécialités médicales et gérontologie, rue Michel-Polonovski, 59037 Lille cedex, France; Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), 59037 Lille cedex, France; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMInENT), 59000 Lille, France; LIRIC, UMR 995, EA2686, 59000 Lille, France
| | - D Launay
- UFR de médecine, université de Lille, 59000 Lille, France; Service de médecine interne et immunologie clinique, hôpital Claude-Huriez, CHRU de Lille, pôle spécialités médicales et gérontologie, rue Michel-Polonovski, 59037 Lille cedex, France; Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), 59037 Lille cedex, France; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMInENT), 59000 Lille, France; LIRIC, UMR 995, EA2686, 59000 Lille, France.
| | - P Y Hatron
- UFR de médecine, université de Lille, 59000 Lille, France; Service de médecine interne et immunologie clinique, hôpital Claude-Huriez, CHRU de Lille, pôle spécialités médicales et gérontologie, rue Michel-Polonovski, 59037 Lille cedex, France; Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), 59037 Lille cedex, France; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMInENT), 59000 Lille, France
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A case of systemic lupus erythematosus presenting with an acute abdomen: successful treatment with steroid. Case Rep Med 2015; 2014:318939. [PMID: 25580129 PMCID: PMC4279847 DOI: 10.1155/2014/318939] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2014] [Accepted: 11/30/2014] [Indexed: 11/18/2022] Open
Abstract
Abdominal pain continues to pose diagnostic challenges for emergency clinicians. A 56-year-old Japanese woman was referred to our hospital due to severe abdominal pain which presented as occasional epigastric pain five months before and intermittent abdominal pain. She had a past history of ileus twice, for both of which laparotomy was performed without an alimentary tract resection. The wall thickening with marked three-wall structure from terminal ileum to sigmoid colon was seen and bladder wall was irregularly thick and enhanced irregularly. Among the differential diagnosis of the acute abdomen, autoimmune diseases were suspected, especially lupus erythematosus and Henoch-Schönlein purpura. On the second day of admission, abdominal pain worsened. The results of examinations of antinuclear antibody, anti-double-stranded DNA antibody, ANCA, and the complements were not obtained at that time; however, we started 1-g steroid pulse treatment for three days with success. With the results obtained later, the patient was given a diagnosis of probable systemic lupus erythematosus (SLE). The present case shows that SLE can present with acute abdomen and should be included in the wide range of the differential diagnosis of acute abdomen.
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Rodriguez EA, Sussman DA, Rodriguez VR. Systemic lupus erythematosus pancreatitis: an uncommon presentation of a common disease. AMERICAN JOURNAL OF CASE REPORTS 2014; 15:501-3. [PMID: 25399483 PMCID: PMC4237072 DOI: 10.12659/ajcr.891281] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
Patient: Female, 21 Final Diagnosis: Systemic lupus erythematosus pancreatitis Symptoms: Abdominal pain Medication: — Clinical Procedure: — Specialty: Gastroenterology and Hepatology
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Affiliation(s)
- Eduardo A Rodriguez
- Internal Medicine Resident, University of Miami Palm Beach Regional Campus, Atlantis, USA
| | - Daniel A Sussman
- Department of Internal Medicine, Division of Gastroenterology, University of Miami, Miami, USA
| | - Vanessa R Rodriguez
- Department of Gastroenterology, Sergio Bernales National Hospital, Lima, Peru
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García Carrasco M, Mendoza Pinto C, López Colombo A, Méndez Martínez S, Andari Sawaya R, Muñoz Guarneros M, Vázquez De Lara L, Lescas Hernández M, Montiel Jarquín A, Ramos Álvarez G, Schmulson M. Irritable bowel syndrome-type symptoms in female patients with mild systemic lupus erythematosus: frequency, related factors and quality of life. Neurogastroenterol Motil 2013; 25:958-66. [PMID: 24112121 DOI: 10.1111/nmo.12230] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/09/2013] [Accepted: 08/14/2013] [Indexed: 02/08/2023]
Abstract
