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Nikolic S, Lanzillotta M, Panic N, Brismar TB, Moro CF, Capurso G, Della Torre E, Löhr J, Vujasinovic M. Unraveling the relationship between autoimmune pancreatitis type 2 and inflammatory bowel disease: Results from two centers and systematic review of the literature. United European Gastroenterol J 2022; 10:496-506. [PMID: 35526270 PMCID: PMC9427095 DOI: 10.1002/ueg2.12237] [Citation(s) in RCA: 18] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/04/2022] [Accepted: 04/14/2022] [Indexed: 11/07/2022] Open
Abstract
INTRODUCTION The relationship between autoimmune pancreatitis (AIP) type 2 and inflammatory bowel disease (IBD) has been established and previously described within International Consensus Diagnostic Criteria. However, it is unknown if the presence of IBD changes the natural disease course of AIP type 2. Our aim was to investigate the association between AIP type 2 and IBD as well as to systematically summarize all the existing evidence in the literature. METHODS Electronic medical record analysis was conducted in two centers (in Stockholm, Sweden, and Milan, Italy; records dated between January 2001 and June 2021). Additionally, we conducted a systematic review of the literature. RESULTS A total of 35 patients (18 females, 51.4%) fulfilled the diagnostic criteria of AIP type 2 and were included in the study. A diagnosis of IBD was established in 29 patients (82.8%), ulcerative colitis in 17 (58.6%) and Crohn's disease in 11 (37.9%). Median follow-up was 54 months. AIP patients with IBD commonly presented with abdominal pain and/or acute pancreatitis at diagnosis, the latter was prevailing in concomitant and later IBD onset. These patients more frequently used steroids, but there were no differences in relapse rates. Concomitant onset of IBD was associated with the development of diabetes mellitus. There were no cases of colon or pancreatic malignancy during follow-up. In our systematic analysis, a total of 693 AIP type 2 patients were included from 24 single-center retrospective studies and 8 multicenter retrospective studies. A diagnosis of IBD was reported in 330 (47.8%) patients. Relapse rate was 20.0%. CONCLUSIONS Clinical and radiological remission of AIP type 2 was high, while the cumulative incidence of relapse is around 20%. Our results show that concomitance of IBD imposes no obvious risk of a different disease course for AIP type 2.
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Affiliation(s)
- Sara Nikolic
- Department of Medicine HuddingeKarolinska InstitutetStockholmSweden
- Department of GastroenterologyClinic for Internal MedicineUniversity Medical Centre MariborMariborSlovenia
| | - Marco Lanzillotta
- Università Vita‐Salute San RaffaeleIRCCS San Raffaele Scientific InstituteMilanItaly
- Unit of ImmunologyRheumatology, Allergy and Rare Diseases (Unirar)IRCCS San Raffaele Scientific Institute, ss MilanMilanItaly
| | - Nikola Panic
- Faculty of MedicineUniversity of BelgradeDigestive Endoscopy UnitUniversity Clinic “Dr Dragisa Misovic”BelgradeSerbia
| | - Torkel B. Brismar
- Department of RadiologyKarolinska University HospitalStockholmSweden
| | - Carlos Fernández Moro
- Department of Clinical Pathology and Cancer DiagnosticsKarolinska University HospitalStockholmSweden
| | - Gabriele Capurso
- Division of Pancreatic Surgery and Endosonography DivisionPancreas Translational and Clinical Research CenterIRCCS San Raffaele Scientific InstituteMilanItaly
| | - Emanuel Della Torre
- Università Vita‐Salute San RaffaeleIRCCS San Raffaele Scientific InstituteMilanItaly
- Unit of ImmunologyRheumatology, Allergy and Rare Diseases (Unirar)IRCCS San Raffaele Scientific Institute, ss MilanMilanItaly
| | - J.‐Matthias Löhr
- Department of Upper Digestive DiseasesKarolinska University HospitalStockholmSweden
- Department of Clinical ScienceIntervention, and Technology (CLINTEC)Karolinska InstitutetStockholmSweden
| | - Miroslav Vujasinovic
- Department of Medicine HuddingeKarolinska InstitutetStockholmSweden
- Department of Upper Digestive DiseasesKarolinska University HospitalStockholmSweden
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Update on classification, diagnosis, and management of immunoglobulin G4-related disease. Chin Med J (Engl) 2022; 135:381-392. [PMID: 34985023 PMCID: PMC8869566 DOI: 10.1097/cm9.0000000000001891] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized chronic fibro-inflammatory autoimmune disease, and its recognition has been constantly increasing worldwide over the last few years. A correct and timely recognition, as well as appropriate intervention, is crucial for the treatment of IgG4-RD. For certain subtypes of IgG4-RD, organ-specific criteria are formulated to make the diagnosis more accurate. New biomarkers have emerged in the recent years to aid the disease diagnosis, its prognosis prediction, as well as therapy response monitoring. Although recurrence is very common in IgG4-RD, glucocorticoid is still the first-line treatment for the majority of patients. The factors that affect the likelihood of disease relapse are multifaceted. The selection strategy of various steroid-sparing agents is still being explored. Besides, when patients have special sites involvement leading to severe clinical conditions, surgical operation or interventional therapy should also be considered. An update on classification, diagnosis, and management of IgG4-RD is provided in the current study to fully elucidate the recommended clinical practice of this mysterious disease.
