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1
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Yarahmadi A, Afkhami H. Potential relationship between Helicobacter pylori infection and autoimmune disorders: A narrative review. Microb Pathog 2025; 205:107572. [PMID: 40220801 DOI: 10.1016/j.micpath.2025.107572] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/11/2024] [Revised: 04/06/2025] [Accepted: 04/09/2025] [Indexed: 04/14/2025]
Abstract
Helicobacter pylori (H. pylori) is a spiral-shaped, gram-negative, flagellated bacteria that causes gastritis symptoms. This bacterium, particularly in individuals with a genetic predisposition, has been implicated in the pathogenesis of several autoimmune diseases (AD) through complex mechanisms involving the interaction of cellular and humoral immune responses. This review article tells you about the link between H. pylori infection and several types of AD, including systemic lupus erythematosus (SLE), autoimmune pancreatitis (AIP), rheumatoid arthritis (RA), Sjögren syndrome (SjS), psoriasis, and antiphospholipid syndrome (APS). We conducted a comprehensive analysis of the current literature to elucidate the potential role of H. pylori as a triggering factor for these disorders. Our findings suggest a significant correlation between H. pylori infection and the onset or exacerbation of specific ADs. This relationship is common mechanisms, including molecular mimicry, chronic inflammation, epitope spreading, and cytokine dysregulation. While H. pylori is implicated in AD, other factors such as genetic predisposition, environmental triggers, and other microbial agents also play crucial roles. Other pathogens, such as Epstein-Barr virus (EBV), cytomegalovirus (CMV), and bacteria like Mycobacterium tuberculosis and Chlamydia pneumoniae have been linked to ADs. These shared pathways highlight the potential role of H. pylori as a unifying factor in the pathogenesis of diverse ADs. Further research is necessary to fully understand the interactions between H. pylori and the immune system in the context of autoimmunity. This review aims to provide a detailed overview of the knowledge on this topic, highlighting the need for additional studies to clarify these complex relationships.
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Affiliation(s)
- Aref Yarahmadi
- Department of Biology, Khorramabad Branch, Islamic Azad University, Khorramabad, Iran
| | - Hamed Afkhami
- Cellular and Molecular Research Center, Qom University of Medical Sciences, Qom, Iran; Nervous System Stem Cells Research Center, Semnan University of Medical Sciences, Semnan, Iran; Department of Medical Microbiology, Faculty of Medicine, Shahed University, Tehran, Iran.
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2
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Mol B, Werner E, Culver EL, van der Meer AJ, Bogaards JA, Ponsioen CY. Epidemiological and economical burden of cholestatic liver disease. Hepatology 2025:01515467-990000000-01224. [PMID: 40168457 DOI: 10.1097/hep.0000000000001341] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/02/2025] [Accepted: 03/24/2025] [Indexed: 04/03/2025]
Abstract
The main cholestatic liver diseases comprise primary sclerosing cholangitis, primary biliary cholangitis, and IgG4-related cholangitis. Despite being classified as rare diseases, these are becoming gradually more important in the field of hepatology since their incidence is slowly rising while the viral hepatitis burden is declining. Cholestatic liver diseases now rank among the 3 most frequent indications for liver transplantation in many Western countries. An accurate understanding of the epidemiology and burden of disease on both the individual and society of cholestatic diseases is of great importance. This review aims to provide a comprehensive overview of the current literature on the epidemiology, health-related quality of life, and economic burden of primary sclerosing cholangitis, primary biliary cholangitis, and IgG4-related cholangitis.
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Affiliation(s)
- Bregje Mol
- Department of Gastroenterology and Hepatology, Amsterdam University Medical Centre, Amsterdam, The Netherlands
- Endocrinology and Metabolism, Amsterdam Institute of Gastroenterology, Amsterdam, The Netherlands
| | - Ellen Werner
- Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center Rotterdam, The Netherlands
| | - Emma L Culver
- Oxford Liver Unit, John Radcliffe Hospital, Oxford, UK
| | - Adriaan J van der Meer
- Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center Rotterdam, The Netherlands
| | - Johannes A Bogaards
- Department of Epidemiology and Data Science, Amsterdam University Medical Centre, Amsterdam, The Netherlands
| | - Cyriel Y Ponsioen
- Department of Gastroenterology and Hepatology, Amsterdam University Medical Centre, Amsterdam, The Netherlands
- Endocrinology and Metabolism, Amsterdam Institute of Gastroenterology, Amsterdam, The Netherlands
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3
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Woo J, Sano K, Oshio K, Suzuki A, Isayama H, Saiura A, Fukumura Y, Kamagata K, Kuwatsuru R, Aoki S. Differentiation of Autoimmune Pancreatitis From Pancreatic Ductal Adenocarcinoma by Diffusion-Weighted Magnetic Resonance Imaging With Weighted Diffusion Subtraction. Cureus 2025; 17:e81438. [PMID: 40303521 PMCID: PMC12037419 DOI: 10.7759/cureus.81438] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/29/2025] [Indexed: 05/02/2025] Open
Abstract
Purpose Weighted diffusion subtraction (WDS) is a new imaging technique derived from a pair of diffusion-weighted images (DWIs) using low and high b values. This study assesses the diagnostic performance of WDS in distinguishing pancreatic ductal adenocarcinoma (PDAC) from autoimmune pancreatitis (AIP) by comparing the efficacy of DWI alone compared to DWI with WDS. Methods The patient cohort consisted of 10 patients diagnosed with AIP and 28 patients diagnosed with PDAC. Two blinded radiologists reviewed the MRI images and assessed their diagnostic accuracy and confidence levels. Differences between groups A (DWI alone) and B (DWI added WDS) were assessed, and a receiver operating characteristic (ROC) curve analysis was performed. Inter-reader agreement was analyzed using weighted κ statistics, and apparent diffusion coefficient (ADC) values were calculated for both AIP and PDAC. Results In diagnostic accuracy, Group B demonstrated significantly higher area under the curve (AUC) values than Group A for both readers. The inter-reader agreement was evaluated as "substantial" in both groups. The mean confidence scores for Group B were significantly higher than those for Group A for both readers. Conclusion The addition of WDS to DWI may enhance the visual differentiation between AIP and PDAC, potentially improving diagnostic accuracy and confidence.