BACKGROUND Irritable bowel syndrome (IBS) impairs quality of life (HRQOL), as does systemic lupus erythematosus (SLE). Both are more common in women and are associated with fibromyalgia (FM). However, the relationship between IBS and SLE and its impact on HRQOL has not been explored. Therefore, we aimed to study the frequency and features likely to influence the presence of IBS-type symptoms in SLE and their impact on HRQOL. METHODS Female patients with SLE were studied. The presence of IBS-type symptoms and bowel habit subtype were established by Rome III criteria and HRQOL was assessed using the SF-36. Fibromyalgia and depression were assessed using the American College of Rheumatology criteria and CES-D scale, respectively. KEY RESULTS A total of 105 consecutive patients (43.62 ± 11.34 years old) were included; 48.6% had IBS-type symptoms (SLE+IBS) and were classified as IBS-C: 23.5%, IBS-D: 37.3%, and IBS-M: 39.2%. In addition, 23.8% had FM. SLE+IBS vs Non-IBS SLE patients had higher SLE activity scores (2.55 ± 1.65 vs 1.74 ± 2.19; p = 0.03), were more likely to have FM (33.0% vs 14.8%; p = 0.02) and depression (41.1% vs 25.9%, p = 0.04). Logistic multivariate analysis showed that IBS-type symptoms were associated with FM (OR = 2.85, 95% CI: 1.11-7.43) and depression (OR = 1.07, 95% CI: 1.02-1.13). Finally, SLE+IBS vs Non-IBS SLE patients had lower SF-36 scores (49.65 ± 18.57 vs 62.67 ± 18.14; p = 0.02). CONCLUSIONS & INFERENCES IBS-type symptoms are highly prevalent among women with SLE and are associated with FM and depression. SLE+IBS patients had worse HRQOL vs Non-IBS SLE, independently of FM status. We suggest that treating IBS symptoms may improve HRQOL in women with SLE.
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Affiliation(s)
- M García Carrasco
- Systemic Autoimmune Diseases Research Unit, Hospital General Regional No. 36, Instituto Mexicano del Seguro Social, Puebla, Puebla, México; Centro de Investigación Biomédica de Oriente, Instituto Mexicano del Seguro Social, Metepec, Puebla, México; Immunology and Rheumatology, Medicine School, Benemérita Universidad Autónoma de Puebla, Puebla, Puebla, México
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Thomaidis T, Goetz M, Gregor SP, Hoffman A, Kouroumalis E, Moehler M, Galle PR, Schwarting A, Kiesslich R. Irritable bowel syndrome and organic diseases: A comparative analysis of esophageal motility. World J Gastroenterol 2013; 19:6408-6415. [PMID: 24151359 PMCID: PMC3801311 DOI: 10.3748/wjg.v19.i38.6408] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/14/2012] [Revised: 01/24/2013] [Accepted: 05/10/2013] [Indexed: 02/06/2023] Open
Abstract
AIM: To assess the esophageal motility in patients with irritable bowel syndrome (IBS) and to compare those with patients with autoimmune disorders.
METHODS: 15 patients with IBS, 22 with systemic lupus erythematosus (SLE) and 19 with systemic sclerosis (SSc) were prospectively selected from a total of 115 patients at a single university centre and esophageal motility was analysed using standard manometry (Mui Scientific PIP-4-8SS). All patients underwent esophago-gastro-duodenoscopy before entering the study so that only patients with normal endoscopic findings were included in the current study. All patients underwent a complete physical, blood biochemistry and urinary examination. The grade of dysphagia was determined for each patient in accordance to the intensity and frequency of the presented esophageal symptoms. Furthermore, disease activity scores (SLEDAI and modified Rodnan score) were obtained for patients with autoimmune diseases. Outcome parameter: A correlation coefficient was calculated between amplitudes, velocity and duration of the peristaltic waves throughout esophagus and patients’ dysphagia for all three groups.
RESULTS: There was no statistical difference in the standard blood biochemistry and urinary analysis in all three groups. Patients with IBS showed similar pathologic dysphagia scores compared to patients with SLE and SSc. The mean value of dysphagia score was in IBS group 7.3, in SLE group 6.73 and in SSc group 7.56 with a P-value > 0.05. However, the manometric patterns were different. IBS patients showed during esophageal manometry peristaltic amplitudes at the proximal part of esophagus greater than 60 mmHg in 46% of the patients, which was significant higher in comparison to the SLE (11.8%) and SSc-Group (0%, P = 0.003). Furthermore, IBS patients showed lower mean resting pressure of the distal esophagus sphincter (Lower esophageal sphincter, 22 mmHg) when compared with SLE (28 mmHg, P = 0.037) and SSc (26 mmHg, P = 0.052). 23.5% of patients with SLE showed amplitudes greater as 160 mmHg in the distal esophagus (IBS and SSc: 0%) whereas 29.4% amplitudes greater as 100 mmHg in the middle one (IBS: 16.7%, SSc: 5.9% respectively, P = 0.006). Patients with SSc demonstrated, as expected, in almost half of the cases reduced peristalsis or even aperistalsis in the lower two thirds of the esophagus. SSc patients demonstrated a negative correlation coefficient between dysphagia score, amplitude and velocity of peristaltic activity at middle and lower esophagus [r = -0.6, P < 0.05].