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Masood M. Autoimmune pancreatitis: What we know so far. JGH Open 2021; 6:3-10. [PMID: 35071782 PMCID: PMC8762623 DOI: 10.1002/jgh3.12688] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2021] [Revised: 11/12/2021] [Accepted: 11/20/2021] [Indexed: 12/19/2022]
Abstract
Autoimmune pancreatitis (AIP) is a rare, often‐missed disease that involves inflammation of the pancreas and strictures of the pancreatic duct. Its prevalence and incidence in the United States remain scarce. The disease has a varied presentation and often mimics pancreatic malignancy, which can make the diagnosis challenging. Most patients have an excellent response to corticosteroid therapy. Immunomodulators may be used in some cases. Rituximab is an effective, emerging treatment in steroid‐refractory cases. This study aims to review the two distinct types of AIP and provide a detailed analysis of the diagnostic approach and treatment modalities.
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Affiliation(s)
- Muaaz Masood
- Department of Internal Medicine Medical College of Georgia at Augusta University Augusta Georgia USA
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Huang XM, Shi ZS, Ma CL. Multifocal autoimmune pancreatitis: A retrospective study in a single tertiary center of 26 patients with a 20-year literature review. World J Gastroenterol 2021; 27:4429-4440. [PMID: 34366614 PMCID: PMC8316903 DOI: 10.3748/wjg.v27.i27.4429] [Citation(s) in RCA: 1] [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: 01/28/2021] [Revised: 02/28/2021] [Accepted: 04/25/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Multifocal-type autoimmune pancreatitis (AIP), sometimes forming multiple pancreatic masses, is frequently misdiagnosed as pancreatic malignancy in routine clinical practice. It is critical to know the imaging features of multifocal-type AIP to prevent misdiagnosis and unnecessary surgery. To the best of our knowledge, there have been no studies evaluating the value of diffusionweighted imaging (DWI), axial fat-suppressed T1 weighted image (T1WI), and dynamic contrast enhanced-computed tomography (DCE-CT) in detecting the lesions of multifocal-type AIP.
AIM To clarify the exact prevalence and radiological findings of multifocal AIP in our cohorts and compare the sensitivity of DWI, axial fat-suppressed T1WI, and DCE-CT for detecting AIP lesions. We also compared radiological features between multifocal AIP and pancreatic ductal adenocarcinoma with several key imaging landmarks.
METHODS Twenty-six patients with proven multifocal AIP were retrospectively included. Two blinded independent radiologists rated their confidence level in detecting the lesions on a 5-point scale and assessed the diagnostic performance of DWI, axial fat-suppressed T1WI, and DCE-CT. CT and magnetic resonance imaging of multifocal AIP were systematically reviewed for typical imaging findings and compared with the key imaging features of pancreatic ductal adenocarcinoma.