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Affiliation(s)
- Jun Woo
- Department of Radiology, Jikei University School of Medicine, Tokyo, JPN
| | - Katsuhiro Sano
- Department of Radiology, Juntendo University Graduate School of Medicine, Tokyo, JPN
| | - Koichi Oshio
- Department of Radiology, Juntendo University Graduate School of Medicine, Tokyo, JPN
| | - Akiyoshi Suzuki
- Department of Radiology, Juntendo University Graduate School of Medicine, Tokyo, JPN
| | - Hiroyuki Isayama
- Department of Gastroenterology, Juntendo University Graduate School of Medicine, Tokyo, JPN
| | - Akio Saiura
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University Graduate School of Medicine, Tokyo, JPN
| | - Yuki Fukumura
- Department of Human Pathology, Juntendo University Graduate School of Medicine, Tokyo, JPN
| | - Koji Kamagata
- Department of Radiology, Juntendo University Graduate School of Medicine, Tokyo, JPN
| | - Ryohei Kuwatsuru
- Department of Radiology, Juntendo University Graduate School of Medicine, Tokyo, JPN
| | - Shigeki Aoki
- Department of Radiology, Juntendo University Graduate School of Medicine, Tokyo, JPN
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4
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Ghaffari Jolfayi A, Salmanipour A, Heshmat-Ghahdarijani K, Meshgi S, Dastmardi M, Salehabadi G, Azimi A, Bazargany MHM, Roudsari PP, Mahmoodiyeh B, Mohammadzadeh A. Imaging findings in cardiovascular involvements of IgG4-related disease: a systematic review study. Rheumatology (Oxford) 2025; 64:943-951. [PMID: 39271148 DOI: 10.1093/rheumatology/keae494] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/17/2024] [Revised: 07/24/2024] [Accepted: 08/30/2024] [Indexed: 09/15/2024] Open
Abstract
BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by IgG4-positive plasma cell infiltration that can affect multiple organs, including the cardiovascular system. The diagnosis of IgG4-RD relies on a combination of clinical, serological, radiological and pathological findings. However, due to the varied and insidious clinical presentations, normal IgG4 levels in a significant percentage of patients and frequent multi-organ involvement, imaging plays a crucial role in the diagnosis of IgG4-RD. The aim of this study is to comprehensively examine the imaging findings in IgG4-related cardiovascular disease for accurate diagnosis and appropriate treatment. METHODS A systematic search was conducted across the electronic databases PubMed, Scopus, Embase and Web of Science, to 1 September 2023, following PRISMA guidelines, searching for studies reporting detailed cardiovascular imaging findings in IgG4-RD. RESULTS The search yielded 68 studies (60 case reports, 5 case series, 2 cross-sectional, 1 case-control) with 120 cases of cardiovascular IgG4-RD. Most of the cases were male, averaging 62.8 years. The common initial symptoms were dyspnoea and chest pain. The most common imaging finding was vasculopathy, including vessel wall thickening, periarteritits, periaortitis, aortitis, stenosis, ectasia, aneurysm formation, intramural haemorrhage, fistula formation and dissection, followed by pericardial involvement and mediastinal masses. Case series and cross-sectional studies also showed vasculopathy to be the most common finding on various imaging modalities, including angiography and PET/CT, highlighting the complex pathology of IgG4-RD. CONCLUSION This study evaluated current IgG4-RD articles, revealing a higher prevalence in men and vasculopathy as the most common cardiovascular complication.
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Affiliation(s)
- Amir Ghaffari Jolfayi
- Cardiovascular Research Center, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran
| | - Alireza Salmanipour
- Cardiovascular Research Center, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran
| | - Kiyan Heshmat-Ghahdarijani
- Heart Failure Research Center, Cardiovascular Research Institute, Isfahan University of Medical Sciences, Isfahan, Iran
| | - Shahla Meshgi
- Iran Department of Radiology, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran
| | - Maedeh Dastmardi
- Cardiovascular Research Center, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran
| | - Ghazaleh Salehabadi
- Cardiovascular Research Center, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran
| | - Amir Azimi
- Cardiovascular Research Center, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran
| | | | | | - Behnaz Mahmoodiyeh
- Iran Department of Radiology, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran
| | - Ali Mohammadzadeh
- Heart Failure Research Center, Cardiovascular Research Institute, Isfahan University of Medical Sciences, Isfahan, Iran
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Metelli F, Manfredi G, Pagano N, Buscarini E, Crinò SF, Armellini E. The Role of Endoscopic Ultrasound and Ancillary Techniques in the Diagnosis of Autoimmune Pancreatitis: A Comprehensive Review. Diagnostics (Basel) 2024; 14:1233. [PMID: 38928649 PMCID: PMC11202526 DOI: 10.3390/diagnostics14121233] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2024] [Revised: 06/04/2024] [Accepted: 06/07/2024] [Indexed: 06/28/2024] Open
Abstract
Autoimmune pancreatitis (AIP) is a unique form of chronic pancreatitis with a multifactorial pathogenesis. Historically, it has been classified as type 1 and type 2, according to its clinical and histological features. The diagnosis of AIP is challenging and relies on a combination of clinical, histopathologic, serologic, and imaging characteristics. In the available guidelines, the imaging hallmarks of AIP are based on cross-sectional imaging and cholangiopancreatography retrograde endoscopic findings. Endoscopic ultrasound (EUS) is generally used for pancreatic tissue acquisition to rule out pancreatic cancer and diagnose AIP with limited accuracy. Several papers reported the reliability of EUS for providing informative morphologic features of AIP. Nowadays, the improvement in the resolution of EUS conventional images and the development of new ancillary technologies have further increased the diagnostic yield of EUS: contrast-enhanced EUS and EUS elastography are non-invasive and real-time techniques that strongly support the diagnosis and management of pancreatic diseases. In this review article, we will present the role of conventional EUS and ancillary diagnostic techniques in the diagnosis of AIP to support clinicians and endosonographers in managing this condition.
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Affiliation(s)
- Flavio Metelli
- Gastroenterology and Endoscopy Department, ASST Maggiore Hospital Crema, 26013 Crema, Italy; (F.M.); (G.M.); (E.B.)
| | - Guido Manfredi
- Gastroenterology and Endoscopy Department, ASST Maggiore Hospital Crema, 26013 Crema, Italy; (F.M.); (G.M.); (E.B.)
| | - Nico Pagano
- Gastroenterology Unit, Department of Oncological and Specialty Medicine, University Hospital Maggiore della Carità, 28100 Novara, Italy;
| | - Elisabetta Buscarini
- Gastroenterology and Endoscopy Department, ASST Maggiore Hospital Crema, 26013 Crema, Italy; (F.M.); (G.M.); (E.B.)
| | - Stefano Francesco Crinò
- Diagnostic and Interventional Endoscopy of Pancreas, Pancreas Institute, University of Verona, 37134 Verona, Italy;
| | - Elia Armellini
- Gastroenterology and Endoscopy Unit, ASST-Bergamoest, 24068 Seriate, Italy
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Pădureanu V, Drăgoescu AN, Pădureanu R, Rośu MM, Rădulescu D, Dop D, For£ofoiu MC. Treatment approaches in autoimmune pancreatitis (Review). Biomed Rep 2024; 20:26. [PMID: 38259589 PMCID: PMC10801350 DOI: 10.3892/br.2023.1714] [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] [Received: 09/02/2023] [Accepted: 12/11/2023] [Indexed: 01/24/2024] Open
Abstract
Autoimmune pancreatitis (AIP) is a rare disease. There are two distinct types of AIP: AIP type 1 (AIP-1), a pancreatic manifestation of a multi-organ disease linked to immunoglobulin (Ig)G4, and AIP type 2 (AIP-2), a pancreas-specific disease unrelated to IgG4. The usual course of treatment for AIP is oral corticosteroid medication. Rituximab has also been recommended for recurrent AIP-1 in order to initiate remission and provide ongoing treatment. Immunomodulators such as azathioprine are used to keep certain patients in remission. Evaluation also takes into account a number of pharmacological alternatives, including biologic drugs like anti-tumor necrosis factor therapy, a safe and efficient second-line treatment for AIP-2 relapse or steroid dependence. Corticosteroids and immunosuppressants, which are poorly tolerated due to considerable side effects, are being replaced by other biologic drugs, which may offer a beneficial therapeutic alternative.