CONCLUSION: IBS patients have comparable dysphagia-scores as patients with autoimmune disorders. The different manometric patterns might allow differentiating esophageal symptoms based on IBS from other organic diseases.
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MESH Headings
- Adult
- Autoimmunity
- Deglutition
- Deglutition Disorders/diagnosis
- Deglutition Disorders/etiology
- Deglutition Disorders/physiopathology
- Endoscopy, Digestive System
- Esophageal Sphincter, Lower/physiopathology
- Esophagus/physiopathology
- Female
- Germany
- Humans
- Irritable Bowel Syndrome/complications
- Irritable Bowel Syndrome/diagnosis
- Irritable Bowel Syndrome/physiopathology
- Lupus Erythematosus, Systemic/complications
- Lupus Erythematosus, Systemic/diagnosis
- Lupus Erythematosus, Systemic/immunology
- Lupus Erythematosus, Systemic/physiopathology
- Male
- Manometry
- Middle Aged
- Peristalsis
- Prospective Studies
- Scleroderma, Systemic/complications
- Scleroderma, Systemic/diagnosis
- Scleroderma, Systemic/immunology
- Scleroderma, Systemic/physiopathology
- Severity of Illness Index
- Surveys and Questionnaires
- Time Factors
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28
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Smith LW, Petri M. Lupus enteritis: an uncommon manifestation of systemic lupus erythematosus. J Clin Rheumatol 2013; 19:84-6. [PMID: 23364660 DOI: 10.1097/rhu.0b013e318284794e] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
We report the case of a 25-year-old Iraqi woman who had multiple hospitalizations at an outside hospital for abdominal pain, nausea, and diarrhea without any evidence of systemic lupus erythematosus. Laboratory investigations finally showed a positive antinuclear antibody (1280), positive anti-dsDNA, anti-β2 glycoprotein I, low complement, positive Coombs tests, and leukopenia. A kidney biopsy showed ISN class II lupus nephritis. An ileal biopsy and angiogram were unremarkable. A computed tomography showed marked and dramatic bowel edema involving the small and large bowel ("target sign"), dilatation of intestinal segments, engorgement of mesenteric vessels ("comb sign"), and increased attenuation of mesenteric fat. These cardinal signs on computed tomography scan led to the correct diagnosis of lupus enteritis. Treatment was commenced with high-dose corticosteroids followed by mycophenolate mofetil, hydroxychloroquine, and then oral cyclophosphamide, but failed. The patient was eventually treated with the Euro-Lupus intravenous cyclophosphamide regimen, which resulted in significant clinical and radiological resolution.
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Affiliation(s)
- Lanaya Williams Smith
- Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, MD, USA.
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Önder FO, Yürekli ÖT, Öztaş E, Kalkan İH, Köksal AŞ, Akdoğan M, Şaşmaz N, Kayaçetin E. Features of systemic lupus erythematosus in patients with autoimmune hepatitis. Rheumatol Int 2012; 33:1581-5. [DOI: 10.1007/s00296-012-2607-x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2012] [Accepted: 12/08/2012] [Indexed: 10/27/2022]
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30
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Goh YP, Naidoo P, Ngian GS. Imaging of systemic lupus erythematosus. Part II: gastrointestinal, renal, and musculoskeletal manifestations. Clin Radiol 2012; 68:192-202. [PMID: 22901453 DOI: 10.1016/j.crad.2012.06.109] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2011] [Revised: 05/27/2012] [Accepted: 06/01/2012] [Indexed: 12/22/2022]
Abstract
Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease that has a relapsing and remitting course. It has a wide range of presentations with various organ manifestations. In this review, we have compiled the radiological findings of gastrointestinal, renal, and musculoskeletal manifestations of SLE.
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Affiliation(s)
- Y P Goh
- Department of Diagnostic Imaging, Monash Medical Centre, Australia.