RESULTS Among 118 patients with AIP, 26 (22.0%) had multiple lesions (56 lesions). Ulcerative colitis was associated with multifocal AIP in 7.7% (2/26) of patients, and Crohn’s disease was present in 15.3% (4/26) of patients. In multifocal AIP, multiple lesions, delayed homogeneous enhancement, multifocal strictures of the main pancreatic duct, capsule-like rim, lower apparent diffusion coefficient values, and elevated serum Ig4 level were observed significantly more frequently than pancreatic ductal adenocarcinoma, whereas the presence of capsule-like rim in multifocal-type AIP was lower in frequency than total AIP. Of these lesions of multifocal AIP, DWI detected 89.3% (50/56) and 82.1% (46/56) by the senior and junior radiologist, respectively.
CONCLUSION Multifocal AIP is not as rare as previously thought and was seen in 22.0% of our patients. The diagnostic performance of DWI for detecting multifocal AIP was best followed by axial fat-suppressed T1WI and DCE-CT.
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Affiliation(s)
- Xin-Ming Huang
- Department of Radiology, The First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, Fujian Province, China
- Department of Radiology, Fujian Medical University Union Hospital, Fuzhou 350005, Fujian Province, China
| | - Zhen-Shan Shi
- Department of Radiology, The First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, Fujian Province, China
| | - Cheng-Le Ma
- Department of Radiology, The First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, Fujian Province, China
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Demir AM, Aydin F, Acar B, Kurt T, Poyraz A, Kiremitci S, Gülleroglu B, Azili MN, Bayrakci US. IgG4-related disease and ANCA positive vasculitis in childhood: a case-based review. Clin Rheumatol 2021; 40:3817-3825. [PMID: 33590421 DOI: 10.1007/s10067-021-05635-5] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2020] [Revised: 01/31/2021] [Accepted: 02/03/2021] [Indexed: 12/24/2022]
Abstract
Autoimmune pancreatitis (AIP) type 1 is an IgG4-related disease (IgG4-RD), characterized by inflammatory pseudotumors and histologically by dense lymphoplasmacytic infiltrates rich in IgG4 positive plasma cells, storiform fibrosis, and obliterative phlebitis. Although quite rare, IgG4-RD was found to be associated with medium or small vessel vasculitides. A new overlap syndrome between IgG4-RD and ANCA-associated vasculitis (AAV) has recently been described in the adult population. Here we present a 16-year-old adolescent girl admitted with abdominal pain, episcleritis, palpable purpura, salivary gland enlargement, and bloody diarrhea. Laboratory investigations revealed findings of glomerulonephritis. Abdominal imaging surprisingly revealed a focal mass in the pancreatic tail, while the c-ANCA level was found to be quite high as well as serum IgG4 level. Biopsy of the pancreatic mass showed lymphoplasmacytic IgG4 positive cells infiltrating the pancreas with storiform fibrosis compatible with IgG4-related AIP. The renal biopsy that was done simultaneously showed necrotizing granulomatous vasculitis indicating AAV. Renal biopsy showed IgG4 positive plasma cells very rarely by immunohistochemical examination, which does not indicate any significance for IgG4-RD. Our diagnosis was IgG4-related AIP and AAV overlap syndrome, which has not been reported in the pediatric populations yet. IgG4-RD should be investigated in patients with ANCA-associated vasculitis who shows atypical organ involvement. We searched the Pubmed/Medline and Google Scholar databases to identify clinical findings, treatment, and outcome of the patients with IgG4-related AIP and AAV.
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Affiliation(s)
- Arzu Meltem Demir
- Department of Pediatric Gastroenterology, Ankara City Hospital, Ankara, Turkey.