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Affiliation(s)
- Vlad Pădureanu
- Department of Internal Medicine, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania
| | - Alice Nicoleta Drăgoescu
- Department of Anaesthesiology and Intensive Care, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania
| | - Rodica Pădureanu
- Department of Internal Medicine, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania
| | - Maria Magdalena Rośu
- Department of Diabetes, Nutrition and Metabolic Diseases, County Clinical Emergency Hospital of Craiova, Craiova 200642, Romania
| | - Dumitru Rădulescu
- Department of Pediatrics, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania
| | - Dalia Dop
- Department of Surgery, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania
| | - Mircea Cătălin For£ofoiu
- Department of Internal Medicine, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania
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Miller FH, Lopes Vendrami C, Hammond NA, Mittal PK, Nikolaidis P, Jawahar A. Pancreatic Cancer and Its Mimics. Radiographics 2023; 43:e230054. [PMID: 37824413 DOI: 10.1148/rg.230054] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/14/2023]
Abstract
Pancreatic ductal adenocarcinoma (PDAC) is the most common primary pancreatic malignancy, ranking fourth in cancer-related mortality in the United States. Typically, PDAC appears on images as a hypovascular mass with upstream pancreatic duct dilatation and abrupt duct cutoff, distal pancreatic atrophy, and vascular encasement, with metastatic involvement including lymphadenopathy. However, atypical manifestations that may limit detection of the underlying PDAC may also occur. Atypical PDAC features include findings related to associated conditions such as acute or chronic pancreatitis, a mass that is isointense to the parenchyma, multiplicity, diffuse tumor infiltration, associated calcifications, and cystic components. Several neoplastic and inflammatory conditions can mimic PDAC, such as paraduodenal "groove" pancreatitis, autoimmune pancreatitis, focal acute and chronic pancreatitis, neuroendocrine tumors, solid pseudopapillary neoplasms, metastases, and lymphoma. Differentiation of these conditions from PDAC can be challenging due to overlapping CT and MRI features; however, certain findings can help in differentiation. Diffusion-weighted MRI can be helpful but also can be nonspecific. Accurate diagnosis is pivotal for guiding therapeutic planning and potential outcomes in PDAC and avoiding biopsy or surgical treatment of some of these mimics. Biopsy may still be required for diagnosis in some cases. The authors describe the typical and atypical imaging findings of PDAC and features that may help to differentiate PDAC from its mimics. ©RSNA, 2023 Online supplemental material is available for this article. Quiz questions for this article are available through the Online Learning Center. See the invited commentary by Zins in this issue.
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Affiliation(s)
- Frank H Miller
- From the Department of Radiology, Northwestern Memorial Hospital, Northwestern University Feinberg School of Medicine, 676 N St. Clair St, Ste 800, Chicago, IL 60611 (F.H.M., C.L.V., N.A.H., P.N., A.J.); and Department of Radiology and Imaging, Medical College of Georgia, Augusta, GA (P.K.M.)
| | - Camila Lopes Vendrami
- From the Department of Radiology, Northwestern Memorial Hospital, Northwestern University Feinberg School of Medicine, 676 N St. Clair St, Ste 800, Chicago, IL 60611 (F.H.M., C.L.V., N.A.H., P.N., A.J.); and Department of Radiology and Imaging, Medical College of Georgia, Augusta, GA (P.K.M.)
| | - Nancy A Hammond
- From the Department of Radiology, Northwestern Memorial Hospital, Northwestern University Feinberg School of Medicine, 676 N St. Clair St, Ste 800, Chicago, IL 60611 (F.H.M., C.L.V., N.A.H., P.N., A.J.); and Department of Radiology and Imaging, Medical College of Georgia, Augusta, GA (P.K.M.)
| | - Pardeep K Mittal
- From the Department of Radiology, Northwestern Memorial Hospital, Northwestern University Feinberg School of Medicine, 676 N St. Clair St, Ste 800, Chicago, IL 60611 (F.H.M., C.L.V., N.A.H., P.N., A.J.); and Department of Radiology and Imaging, Medical College of Georgia, Augusta, GA (P.K.M.)
| | - Paul Nikolaidis
- From the Department of Radiology, Northwestern Memorial Hospital, Northwestern University Feinberg School of Medicine, 676 N St. Clair St, Ste 800, Chicago, IL 60611 (F.H.M., C.L.V., N.A.H., P.N., A.J.); and Department of Radiology and Imaging, Medical College of Georgia, Augusta, GA (P.K.M.)
| | - Anugayathri Jawahar
- From the Department of Radiology, Northwestern Memorial Hospital, Northwestern University Feinberg School of Medicine, 676 N St. Clair St, Ste 800, Chicago, IL 60611 (F.H.M., C.L.V., N.A.H., P.N., A.J.); and Department of Radiology and Imaging, Medical College of Georgia, Augusta, GA (P.K.M.)
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Agboola AA, Mohamed KH, Syed M, Shiwlani S, Butt R, Reza RR, Haseeb M, Nasir H. Type 1 Autoimmune Pancreatitis Masquerading as Pancreatic Head Carcinoma. Cureus 2023; 15:e47471. [PMID: 38022068 PMCID: PMC10662655 DOI: 10.7759/cureus.47471] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/22/2023] [Indexed: 12/01/2023] Open
Abstract
Obstructive jaundice is a joint clinical presentation with many etiologies, including pancreatic cancer and autoimmune pancreatitis (AIP). Differentiating between these two conditions is pivotal due to the divergent management approaches and prognoses. In this case report, we present a case of a 49-year-old female patient who presented with weight loss, intermittent chronic abdominal pain, and jaundice. She was initially suspected of having pancreatic cancer because of clinical presentation and imaging findings. However, she was ultimately diagnosed with Type 1 AIP due to histopathology findings and elevated immunoglobulin G4. This case highlights the complexities in diagnosis, the role of advanced imaging techniques and tissue sampling, and the lessons learned regarding managing this challenging clinical scenario.