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31
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Chng HH, Tan BE, Teh CL, Lian TY. Major gastrointestinal manifestations in lupus patients in Asia: lupus enteritis, intestinal pseudo-obstruction, and protein-losing gastroenteropathy. Lupus 2011; 19:1404-13. [PMID: 20947549 DOI: 10.1177/0961203310374337] [Citation(s) in RCA: 43] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
Gastrointestinal (GI) symptoms are common in patients with systemic lupus erythematosus (SLE) and may be due to the disease itself, side-effects of medications, or non-SLE causes. However, GI manifestations of lupus attract far less attention than the other major organ involvements, are infrequently reviewed and rarely documented in published lupus databases or cohort studies including those from countries in Asia. According to three reports from two countries in Asia, the cumulative prevalence of SLE GI manifestations range from 3.8% to 18%. In this review, we focus on three major GI manifestations in patients from Asian countries: lupus enteritis, intestinal pseudo-obstruction, and protein-losing gastroenteropathy, for which early recognition improves outcome and reduces morbidity and mortality.
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Affiliation(s)
- H H Chng
- Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore.
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Abstract
Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune connective tissue disease with protean manifestations. Most often it presents with mucocutaneous, musculoskeletal or renal involvement. In comparison, gastrointestinal (GI) manifestations of SLE are far less common. The case presented here highlights the differential diagnosis of GI manifestations of SLE that range from non-life-threatening to serious life-threatening complications, including some of the complications of on-going drug treatments. While some of them present as 'acute abdomen', others are more subacute or chronic, yet serious enough to be life-threatening. The serious GI manifestations of SLE include mesenteric vasculitis causing perforation or hemorrhage with peritonitis, acute pancreatitis and intestinal pseudo-obstruction. The patient in this paper had clinical features, imaging findings and laboratory parameters that helped the treating physician to narrow down the diagnostic possibilities and finally, in making the diagnosis of lupus-pancreatitis. She was treated with intravenous 'bolus' (i.v.-pulse) methylprednisolone for 3 days, i.v.-pulse cyclophosphamide 750 mg (one dose) along with oral methylprednisolone and other supportive measures including blood transfusions. This led to prompt and complete recovery.
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Affiliation(s)
- Anand N Malaviya
- Department of Rheumatology, ISIC Superspeciality Hospital, Vasant Kunj, New Delhi, India.
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33
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de Carvalho JF. Mesenteric vasculitis in a systemic lupus erythematosus patient with a low sledai: an uncommon presentation. Clinics (Sao Paulo) 2010; 65:337-40. [PMID: 20360927 PMCID: PMC2845777 DOI: 10.1590/s1807-59322010000300016] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
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35
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Nguyen VA, Pachinger O, Jaschke W, Tzankov A, Semenitz B, Sepp N. Severe mesenteric and coronary vasculitis: a late and uncommon complication in a patient with chronic discoid lupus erythematosus. Scand J Rheumatol 2009; 35:491-4. [PMID: 17343262 DOI: 10.1080/03009740600709899] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
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36
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Abdominal Manifestations of Systemic Lupus Erythematosus: Spectrum of Imaging Findings. Can Assoc Radiol J 2009; 60:121-32. [DOI: 10.1016/j.carj.2009.06.001] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022] Open
Abstract
Systemic lupus erythematosus is an immune-mediated syndrome in which the immune response is to non–organ-specific antigens, and virtually every organ in the abdominal cavity may become involved. Only renal involvement forms part of the diagnostic criteria, however, a combination of typically nonspecific findings, including peritoneal surface, enteric, renal, renal tract, pancreatic, adrenal, hepatobiliary, and splenic manifestations, should be looked for in patients with known lupus or other connective tissue disease who are undergoing abdominal imaging and may suggest the diagnosis in patients presenting with an acute abdomen. Our work presents the spectrum of imaging findings of abdominal manifestations of systemic lupus erythematosus.