| | - Fatma Aydin
- Department of Pediatric Rheumatology, Ankara City Hospital, Ankara, Turkey
| | - Banu Acar
- Department of Pediatric Rheumatology, Ankara City Hospital, Ankara, Turkey
| | - Tuba Kurt
- Department of Pediatric Rheumatology, Ankara City Hospital, Ankara, Turkey
| | - Aylar Poyraz
- Department of Pathology, Gazi University Medical Faculty, Ankara, Turkey
| | - Saba Kiremitci
- Department of Pathology, Ankara University Medical Faculty, Ankara, Turkey
| | - Basak Gülleroglu
- Department of Pediatric Radiology, Ankara City Hospital, Ankara, Turkey
| | - Müjdem Nur Azili
- Department of Pediatric Surgery, Ankara City Hospital, Ankara, Turkey
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Facciorusso A, Barresi L, Cannizzaro R, Antonini F, Triantafyllou K, Tziatzios G, Muscatiello N, Hart PA, Wani S. Diagnostic yield of endoscopic ultrasound-guided tissue acquisition in autoimmune pancreatitis: a systematic review and meta-analysis. Endosc Int Open 2021; 9:E66-E75. [PMID: 33403238 PMCID: PMC7775812 DOI: 10.1055/a-1293-7279] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/13/2020] [Accepted: 09/28/2020] [Indexed: 02/08/2023] Open
Abstract
Background and study aims There is limited evidence on the diagnostic performance of endoscopic ultrasound (EUS)-guided tissue acquisition in autoimmune pancreatitis (AIP). The aim of this meta-analysis was to provide a pooled estimate of the diagnostic performance of EUS-guided fine-needle aspiration (FNA) and fine-needle biopsy (FNB) in patients with AIP. Patients and methods Computerized bibliographic search was performed through January 2020. Pooled effects were calculated using a random-effects model by means of DerSimonian and Laird test. Primary endpoint was diagnostic accuracy compared to clinical diagnostic criteria. Additional outcomes were definitive histopathology, pooled rates of adequate material for histological diagnosis, sample adequacy, mean number of needle passes. Diagnostic sensitivity and safety data were also analyzed. Results Fifteen studies with 631 patients were included, of which four were prospective series and one randomized trial. Overall diagnostic accuracy of EUS tissue acquisition was 54.7 % (95 % confidence interval, 40.9 %-68.4 %), with a clear superiority of FNB over FNA (63 %, 52.7 % to 73.4 % versus 45.7 %, 26.5 %-65 %; p < 0.001). FNB provided level 1 of histological diagnosis in 44.2 % of cases (30.8 %-57.5 %) as compared to 21.9 % (10 %-33.7 %) with FNA ( P < 0.001). The rate of definitive histopathology of EUS tissue sampling was 20.7 % (12.9 %-28.5 %) and it was significantly higher with FNB (24.3 %, 11.8 %-36.8 %) as compared to FNA (14.7 %, 5.4 %-23.9 %; P < 0.001). Less than 1 % of subjects experienced post-procedural acute pancreatitis. Conclusion The results of this meta-analysis demonstrate that the diagnostic performance of EUS-guided tissue acquisition is modest in patients with AIP, with an improved performance of FNB compared to FNA.
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Affiliation(s)
| | - Luca Barresi
- Endoscopy Service, Department of Diagnostic and Therapeutic Services, Mediterranean Institute for Transplantation and Advanced Specialized Therapies (IRCCS – ISMETT), Palermo, Italy
| | - Renato Cannizzaro
- Oncological Gastroenterology, Centro di Riferimento Oncologico di Aviano (CRO) IRCCS, Aviano, Italy
| | - Filippo Antonini
- Gastroenterology and Endoscopy Unit, Marche Polytechnic University, A. Murri Hospital, Fermo, Italy
| | - Konstantinos Triantafyllou
- Hepatogastroenterology Unit, Second Department of Internal Medicine – Propaedeutic, Research Institute and Diabetes Center, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Georgios Tziatzios
- Hepatogastroenterology Unit, Second Department of Internal Medicine – Propaedeutic, Research Institute and Diabetes Center, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | | | - Phil A. Hart
- Division of Gastroenterology, Hepatology, and Nutrition, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
| | - Sachin Wani
- University of Colorado Anschutz Medical Campus, Aurora, Colorado, United States
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Lanzillotta M, Vinge-Holmquist O, Overbeek KA, Poulsen JL, Demirci AF, Macinga P, Löhr M, Rosendahl J. PrescrAIP: A Pan-European Study on Current Treatment Regimens of Auto-Immune Pancreatitis. Front Med (Lausanne) 2020; 7:408. [PMID: 32850908 PMCID: PMC7419461 DOI: 10.3389/fmed.2020.00408] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/22/2020] [Accepted: 06/29/2020] [Indexed: 12/22/2022] Open
Abstract