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Affiliation(s)
| | - Khalid H Mohamed
- Neurology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, GBR
| | - Maria Syed
- Surgery, Aga Khan University Hospital, Karachi, PAK
| | | | - Rowaida Butt
- Family Medicine, Avalon University School of Medicine, Ohio, USA
| | | | - Muhammad Haseeb
- Internal Medicine, Allama Iqbal Medical College, Lahore, PAK
- Internal Medicine, Bahria International Hospital, Lahore, PAK
| | - Hira Nasir
- Internal Medicine, Mayo Hospital, Lahore, PAK
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Alius C, Serban D, Tribus LC, Costea DO, Cristea BM, Serboiu C, Motofei I, Dascalu AM, Velescu B, Tudor C, Socea B, Bobirca A, Vancea G, Tanasescu D, Bratu DG. When Not to Operate on Acute Cases-A Surgeon's Perspective on Rapid Assessment of Emergency Abdominopelvic Computed Tomography. J Imaging 2023; 9:200. [PMID: 37888307 PMCID: PMC10607302 DOI: 10.3390/jimaging9100200] [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: 08/26/2023] [Revised: 09/21/2023] [Accepted: 09/25/2023] [Indexed: 10/28/2023] Open
Abstract
Clinical problem solving evolves in parallel with advances in technology and discoveries in the medical field. However, it always reverts to basic cognitive processes involved in critical thinking, such as hypothetical-deductive reasoning, pattern recognition, and compilation models. When dealing with cases of acute abdominal pain, clinicians should employ all available tools that allow them to rapidly refine their analysis for a definitive diagnosis. Therefore, we propose a standardized method for the quick assessment of abdominopelvic computed tomography as a supplement to the traditional clinical reasoning process. This narrative review explores the cognitive basis of errors in reading imaging. It explains the practical use of attenuation values, contrast phases, and windowing for non-radiologists and details a multistep protocol for finding radiological cues during CT reading and interpretation. This systematic approach describes the salient features and technical tools needed to ascertain the causality between clinical patterns and abdominopelvic changes visible on CT scans from a surgeon's perspective. It comprises 16 sections that should be read successively and that cover the entire abdominopelvic region. Each section details specific radiological signs and provides clear explanations for targeted searches, as well as anatomical and technical hints. Reliance on imaging in clinical problem solving does not make a decision dichotomous nor does it guarantee success in diagnostic endeavors. However, it contributes exact information for supporting the clinical assessments even in the most subtle and intricate conditions.
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Affiliation(s)
- Catalin Alius
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania; (C.A.); (B.M.C.); (C.S.); (I.M.); (A.M.D.); (C.T.); (B.S.); (A.B.); (G.V.)
- Fourth General Surgery Department, Emergency University Hospital Bucharest, 050098 Bucharest, Romania
| | - Dragos Serban
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania; (C.A.); (B.M.C.); (C.S.); (I.M.); (A.M.D.); (C.T.); (B.S.); (A.B.); (G.V.)
- Fourth General Surgery Department, Emergency University Hospital Bucharest, 050098 Bucharest, Romania
| | - Laura Carina Tribus
- Faculty of Dental Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania
- Department of Internal Medicine, Ilfov Emergency Clinic Hospital Bucharest, 022104 Bucharest, Romania
| | - Daniel Ovidiu Costea
- Faculty of Medicine, Ovidius University Constanta, 900470 Constanta, Romania;
- General Surgery Department, Emergency County Hospital Constanta, 900591 Constanta, Romania
| | - Bogdan Mihai Cristea
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania; (C.A.); (B.M.C.); (C.S.); (I.M.); (A.M.D.); (C.T.); (B.S.); (A.B.); (G.V.)
| | - Crenguta Serboiu
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania; (C.A.); (B.M.C.); (C.S.); (I.M.); (A.M.D.); (C.T.); (B.S.); (A.B.); (G.V.)
| | - Ion Motofei
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania; (C.A.); (B.M.C.); (C.S.); (I.M.); (A.M.D.); (C.T.); (B.S.); (A.B.); (G.V.)
- Department of General Surgery, Emergency Clinic Hospital “Sf. Pantelimon” Bucharest, 021659 Bucharest, Romania
| | - Ana Maria Dascalu
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania; (C.A.); (B.M.C.); (C.S.); (I.M.); (A.M.D.); (C.T.); (B.S.); (A.B.); (G.V.)
| | - Bruno Velescu
- Faculty of Pharmacy, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania
| | - Corneliu Tudor
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania; (C.A.); (B.M.C.); (C.S.); (I.M.); (A.M.D.); (C.T.); (B.S.); (A.B.); (G.V.)
- Fourth General Surgery Department, Emergency University Hospital Bucharest, 050098 Bucharest, Romania
| | - Bogdan Socea
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania; (C.A.); (B.M.C.); (C.S.); (I.M.); (A.M.D.); (C.T.); (B.S.); (A.B.); (G.V.)
- Department of General Surgery, Emergency Clinic Hospital “Sf. Pantelimon” Bucharest, 021659 Bucharest, Romania
| | - Anca Bobirca
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania; (C.A.); (B.M.C.); (C.S.); (I.M.); (A.M.D.); (C.T.); (B.S.); (A.B.); (G.V.)
| | - Geta Vancea
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania; (C.A.); (B.M.C.); (C.S.); (I.M.); (A.M.D.); (C.T.); (B.S.); (A.B.); (G.V.)
- Clinical Hospital of Infectious and Tropical Diseases “Dr. Victor Babes”, 030303 Bucharest, Romania
| | - Denisa Tanasescu
- Department of Nursing and Dentistry, Faculty of General Medicine, ‘Lucian Blaga’ University of Sibiu, 550169 Sibiu, Romania;
| | - Dan Georgian Bratu
- Faculty of Medicine, University “Lucian Blaga”, 550169 Sibiu, Romania;
- Department of Surgery, Emergency County Hospital Sibiu, 550245 Sibiu, Romania
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10
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Cao H, Cao SQ, Gao YT, Du F. Clinical features of IgG4-associated autoimmune pancreatitis and factors related to its recurrence. Shijie Huaren Xiaohua Zazhi 2023; 31:677-687. [DOI: 10.11569/wcjd.v31.i16.677] [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: 06/10/2023] [Revised: 06/21/2023] [Accepted: 08/21/2023] [Indexed: 08/28/2023] Open
Abstract
BACKGROUND IgG4-associated autoimmune pancreatitis (IgG4-AIP) is a kind of autoimmune-mediated pancreatitis. The clinical manifestations of IgG4-AIP are not specific and the differential diagnosis is often difficult, and the indications and course of glucocorticoid treatment for the disease are still controversial. The aim of this study was to improve the understanding, diagnosis, and treatment of the disease by analyzing the clinical characteristics of the disease, and to reduce the recurrence rate of the disease by analyzing the factors related to its recurrence.
AIM To investigate the clinical features, therapeutic efficacy, and recurrence of IgG4-AIP.
METHODS The clinical data of 46 patients with IgG4-AIP were analyzed retrospectively.
RESULTS The male to female ratio of IgG4-AIP patients was 10.5:1. The most common clinical manifestation was obstructive jaundice. Approximately 52.1% of patients had extrapancreatic organ involvement, with the most commonly affected extrapancreatic organ being the bile duct. The vast majority (91.3%) of patients had higher serum IgG4 levels than normal. The imaging findings were diffuse swelling or local enlargement of the pancreas with stricture of the biliary and pancreatic ducts. The majority (81.2%) of patients underwent pancreatic biopsy under the guidance of endoscopic ultrasonography. Typical pathological findings included fibrous tissue proliferation, lymphocyte and plasma cell infiltration, and acinar atrophy of the pancreas. Half of the patients showed a large number of IgG4 positive plasma cells as revealed by immunohistochemistry. Glucocorticoids were effective for IgG4-AIP, but 36.8% of the patients relapsed after remission, and 71.4% relapsed after stopping glucocorticoids. Logistic regression analysis showed that the risk factors for recurrence were pancreatic portal hypertension and involvement of extra-pancreatic organs. Low-dose glucocorticoid maintenance therapy could prevent recurrence.