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37
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Kell DB. Iron behaving badly: inappropriate iron chelation as a major contributor to the aetiology of vascular and other progressive inflammatory and degenerative diseases. BMC Med Genomics 2009; 2:2. [PMID: 19133145 PMCID: PMC2672098 DOI: 10.1186/1755-8794-2-2] [Citation(s) in RCA: 376] [Impact Index Per Article: 23.5] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2008] [Accepted: 01/08/2009] [Indexed: 01/19/2023] Open
Abstract
BACKGROUND The production of peroxide and superoxide is an inevitable consequence of aerobic metabolism, and while these particular 'reactive oxygen species' (ROSs) can exhibit a number of biological effects, they are not of themselves excessively reactive and thus they are not especially damaging at physiological concentrations. However, their reactions with poorly liganded iron species can lead to the catalytic production of the very reactive and dangerous hydroxyl radical, which is exceptionally damaging, and a major cause of chronic inflammation. REVIEW We review the considerable and wide-ranging evidence for the involvement of this combination of (su)peroxide and poorly liganded iron in a large number of physiological and indeed pathological processes and inflammatory disorders, especially those involving the progressive degradation of cellular and organismal performance. These diseases share a great many similarities and thus might be considered to have a common cause (i.e. iron-catalysed free radical and especially hydroxyl radical generation).The studies reviewed include those focused on a series of cardiovascular, metabolic and neurological diseases, where iron can be found at the sites of plaques and lesions, as well as studies showing the significance of iron to aging and longevity. The effective chelation of iron by natural or synthetic ligands is thus of major physiological (and potentially therapeutic) importance. As systems properties, we need to recognise that physiological observables have multiple molecular causes, and studying them in isolation leads to inconsistent patterns of apparent causality when it is the simultaneous combination of multiple factors that is responsible.This explains, for instance, the decidedly mixed effects of antioxidants that have been observed, since in some circumstances (especially the presence of poorly liganded iron) molecules that are nominally antioxidants can actually act as pro-oxidants. The reduction of redox stress thus requires suitable levels of both antioxidants and effective iron chelators. Some polyphenolic antioxidants may serve both roles.Understanding the exact speciation and liganding of iron in all its states is thus crucial to separating its various pro- and anti-inflammatory activities. Redox stress, innate immunity and pro- (and some anti-)inflammatory cytokines are linked in particular via signalling pathways involving NF-kappaB and p38, with the oxidative roles of iron here seemingly involved upstream of the IkappaB kinase (IKK) reaction. In a number of cases it is possible to identify mechanisms by which ROSs and poorly liganded iron act synergistically and autocatalytically, leading to 'runaway' reactions that are hard to control unless one tackles multiple sites of action simultaneously. Some molecules such as statins and erythropoietin, not traditionally associated with anti-inflammatory activity, do indeed have 'pleiotropic' anti-inflammatory effects that may be of benefit here. CONCLUSION Overall we argue, by synthesising a widely dispersed literature, that the role of poorly liganded iron has been rather underappreciated in the past, and that in combination with peroxide and superoxide its activity underpins the behaviour of a great many physiological processes that degrade over time. Understanding these requires an integrative, systems-level approach that may lead to novel therapeutic targets.
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Affiliation(s)
- Douglas B Kell
- School of Chemistry and Manchester Interdisciplinary Biocentre, The University of Manchester, 131 Princess St, Manchester, M1 7DN, UK.
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Carducci M, Calcaterra R, Mussi A, Franco G, Morrone A. Acute pancreatitis as initial manifestation of systemic lupus erythematosus and subacute cutaneous lupus erythematosus: report of two cases. Lupus 2008; 17:695-7. [DOI: 10.1177/0961203308090429] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Affiliation(s)
- M Carducci
- San Gallicano Institute IRCCS, Rome, Italy
| | | | - A Mussi
- San Gallicano Institute IRCCS, Rome, Italy
| | - G Franco
- San Gallicano Institute IRCCS, Rome, Italy
| | - A Morrone
- San Gallicano Institute IRCCS, Rome, Italy
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Mechanical bowel strangulation mimicking mesenteric vasculitis in a systemic lupus erythematosus patient. South Med J 2008; 101:436-8. [PMID: 18360324 DOI: 10.1097/smj.0b013e318167d6a8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
A case of systemic lupus erythematosus with jaundice and vague abdominal pain which did not respond to steroid pulse therapy is presented. Noninvasive examinations and imaging studies showed ileus. Two weeks later, an emergency laparotomy was performed because of severe refractory abdominal pain and hemodynamic decompensation. An ischemic part of the terminal ileum was resected. It was pathologically determined to be ischemic bowel disease because of mechanical strangulation resulting from adhesion band, but without evidence of vasculitis, atherosclerotic change, or thrombosis. After intensive postoperative care, the patient gradually recovered. This unusual case shows that nonlupus-related mechanical strangulation should be considered in the differential diagnosis of acute abdomen in lupus patients, particularly in those who have received steroid therapy or have a history of previous abdominal operation.
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40
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Affiliation(s)
- Satish Gopal
- Department of Internal Medicine, Norwalk Hospital, Norwalk, Connecticut, USA.