Introduction: Treatment of autoimmune pancreatitis (AIP) is based solely on consensus and has yet to become standardized. Consequently, therapeutic regimens vary greatly between countries and centers, and largely depend on the experience of the physician. At this moment, the optimal regimen for inducing disease remission and preventing relapse is unknown. Objectives: The primary objective of this study is to describe current treatment regimens used in Europe, and to compare their effectiveness in inducing remission and preventing and treating relapse. The secondary objectives are: to identify risk factors for relapse; to assess the diagnostic accuracy of the Unified-AIP criteria; to assess the performance of the M-ANNHEIM score for predicting relapse; and to assess long-term outcomes including pancreatic exocrine insufficiency and pancreatic cancer. Methods: This is an international, retrospective, observational cohort study, performed in over 40 centers from 16 European countries. Eligible are all patients diagnosed with AIP from 2005 onwards, regardless of the used diagnostic criteria. Data on study subjects will be retrieved from the hospital's electronic medical records and registered with a standardized, web-based, electronic case report form (eCRF). To compare the effectiveness of treatment regimens in inducing remission, preventing relapse, and treating relapse, subjects will be stratified in groups based on: type of therapy; initial therapy dose; cumulative therapy dose; therapy tapering speed and duration; and having received maintenance therapy or not. Ethics and Dissemination: Ethical and/or institutional review board approvals are obtained by all participating centers according to local regulations. The study complies with the General Data Protection Regulation (GDPR). All manuscripts resulting from the study will be submitted to peer-reviewed journals. Conclusion: This is the first pan-European retrospective registry for AIP. It will produce the first large-scale data on treatment of European patients with AIP, providing answers on the use and effectiveness of treatment regimens. In the future, this collaboration may provide a network for continuation into a prospective European registry.
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Affiliation(s)
- Marco Lanzillotta
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), San Raffaele Scientific Institute, Milan, Italy
| | - Olof Vinge-Holmquist
- Department of Gastroenterological Surgery, Akershus University Hospital, Loerenskog, Norway
| | - Kasper A Overbeek
- Department of Gastroenterology & Hepatology, Erasmus University Medical Center, Rotterdam, Netherlands
| | - Jakob L Poulsen
- Centre for Pancreatic Diseases, Department of Gastroenterology & Hepatology, Aalborg University Hospital, Aalborg, Denmark
| | - A Fatih Demirci
- Department of Internal Medicine, Marmara University Research and Education Hospital, Istanbul, Turkey
| | - Peter Macinga
- Department of Gastroenterology and Hepatology, Institute for Clinical and Experimental Medicine, Prague, Czechia
| | - Matthias Löhr
- Gastroenterology and Hepatology, Gastrocentrum, Karolinska University Hospital, Karolinska Universitetssjukhuset, Stockholm, Sweden
| | - Jonas Rosendahl
- Department of Internal Medicine I, Martin Luther University, Halle (Saale), Germany
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Lee TH. Differentiation of Type 2 Autoimmune Pancreatitis Presenting as Clinical Acute Pancreatitis. Gut Liver 2019; 13:385-387. [PMID: 31284361 PMCID: PMC6622572 DOI: 10.5009/gnl19175] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Affiliation(s)
- Tae Hoon Lee
- Department of Internal Medicine, Soonchunhyang University Cheonan Hospital, Soonchunhyang University School of Medicine, Cheonan, Korea
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Rana SS, Gupta R, Nada R, Gupta P, Basher R, Mittal BR, Sharma RK, Rawat A. Clinical profile and treatment outcomes in autoimmune pancreatitis: a report from North India. Ann Gastroenterol 2018; 31:506-512. [PMID: 29991897 PMCID: PMC6033754 DOI: 10.20524/aog.2018.0267] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/01/2018] [Accepted: 03/24/2018] [Indexed: 12/11/2022] Open
Abstract
BACKGROUND Autoimmune pancreatitis (AIP) is a rare disease, and data from countries like India concerning its clinical presentation and long-term outcomes are scarce. We retrospectively evaluated the clinical presentation, imaging features and treatment outcomes of patients with AIP. METHODS We carried out a retrospective analysis of our database to identify patients diagnosed with and treated for AIP at our unit in a tertiary care hospital in North India. RESULTS Eighteen patients with AIP (mean age: 54.9±11.1 years; 13 male) were evaluated. Of these, 9 (50%) patients had probable type 1 AIP, 2 (11%) patients probable type 2 AIP, and 4 (22%) definite type 1 AIP. Patients with type 2 AIP were significantly younger than patients with type 1 (40.0±2.8 vs. 58.4±9.6 years). In type 1 AIP, other organ involvement was observed in 3/18 (17%) patients, whereas both patients with type 2 AIP had coexisting ulcerative colitis. The diagnosis of AIP was made after resective surgery in 6/18 (33.0%) patients. An accurate diagnosis of AIP could be made in all patients who underwent resection or core biopsy, but cytological examination after endoscopic ultrasound-guided fine-needle aspiration could not provide a definitive diagnosis in any patient. Initial treatment with steroids was given to 12 (67%) patients, with a 100% response, but the disease relapsed in 5/13 (38%) patients over a mean follow-up period of 34.2±21.6 weeks. CONCLUSION AIP is not rare in India and the majority of clinical manifestations, imaging features, treatment response and long-term outcomes are similar to those reported in the literature.