CONCLUSION Due to the lack of specific clinical manifestation, IgG4-AIP is easily misdiagnosed as pancreatic cancer. The main manifestations of IgG4-AIP are increased serum IgG4 level, diffuse or local swelling of pancreas with biliopancreatic duct stricture, multiple organ involvement, IgG4 positive plasma cell infiltration, tissue fibrosis, and response to treatment with glucocorticoids. The risk factors for recurrence of IgG4-AIP are pancreatic portal hypertension and extrapancreatic organ involvement. Low-dose glucocorticoid maintenance therapy can prevent recurrence.
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Affiliation(s)
- Hui Cao
- Department of Gastroenterology, Liyuan Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430066, Hubei Province, China
| | - Shi-Qiong Cao
- Department of Gastroenterology, Liyuan Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430066, Hubei Province, China
| | - Yu-Tong Gao
- Department of Pathology, Union Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Fan Du
- Department of Gastroenterology, Union Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
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11
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Kamisawa T. Epidemiology of Autoimmune Pancreatitis. THE PANCREAS 2023:533-539. [DOI: 10.1002/9781119876007.ch68] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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12
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Flisfisch S, Schäfer E. Resolution of a periapical lesion in an autoimmune pancreatitis patient treated with long-term low-dose glucocorticoids: a case report. Head Face Med 2023; 19:22. [PMID: 37287065 DOI: 10.1186/s13005-023-00366-1] [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: 03/09/2023] [Accepted: 05/20/2023] [Indexed: 06/09/2023] Open
Abstract
BACKGROUND Patients diagnosed with an autoimmune disease are often systemically medicated with glucocorticoids. Autoimmune pancreatitis (AIP) Type 1 is considered a rare autoimmune disease, which is very well responsive to glucocorticoids and hence can be treated optionally on a long-term basis using low dose of the drug. Apical lesions of root canal-treated teeth can be solved by retreatment of the preexisting root canal obturation or via surgical approaches. CASE PRESENTATION This case report relates to a 76-year-old male patient, whose symptomatic acute apical periodontitis was treated nonsurgically by root canal treatment. However, overtime both roots of tooth 46 were associated with asymptomatic apical lesions. Despite progression of the lesions, due to a painless situation, the patient refrained from proceeding with any further treatment options after explaining the pathological pathway with all its consequences. A few years later the patient was prescribed 2.5 mg glucocorticoid prednisone daily for long-term therapy due to an AIP Type 1. Six years later under strict glucocorticoid therapy the apical lesions healed nearly completely and the patient remained free of symptoms without any further interventions. CONCLUSIONS These observations suggest that prospective clinical studies are required to further elucidate the potential healing effect of systemic long-term low-dose glucocorticoid medication on lesions of endodontic origin.
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Affiliation(s)
- Sohar Flisfisch
- University of Bonn, University of Münster, Private Practice, Steinengraben 67, Basel, 4051, Switzerland.
| | - Edgar Schäfer
- Central Interdisciplinary Ambulance in the School of Dentistry, University of Münster, Albert-Schweitzer-Campus 1, building W 30, 48149, Münster, Germany
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13
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Kim SH, Lee YC, Chon HK. Challenges for clinicians treating autoimmune pancreatitis: Current perspectives. World J Clin Cases 2023; 11:30-46. [PMID: 36687190 PMCID: PMC9846983 DOI: 10.12998/wjcc.v11.i1.30] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/12/2022] [Revised: 10/31/2022] [Accepted: 12/19/2022] [Indexed: 01/04/2023] Open
Abstract
Autoimmune pancreatitis (AIP) is a rare disease clinically characterized by obstructive jaundice, unintentional weight loss, acute pancreatitis, focal pancreatic mass, and diabetes. AIP is classified into two subtypes - type 1 and type 2 - according to pathological findings, clinical features, and serology test results, but some cases may be defined as type not otherwise in the absence of pathological findings and inflammatory bowel disease. To address the differences in diagnostic criteria by country, standard diagnostic criteria for AIP were proposed in 2011 by an international consensus of expert opinions. Differential diagnosis of AIP from pancreatic ductal adenocarcinoma is important but remains challenging for clinicians. Fortunately, all subtypes of AIP show dramatic response to steroid treatment. This review discusses the current perspectives on the diagnosis and management of AIP in clinical practice.
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Affiliation(s)
- Seong-Hun Kim
- Division of Gastroenterology, Department of Internal Medicine, Jeonbuk National University Medical School, Jeonju 54907, South Korea
| | - Yun Chae Lee
- Division of Gastroenterology, Department of Internal Medicine, Jeonbuk National University Medical School, Jeonju 54907, South Korea
| | - Hyung Ku Chon
- Department of Internal Medicine, Institution of Wonkwang Medical Science, Wonkwang University School of Medicine and Hospital, Iksan 54538, South Korea
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14
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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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15
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Marya NB, Powers PD, Chari ST, Gleeson FC, Leggett CL, Abu Dayyeh BK, Chandrasekhara V, Iyer PG, Majumder S, Pearson RK, Petersen BT, Rajan E, Sawas T, Storm AC, Vege SS, Chen S, Long Z, Hough DM, Mara K, Levy MJ. Utilisation of artificial intelligence for the development of an EUS-convolutional neural network model trained to enhance the diagnosis of autoimmune pancreatitis. Gut 2021; 70:1335-1344. [PMID: 33028668 DOI: 10.1136/gutjnl-2020-322821] [Citation(s) in RCA: 71] [Impact Index Per Article: 17.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/13/2020] [Revised: 09/10/2020] [Accepted: 09/15/2020] [Indexed: 02/06/2023]
Abstract
OBJECTIVE The diagnosis of autoimmune pancreatitis (AIP) is challenging. Sonographic and cross-sectional imaging findings of AIP closely mimic pancreatic ductal adenocarcinoma (PDAC) and techniques for tissue sampling of AIP are suboptimal. These limitations often result in delayed or failed diagnosis, which negatively impact patient management and outcomes. This study aimed to create an endoscopic ultrasound (EUS)-based convolutional neural network (CNN) model trained to differentiate AIP from PDAC, chronic pancreatitis (CP) and normal pancreas (NP), with sufficient performance to analyse EUS video in real time. DESIGN A database of still image and video data obtained from EUS examinations of cases of AIP, PDAC, CP and NP was used to develop a CNN. Occlusion heatmap analysis was used to identify sonographic features the CNN valued when differentiating AIP from PDAC. RESULTS From 583 patients (146 AIP, 292 PDAC, 72 CP and 73 NP), a total of 1 174 461 unique EUS images were extracted. For video data, the CNN processed 955 EUS frames per second and was: 99% sensitive, 98% specific for distinguishing AIP from NP; 94% sensitive, 71% specific for distinguishing AIP from CP; 90% sensitive, 93% specific for distinguishing AIP from PDAC; and 90% sensitive, 85% specific for distinguishing AIP from all studied conditions (ie, PDAC, CP and NP). CONCLUSION The developed EUS-CNN model accurately differentiated AIP from PDAC and benign pancreatic conditions, thereby offering the capability of earlier and more accurate diagnosis. Use of this model offers the potential for more timely and appropriate patient care and improved outcome.