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41
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Kim DH, Na HJ, Choi YR, Nam DH, Lee CH, Park SM, Kang W. A Case of Extensive Involvement of Lupus Enteritis, from Small Bowel to Rectum. ACTA ACUST UNITED AC 2007. [DOI: 10.4078/jkra.2007.14.3.274] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Affiliation(s)
- Do Hyun Kim
- Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea
| | - Hyoung Jung Na
- Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea
| | - Yu-ri Choi
- Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea
| | - Dong Hyuk Nam
- Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea
| | - Chan Hee Lee
- Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea
| | - Su-Mi Park
- Department of Radiology, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea
| | - Wonseok Kang
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
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42
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Nakashima H, Karimine N, Asoh T, Ueo H, Kohnoe S, Mori M. Risk Factors of Abdominal Surgery in Patients with Collagen Diseases. Am Surg 2006. [DOI: 10.1177/000313480607200916] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Patients with collagen diseases have been reported to demonstrate a greater risk when undergoing surgical operations. To determine the risk factors in abdominal surgery for patients with collagen diseases, 32 patients with collagen diseases who underwent abdominal surgery were analyzed for their clinical features and surgical results by comparing 26 cases from the favorable prognosis group (Group A) and 6 cases resulting in hospital death (Group B). The analysis revealed that emergent operations tended to result in worse outcomes ( P = 0.011) than elective operations and that cases undergoing operations for collagen disease-related problems, including intestinal perforation and acute pancreatitis, also showed a worse postoperative course than those who underwent operations for problems unrelated to collagen diseases, such as carcinomas and cholelithiasis ( P = 0.0006). The dose of steroids administered at the time of operation was also significantly higher in Group B than in Group A ( P = 0.03). These results suggested that the patients with collagen diseases should be followed periodically not only for the primary disease but also for any potential surgical diseases to identify such diseases at an early stage and to avoid an emergent operation, and that patients treated with high doses of steroids also need intensive care after abdominal surgery.
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Affiliation(s)
- Hideaki Nakashima
- Department of Innovative Medical Technology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
- Department of Surgery, Medical Institute of Bioregulation, Kyushu University, Beppu, Japan
| | | | | | - Hiroaki Ueo
- Department of Surgery, Wada Hospital, Usa, Japan
| | - Shunji Kohnoe
- Department of Surgery, Fukuoka Dental College, Fukuoka, Japan
| | - Masaki Mori
- Department of Surgery, Medical Institute of Bioregulation, Kyushu University, Beppu, Japan
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43
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Nitzan O, Elias M, Saliba WR. Systemic lupus erythematosus and inflammatory bowel disease. Eur J Intern Med 2006; 17:313-8. [PMID: 16864003 DOI: 10.1016/j.ejim.2006.02.001] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/29/2005] [Revised: 01/15/2006] [Accepted: 02/03/2006] [Indexed: 01/18/2023]
Abstract
We hereby summarize a case reported by the authors, as well as all of the previously reported cases, of patients suffering from both systemic lupus erythematosus (SLE) and inflammatory bowel disease (IBD) that have been published in the English literature. In most cases, SLE was diagnosed prior to IBD, and the latter was of the ulcerative colitis subtype. Interestingly, the first disease diagnosed was almost never active at the time the second disease manifested itself. Patients with both diseases tended to have less photosensitivity, less arthritis, and less serositis than patients with SLE alone. There were no cases with neurological disorders or overt nephritis. All of these patients had anti-dsDNA and there was a tendency towards more anemia and thrombocytopenia. These patients had a relatively benign course with no flare-ups of lupus during follow-up and a favorable course of their bowel disease as well.