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Affiliation(s)
- Surinder S Rana
- Department of Gastroenterology (Surinder S. Rana, Pankaj Gupta, Ravi kumar Sharma), Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh India
| | - Rajesh Gupta
- Department of Surgery (Rajesh Gupta), Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh India
| | - Ritambhra Nada
- Department of Histopathology (Ritambhra Nada), Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh India
| | - Pankaj Gupta
- Department of Gastroenterology (Surinder S. Rana, Pankaj Gupta, Ravi kumar Sharma), Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh India
| | - Rajinder Basher
- Department of Nuclear Medicine (Rajinder Basher, Bhagwat R. Mittal), Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh India
| | - Bhagwat R Mittal
- Department of Nuclear Medicine (Rajinder Basher, Bhagwat R. Mittal), Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh India
| | - Ravi Kumar Sharma
- Department of Gastroenterology (Surinder S. Rana, Pankaj Gupta, Ravi kumar Sharma), Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh India
| | - Amit Rawat
- Department of Pediatrics (Amit Rawat), Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh India
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Detlefsen S, Klöppel G. IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis. Virchows Arch 2017; 472:545-556. [PMID: 29196804 DOI: 10.1007/s00428-017-2275-z] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/25/2017] [Revised: 11/16/2017] [Accepted: 11/19/2017] [Indexed: 12/19/2022]
Abstract
In 2011, chronic fibroinflammatory processes occurring simultaneously or metachronously in various organs and associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4-positive plasma cells have been recognized as manifestations of a systemic disorder called IgG4-related disease (IgG4-RD). The histologic key findings are lymphoplasmacytic infiltration rich in IgG4-positive plasma cells combined with storiform fibrosis and obliterative phlebitis. Among the organs mainly affected by IgG4-RD are the pancreas and the extrahepatic bile ducts. The pancreatic and biliary alterations have been described under the terms autoimmune pancreatitis (AIP) and sclerosing cholangitis, respectively. These diseases are currently more precisely called IgG4-related pancreatitis (or type 1 AIP to distinguish it from type 2 AIP that is unrelated to IgG4-RD) and IgG4-related sclerosing cholangitis (IgG4-related SC). Clinically and grossly, both diseases commonly imitate pancreatic and biliary adenocarcinoma, tumors that are well known for their dismal prognosis. As IgG4-RD responds to steroid treatment, making a resection of a suspected tumor unnecessary, a biopsy is often required to establish the preoperative diagnosis. This review discusses the morphologic spectrum of IgG4-related pancreatitis and IgG4-related SC and focuses on the biopsy relevant histologic features for the diagnosis and differential diagnosis of these diseases.
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Affiliation(s)
- Sönke Detlefsen
- Department of Pathology, Odense University Hospital, J.B. Winsløws Vej 15, 5000, Odense C, Denmark.
| | - Günter Klöppel
- Department of Pathology, Consultation Center of Pancreatic and Endocrine Tumors, Technical University Munich, Ismaninger Str. 22, 81675, Munich, Germany
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