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Affiliation(s)
- Neil B Marya
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | | | - Suresh T Chari
- Gastroenterology, Hepatology and Nutrition, University of Texas MD Anderson Cancer Center, Houston, Texas, USA
| | - Ferga C Gleeson
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Cadman L Leggett
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | | | | | - Prasad G Iyer
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Shounak Majumder
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Randall K Pearson
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Bret T Petersen
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Elizabeth Rajan
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Tarek Sawas
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Andrew C Storm
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Santhi S Vege
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
| | - Shigao Chen
- Diagnostic Radiology, Mayo Clinic, Rochester, Minnesota, USA
| | - Zaiyang Long
- Diagnostic Radiology, Mayo Clinic, Rochester, Minnesota, USA
| | - David M Hough
- Diagnostic Radiology, Mayo Clinic, Rochester, Minnesota, USA
| | - Kristin Mara
- Biomedical Statistics and Informatics, Mayo Clinic Rochester, Rochester, Minnesota, USA
| | - Michael J Levy
- Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
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16
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Mankoo R, Ali AH, Hammoud GM. Use of artificial intelligence in endoscopic ultrasound evaluation of pancreatic pathologies. Artif Intell Gastrointest Endosc 2021; 2:89-94. [DOI: 10.37126/aige.v2.i3.89] [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] [Received: 06/02/2021] [Revised: 06/20/2021] [Accepted: 06/28/2021] [Indexed: 02/06/2023] Open
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17
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Mankoo R, Ali AH, Hammoud GM. Use of artificial intelligence in endoscopic ultrasound evaluation of pancreatic pathologies. Artif Intell Gastrointest Endosc 2021; 2:88-93. [DOI: 10.37126/aige.v2.i3.88] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
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18
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Ishikawa T, Kawashima H, Ohno E, Iida T, Suzuki H, Uetsuki K, Yamada K, Yashika J, Yoshikawa M, Gibo N, Aoki T, Kataoka K, Mori H, Fujishiro M. Risks and characteristics of pancreatic cancer and pancreatic relapse in autoimmune pancreatitis patients. J Gastroenterol Hepatol 2020; 35:2281-2288. [PMID: 32583452 DOI: 10.1111/jgh.15163] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/07/2020] [Revised: 05/28/2020] [Accepted: 06/22/2020] [Indexed: 12/15/2022]
Abstract
BACKGROUND AND AIM We examined the differences in the risks and characteristics of pancreatic relapse (PR) and pancreatic cancer (PC) in patients with autoimmune pancreatitis (AIP). METHODS We retrospectively reviewed 123 type 1 AIP patients with a median follow-up of 55 months (interquartile range, 27-98). The following items were evaluated: (i) cumulative relapse rates and risk factors, (ii) the incidence of PC, (iii) PR versus PC, and (iv) outcomes after the appearance of morphological changes in the pancreas (focal enlargement, apparent mass lesions, or main pancreatic duct dilation). RESULTS (i) The cumulative PR rates were 1.7% within 1 year, 11.5% within 3 years, and 22.6% within 5 years. Lack of maintenance therapy, IgG4-related sclerosing cholangitis, and IgG4-related kidney disease were identified as independent predictors of relapse. (ii) Two patients (1.6%) were diagnosed with PC at 17 and 22 months after initial AIP diagnosis. (iii) Thirteen (59.1%) and four (18.2%) patients with PR had focal enlargement and main pancreatic duct dilation, respectively. The median CA19-9 level at initial diagnosis was significantly higher in PC patients (21 vs 220.5 U/mL, P = 0.014). (iv) Eight PR patients underwent endoscopic ultrasound-guided fine-needle aspiration, none of whom had malignant findings. PC was diagnosed by ultrasound-guided fine-needle aspiration in both cancer patients. CONCLUSIONS Although the incidence of PC is low, it may mimic PR in AIP patients. Surveillance is important, and when morphological changes occur, biopsy and evaluation of serum IgG4 and CA19-9 levels (particularly if the levels were high before) should be considered.
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Affiliation(s)
- Takuya Ishikawa
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Hiroki Kawashima
- Department of Endoscopy, Nagoya University Hospital, Nagoya, Japan
| | - Eizaburo Ohno
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Tadashi Iida
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Hirotaka Suzuki
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Kota Uetsuki
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Kenta Yamada
- Department of Endoscopy, Nagoya University Hospital, Nagoya, Japan
| | - Jun Yashika
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Masakatsu Yoshikawa
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Noriaki Gibo
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Toshinori Aoki
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Kunio Kataoka
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Hiroshi Mori
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Mitsuhiro Fujishiro
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
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19
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Pelaez-Luna M, Soriano-Rios A, Lira-Treviño AC, Uscanga-Domínguez L. Steroid-responsive pancreatitides. World J Clin Cases 2020; 8:3411-3430. [PMID: 32913848 PMCID: PMC7457102 DOI: 10.12998/wjcc.v8.i16.3411] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/01/2020] [Revised: 07/03/2020] [Accepted: 07/30/2020] [Indexed: 02/05/2023] Open
Abstract
Autoimmune pancreatitis has received considerable attention, especially due to the marked effect of corticosteroid therapy on its clinical course. Knowledge, especially regarding type 1 autoimmune pancreatitis, has significantly increased over the last decades, and despite significant differences in pathophysiology and outcomes, both type 1 and 2 autoimmune pancreatitis are still considered different types of the same disease. Some have proposed a different nomenclature reflecting these differences. Although the term steroid-responsive pancreatitides may be interpreted as synonymous to type 1 and 2 autoimmune pancreatitis, these are not the only pancreatic conditions that show a response to steroid therapy. Acute pancreatitis caused by vasculitis and connective tissue diseases and acute pancreatitis secondary to checkpoint inhibitors or programmed cell death receptor antibody-mediated blockage cancer therapy may also benefit from steroid treatment. This review presents current concepts on these disorders, aiming to increase awareness, analyze similarities and differences, and propose a new nomenclature that reflects their specific particularities, clustering them under the term "steroid-responsive pancreatitides".
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Affiliation(s)
- Mario Pelaez-Luna
- Research Division School of Medicine, Universidad Nacional Autonoma de México, Department of Gastroenterology, National Institute of Medical Sciences and Nutrition "Salvador Zubiran" Mexico City 14000, Mexico
| | - Andrea Soriano-Rios
- Department of Gastroenterology, National Institute of Medical Sciences and Nutrition "Salvador Zubiran" Mexico City 14000, Mexico
| | - Ana C Lira-Treviño
- Department of Gastroenterology, National Institute of Medical Sciences and Nutrition "Salvador Zubiran" Mexico City 14000, Mexico
| | - Luis Uscanga-Domínguez
- Department of Gastroenterology, National Institute of Medical Sciences and Nutrition "Salvador Zubiran" Mexico City 14000, Mexico
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20
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IgG4-related disease in the abdomen and pelvis: atypical findings, pitfalls, and mimics. Abdom Radiol (NY) 2020; 45:2485-2499. [PMID: 32300834 DOI: 10.1007/s00261-020-02526-2] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
IgG4-related disease (IgG4-RD) is a systemic, autoimmune, fibroinflammatory disease that can cause multi-organ damage. Although there have been many trials and studies since its recognition in 2003, there is still much that is unknown. Furthermore, IgG4-RD can affect any organ in the body and often has many mimics and alternative diagnoses, which can make for a challenging workup. Imaging plays a substantial role in the diagnosis of IgG4-RD and is often the first occasion where IgG4-RD comes into consideration. Thus, knowledge about the imaging findings of various manifestations of IgG4-RD can aid in the diagnosis and have a significant impact on patient management. In this article, we review the wide array of imaging findings, both typical and atypical, as well as possible mimics of IgG4-RD in the abdomen and pelvis.