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Affiliation(s)
- O Nitzan
- Department of Internal Medicine C, Hae'meK Medical Center, Afula 18101, Affiliated with the Technion-Israel Institute of Technology, Faculty of Medicine, Haifa, Israel
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44
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45
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Hsu CL, Chen KY, Yeh PS, Hsu YL, Chang HT, Shau WY, Yu CL, Yang PC. Outcome and prognostic factors in critically ill patients with systemic lupus erythematosus: a retrospective study. CRITICAL CARE : THE OFFICIAL JOURNAL OF THE CRITICAL CARE FORUM 2005; 9:R177-83. [PMID: 15987388 PMCID: PMC1175871 DOI: 10.1186/cc3481] [Citation(s) in RCA: 46] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 10/25/2004] [Revised: 12/16/2004] [Accepted: 02/01/2004] [Indexed: 11/15/2022]
Abstract
Introduction Systemic lupus erythematosus (SLE) is an archetypal autoimmune disease, involving multiple organ systems with varying course and prognosis. However, there is a paucity of clinical data regarding prognostic factors in SLE patients admitted to the intensive care unit (ICU). Methods From January 1992 to December 2000, all patients admitted to the ICU with a diagnosis of SLE were included. Patients were excluded if the diagnosis of SLE was established at or after ICU admission. A multivariate logistic regression model was applied using Acute Physiology and Chronic Health Evaluation II scores and variables that were at least moderately associated (P < 0.2) with survival in the univariate analysis. Results A total of 51 patients meeting the criteria were included. The mortality rate was 47%. The most common cause of admission was pneumonia with acute respiratory distress syndrome. Multivariate logistic regression analysis showed that intracranial haemorrhage occurring while the patient was in the ICU (relative risk = 18.68), complicating gastrointestinal bleeding (relative risk = 6.97) and concurrent septic shock (relative risk = 77.06) were associated with greater risk of dying, whereas causes of ICU admission and Acute Physiology and Chronic Health Evaluation II score were not significantly associated with death. Conclusion The mortality rate in critically ill SLE patients was high. Gastrointestinal bleeding, intracranial haemorrhage and septic shock were significant prognostic factors in SLE patients admitted to the ICU.
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Affiliation(s)
- Chia-Lin Hsu
- Division of Pulmonary Medicine, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
| | - Kuan-Yu Chen
- Division of Pulmonary Medicine, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
| | - Pu-Sheng Yeh
- Division of Pulmonary Medicine, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
| | - Yeong-Long Hsu
- Division of Pulmonary Medicine, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
| | - Hou-Tai Chang
- Division of Pulmonary Medicine, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
| | - Wen-Yi Shau
- Assistant Professor, Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan
| | - Chia-Li Yu
- Professor, Division of Rheumatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
| | - Pan-Chyr Yang
- Professor, Division of Pulmonary Medicine, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
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46
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Lian TY, Edwards CJ, Chan SP, Chng HH. Reversible acute gastrointestinal syndrome associated with active systemic lupus erythematosus in patients admitted to hospital. Lupus 2005; 12:612-6. [PMID: 12945720 DOI: 10.1191/0961203303lu433oa] [Citation(s) in RCA: 45] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
Patients with systemic lupus erythematosus (SLE) frequently have gastrointestinal (GI) symptoms. These are usually self-limiting and related to treatment side-effects or concurrent illness. However, abdominal pain may be due to bowel ischaemia which can lead to infarction and perforation. The likelihood of these serious events is increased in individuals with pain severe enough to require assessment in hospital or a SLEDAI score > 5. This paper describes a group of patients with active SLE and GI symptoms severe enough to require admission to hospital using a retrospective review of 52 SLE patients admitted to hospital with acute abdominal symptoms. The results showed that abdominal pain (87%), vomiting (82%) and diarrhoea (67%) had been present for a mean of 4.4 +/- 6.5 days and SLEDAI score was > or = 4 in 83% of patients. CT scanning showed evidence of serositis and bowel involvement in 63% of patients who underwent this investigation. Intravenous (iv) fluids were used in 87%, parenteral steroids in 90% and iv cyclophosphamide in 31%. Most (n = 51) were discharged well. Recurrence of GI symptoms occurred in 12 patients. The conclusions are that active SLE may manifest as an acute gastrointestinal syndrome. Early diagnosis, bowel rest, supportive medical therapy and treatment with corticosteroids and/or immunosupressives can result in a good outcome.
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Affiliation(s)
- T Y Lian
- Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore.
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Abstract
Vasculitis in connective tissue diseases is not an uncommon complication. Vasculitis complicates both rheumatoid arthritis and systemic lupus erythematosis (SLE) in about 4% of cases. Cutaneous lesions, representing small-vessel involvement, are most common; however, widespread, necrotizing visceral medium-and large-vessel involvement, mimicking primary vasculitic syndromes, may also occur. Connective tissue disease-associated vasculitis is separated from primary vasculitis syndromes in classification schemes. Granulomatous large-vessel disease does not occur in connective tissue diseases, suggesting a different pathogenesis. In most disorders, the etiology of vascular inflammation in not completely understood, but basic pathogenic mechanisms can often be distinguished. The role of immune complexes in the inflammatory manifestations of SLE is recognized, and other pathogenic factors such as antineutrophil cytoplasmic antibodies, common in other vasculitides, are infrequent. A diverse spectrum of clinical features, due to inflammatory involvement of arterial and venous vessels of all sizes, characterize several connective tissue diseases including Behçet's disease and SLE. The recognition of disease manifestations due to vasculitis in these disorders has important implications for treatment and may be critical to reduce morbidity and mortality.
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Affiliation(s)
- Kenneth T Calamia
- Division of Rheumatology, Mayo Clinic, Jacksonville, Florida 32224, USA.
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48
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Szollosi Z, Tarr T, Kiss E. Crohn disease versus systemic lupus erythematosus: an autopsy diagnosis of predominantly extraintestinal Crohn disease. Inflamm Bowel Dis 2004; 10:702-3. [PMID: 15472542 DOI: 10.1097/00054725-200409000-00036] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/09/2022]
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49
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Kaneko Y, Hirakata M, Suwa A, Satoh S, Nojima T, Ikeda Y, Mimori T. Systemic lupus erythematosus associated with recurrent lupus enteritis and peritonitis. Clin Rheumatol 2004; 23:351-4. [PMID: 15293099 DOI: 10.1007/s10067-004-0882-y] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2003] [Accepted: 12/30/2003] [Indexed: 10/26/2022]
Abstract
We describe the case of a 41-year-old woman with systemic lupus erythematosus (SLE) who suffered from repeated reversible lupus enteritis characterized by marked edematous thickening of the small intestine. Ultrasonography (US) and computed tomography (CT) manifested as an 'accordion-like appearance' and a 'target-like appearance', respectively. Resolution of gastrointestinal tract wall thickening was observed on follow-up US performed a week after the increase in predinosolone (PSL). We conclude that careful evaluation of sonographic and radiographic findings helps to establish the diagnosis of lupus enteritis.
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Affiliation(s)
- Yuko Kaneko
- Department of Internal Medicine, Keio University, School of Medicine, Shinjuku-ku, Tokyo, Japan.
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Lee CK, Ahn MS, Lee EY, Shin JH, Cho YS, Ha HK, Yoo B, Moon HB. Acute abdominal pain in systemic lupus erythematosus: focus on lupus enteritis (gastrointestinal vasculitis). Ann Rheum Dis 2002; 61:547-50. [PMID: 12006332 PMCID: PMC1754133 DOI: 10.1136/ard.61.6.547] [Citation(s) in RCA: 129] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
OBJECTIVE To determine the causes of acute abdominal pain in systemic lupus erythematosus (SLE) and to compare the clinical and laboratory data, especially antiphospholipid antibodies and the SLE Disease Activity Index (SLEDAI), between lupus enteritis (gastrointestinal vasculitis) and acute abdominal pain without lupus enteritis in patients with SLE. METHODS A retrospective study was carried out for all patients admitted with SLE from 1993 to March 2001. The SLEDAI and laboratory data were collected at the time of diagnosis of SLE and at the time of acute abdominal pain. Lupus enteritis (gastrointestinal vasculitis) was diagnosed by clinical investigation and abdominal computed tomographic findings. RESULTS Chart review identified 175 patients (20 male, 155 female) who had been admitted with SLE. Of these patients, 38 (22%) presented with acute abdominal pain. Lupus enteritis was the most common cause of acute abdominal pain. Patients were divided into three groups: group 1: lupus enteritis (n=17), group 2: acute abdominal pain without lupus enteritis (n=21), and group 3: SLE without acute abdominal pain (n=137). There was no difference in age and sex among the three groups. Antiphospholipid, anti-RNP, anti-Sm, anti-Ro, and anti-La antibodies did not differ among the three groups. There was no difference in the SLEDAI at the time of diagnosis and at the time of acute abdominal pain between groups 1 and 2. Complement, erythrocyte sedimentation rate, C reactive protein, and anti-dsDNA measured at the time of acute abdominal pain did not differ between groups 1 and 2. A drop in the white blood cell count at the time of abdominal pain was more prominent in group 1 than group 2. In lupus enteritis, the jejunum and ileum were the sites most commonly affected. Rectal involvement was rare. Even though four patients relapsed, all the patients with lupus enteritis, including those who relapsed, responded well to corticosteroid. CONCLUSION Lupus enteritis is the most common cause of acute abdominal pain in SLE. All patients with lupus enteritis responded well to a high dose of a corticosteroid without surgical intervention. The SLEDAI and laboratory data, except leucopenia, do not correlate with the occurrence of lupus enteritis.
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Affiliation(s)
- C-K Lee
- Division of Allergy and Rheumatology, Department of Internal Medicine, Asan Medical Centre, Seoul, Korea
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