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21
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Samji V, Haykal T, Danish R, Bachuwa G. A Case of an IgG4-Related Disease Mimicking Malignancy and Resolving With Steroids. Cureus 2020; 12:e9476. [PMID: 32874805 PMCID: PMC7455463 DOI: 10.7759/cureus.9476] [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: 11/12/2022] Open
Abstract
A 77-year-old African American female was referred to oncology for evaluation of an adrenal fossa mass detected on computed tomography scan of the abdomen and pelvis (CT-scan A/P) that was ordered as a work-up for painless hematuria. Further evaluation by positron emission tomography (PET) scan showed hypermetabolic masses in the left suprarenal and right iliac region. The biopsy of the right iliac mass was consistent with IgG4-related disease (IgG4RD). It was supported by an elevated serum IgG4 level. She was treated with prednisone with a good response.
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22
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Clinical features and outcomes of IgG4-related idiopathic orbital inflammatory disease: from a large southern China-based cohort. Eye (Lond) 2020; 35:1248-1255. [PMID: 32661337 DOI: 10.1038/s41433-020-1083-x] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2019] [Revised: 11/13/2019] [Accepted: 07/01/2020] [Indexed: 12/31/2022] Open
Abstract
PURPOSE To investigate the clinical features and treatment outcomes of IgG4-related ophthalmic disease (IgG4-ROD) among idiopathic orbital inflammatory disease (IOID) patients. METHODS The medical records of 165 biopsy-proven IOID patients were retrospectively reviewed. Biopsy specimens were immunostained to detect IgG4 and IgG, and data regarding the clinicopathologic features, treatment outcomes, and recurrence were analyzed. RESULTS Among the 165 IOID patients enrolled, 100 (60.6%) were histopathologically IgG4-positive. The IgG4-positive patients had a lower rate of painful swelling or mass (17.0% versus 33.8%, p = 0.013), a longer symptom duration (p = 0.070), and a lower proportion of eyelid hyperemia (39.0% versus 58.5%, p = 0.014) than the IgG4-negative patients. Increased Ki-67 expression (15.02 ± 6.86%, p < 0.001) was observed in the IgG4-positive patients with characteristic pathological manifestations (more lymphocyte infiltration, nodular plasma cell infiltration, and follicular hyperplasia). IgG4-positive group had a higher recurrence rate in the subgroup of patients treated with surgery plus oral glucocorticoids (p = 0.046), and combined radiotherapy group has a higher cumulative proportion with recurrence (p = 0.011). CONCLUSION Over 60% of biopsy-proven IOID were classified as IgG4-ROD with a stronger proliferation potential. Additional radiotherapy after surgical debulking with oral corticosteroids still has a higher relapse rate, and more effective treatments are needed to prevent recurrence.
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23
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Löhr JM, Beuers U, Vujasinovic M, Alvaro D, Frøkjær JB, Buttgereit F, Capurso G, Culver EL, de-Madaria E, Della-Torre E, Detlefsen S, Dominguez-Muñoz E, Czubkowski P, Ewald N, Frulloni L, Gubergrits N, Duman DG, Hackert T, Iglesias-Garcia J, Kartalis N, Laghi A, Lammert F, Lindgren F, Okhlobystin A, Oracz G, Parniczky A, Mucelli RMP, Rebours V, Rosendahl J, Schleinitz N, Schneider A, van Bommel EFH, Verbeke CS, Vullierme MP, Witt H. European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations. United European Gastroenterol J 2020; 8:637-666. [PMID: 32552502 PMCID: PMC7437085 DOI: 10.1177/2050640620934911] [Citation(s) in RCA: 133] [Impact Index Per Article: 26.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2020] [Accepted: 05/04/2020] [Indexed: 12/12/2022] Open
Abstract
The overall objective of these guidelines is to provide evidence-based recommendations for the diagnosis and management of immunoglobulin G4 (IgG4)-related digestive disease in adults and children. IgG4-related digestive disease can be diagnosed only with a comprehensive work-up that includes histology, organ morphology at imaging, serology, search for other organ involvement, and response to glucocorticoid treatment. Indications for treatment are symptomatic patients with obstructive jaundice, abdominal pain, posterior pancreatic pain, and involvement of extra-pancreatic digestive organs, including IgG4-related cholangitis. Treatment with glucocorticoids should be weight-based and initiated at a dose of 0.6-0.8 mg/kg body weight/day orally (typical starting dose 30-40 mg/day prednisone equivalent) for 1 month to induce remission and then be tapered within two additional months. Response to initial treatment should be assessed at week 2-4 with clinical, biochemical and morphological markers. Maintenance treatment with glucocorticoids should be considered in multi-organ disease or history of relapse. If there is no change in disease activity and burden within 3 months, the diagnosis should be reconsidered. If the disease relapsed during the 3 months of treatment, immunosuppressive drugs should be added.
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Affiliation(s)
- J-Matthias Löhr
- Department of Upper Gastrointestinal Diseases, Karolinska University Hospital, Stockholm, Sweden and Department of Clinical Science, Intervention and Technology (CLINTEC), Karolinska Institutet, Stockholm, Sweden
| | - Ulrich Beuers
- Department of Gastroenterology and Hepatology, Amsterdam University Medical Centers, location AMC, Amsterdam, the Netherlands
| | - Miroslav Vujasinovic
- Department of Upper Gastrointestinal Diseases, Karolinska University Hospital, Stockholm, Sweden and Department of Medicine Huddinge, Karolinska Institutet, Stockholm, Sweden
| | - Domenico Alvaro
- Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
| | | | - Frank Buttgereit
- Department of Rheumatology and Clinical Immunology, Charité University Medicine Berlin, Berlin, Germany
| | - Gabriele Capurso
- PancreatoBiliary Endoscopy and EUS Division Pancreas Translational and Clinical Research Center IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Emma L Culver
- Translational Gastroenterology Unit, John Radcliffe Hospital and Nuffield Department of Medicine, University of Oxford, Oxford, UK
| | - Enrique de-Madaria
- Gastroenterology Department, Alicante University General Hospital, ISABIAL, Alicante, Spain
| | - Emanuel Della-Torre
- School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Disease (UnIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Sönke Detlefsen
- Department of Pathology, Odense Pancreas Center (OPAC), Odense University Hospital, Odense, Denmark
| | - Enrique Dominguez-Muñoz
- Department of Gastroenterology and Hepatology, University Hospital of Santiago de Compostela, Santiago de Compostela, Spain
| | - Piotr Czubkowski
- Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children’s Memorial Health Institute, Warsaw, Poland
| | - Nils Ewald
- Institute of Endocrinology, Diabetology and Metabolism, Johannes Wesling University hospital, Minden, Germany and Justus Liebig University Giessen, Giessen, Germany
| | - Luca Frulloni
- Department of Medicine, Pancreas Institute, University of Verona, Verona, Italy
| | - Natalya Gubergrits
- Department of Internal Medicine, Donetsk National Medical University, Lyman, Ukraine
| | - Deniz Guney Duman
- Department of Gastroenterology, School of Medicine, Marmara University, Istanbul, Turkey
| | - Thilo Hackert
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - Julio Iglesias-Garcia
- Department of Gastroenterology and Hepatology, University Hospital of Santiago de Compostela, Santiago de Compostela, Spain
| | - Nikolaos Kartalis
- Department of Abdominal Radiology, Karolinska University Hospital, Stockholm, Sweden
| | - Andrea Laghi
- Department of Surgical and Medical Sciences and Translational Medicine, Sapienza University of Rome, Sant’Andrea Hospital, Rome, Italy
| | - Frank Lammert
- Department of Medicine II, Saarland University Medical Center, Homburg, Germany
| | - Fredrik Lindgren
- Department of Pediatric Gastroenterology, Hepatology and Nutrition, Karolinska University Hospital, Stockholm, Sweden
| | | | - Grzegorz Oracz
- Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children’s Memorial Health Institute, Warsaw, Poland
| | - Andrea Parniczky
- Institute for Translational Medicine, Szentágothai Research Centre, Medical School, University of Pécs, Pécs, Hungary; Heim Pál National Insitute of Pediatrics, Budapest, Hungary
| | | | - Vinciane Rebours
- Pancreatology Department, Beaujon Hospital, Clichy, Université de Paris, France
| | - Jonas Rosendahl
- Department of Internal Medicine I, Martin Luther University, Halle, Germany
| | - Nicolas Schleinitz
- Département de Médicine Interne Timone, Assistance Publique-Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France
| | - Alexander Schneider
- Department of Gastroenterology and Hepatology, Klinikum Bad Hersfeld, Bad Hersfeld, Germany
| | - Eric FH van Bommel
- Department of Internal Medicine, Dutch National Center of Expertise Retroperitoneal Fibrosis, Albert Schweitzer hospital, Dordrecht, the Netherlands
| | | | | | - Heiko Witt
- Else Kröner-Fresenius-Zentrum für Ernährungsmedizin, Paediatric Nutritional Medicine, Technische Universität München, Freising, Germany
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Abstract
OBJECTIVE Since the time of inception of autoimmune pancreatitis (AIP), our knowledge of autoimmune pancreatitis has expanded significantly. The aim of this review is to provide an update on clinical manifestations, diagnosis, imaging features, and treatment of AIP. BACKGROUND AND CLINICAL SIGNIFICANCE Type 1 AIP is the pancreatic manifestation of IgG4-related systemic disease, which can be diagnosed using a combination of clinical, histopathological, pancreatic imaging findings in conjunction with manifestation in other organs, as well of responsiveness to steroid treatment. It is vital to differentiate AIP from pancreatic cancer since both can mimic each other clinically and radiologically. Type 2 AIP is a rare but distinct subtype of AIP which occurs mostly in the younger patient. CONCLUSION AIP is steroid-responsive chronic pancreatitis with distinct manifestations on imaging.
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Affiliation(s)
- Ashish Khandelwal
- Department of Radiology, Mayo Clinic, 200 First St, Rochester, MN, 55902, USA
| | - Dai Inoue
- Department of Radiology, Kanazawa Univeristy, 1192 Kakumamachi, Kanazawa, Ishikawa, 920-1192, Japan
| | - Naoki Takahashi
- Department of Radiology, Mayo Clinic, 200 First St, Rochester, MN, 55902, USA.
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Chaudhary FMD, Tameez Ud Din A, Siddiqui K, Tameez Ud Din A, Chaudhary SMD. Type 1 Autoimmune Pancreatitis in a Young Male. Cureus 2020; 12:e7676. [PMID: 32426188 PMCID: PMC7228786 DOI: 10.7759/cureus.7676] [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: 11/20/2022] Open
Abstract
Autoimmune pancreatitis (AIP) is a rare entity leading to inflammation of the pancreas. It can be broadly categorized into two types. Type 1 AIP is more common and primarily presents with jaundice. Less commonly it can also progress to multiorgan involvement. Here we report a case of a 19-year-old male who presented to us with complaints of abdominal pain and vomiting. His laboratory investigations showed raised serum amylase and lipase. A contrast-enhanced CT revealed a diffuse enlargement of the pancreas with internal low-density foci. Due to the repeated episodes of pancreatitis, the patient's blood was tested for serum IgG4 (immunoglobulin type G4) which was markedly elevated pointing toward the diagnosis of AIP. Endoscopic ultrasound (EUS) showed a sausage-shaped pancreas with hyper- and hypoechoic strands. EUS-guided fine needle aspiration cytology of the lymph nodes performed in the celiac region showed a mixed population of lymphoid cells. Based on all the workup, our patient was diagnosed as type 1 AIP. He was managed with steroids and his condition progressively improved. This case is clinically significant because of the close resemblance of AIP with other pancreatic disorders like neoplasm. A timely diagnosis can prevent the unnecessary performance of invasive procedures in these patients.
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Affiliation(s)
- Farooq Mohyud Din Chaudhary
- Gastroenterology, Mohyud Din Clinic, Multan, PAK.,Gastroenterology, Nishtar Medical University & Hospital, Multan, PAK
| | | | | | | | - Sana Mohyud Din Chaudhary
- Internal Medicine, Combined Military Hospital Lahore Medical College & Institute of Dentistry, Lahore, PAK
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26
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Masamune A, Kikuta K, Hamada S, Tsuji I, Takeyama Y, Shimosegawa T, Okazaki K. Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2016. J Gastroenterol 2020; 55:462-470. [PMID: 31872350 DOI: 10.1007/s00535-019-01658-7] [Citation(s) in RCA: 101] [Impact Index Per Article: 20.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2019] [Accepted: 12/11/2019] [Indexed: 02/07/2023]
Abstract
BACKGROUND To further clarify the clinico-epidemiological features of autoimmune pancreatitis (AIP) in Japan, we conducted the fourth nationwide epidemiological survey. METHODS This study consisted of two stage surveys; the number of AIP patients was estimated by the first survey and their clinical features were assessed by the second survey. We surveyed the AIP patients who had visited hospitals in 2016. RESULTS The estimated number of AIP patients in 2016 was 13,436, with an overall prevalence rate of 10.1 per 100,000 persons. The estimated number of newly diagnosed patients was 3984, with an annual incidence rate of 3.1 per 100,000 persons. Compared to the 2011 survey, both numbers more than doubled. We obtained detailed clinical information of 1474 AIP patients. The male-to-female sex ratio was 2.94, the mean age was 68.1, and mean age at diagnosis was 64.8. At diagnosis, 63% patients were symptomatic and nearly half of them presented jaundice. Pancreatic cysts were found in 9% of the patients and calcifications in 6%. Histopathological examination was performed in 64%, mainly by endoscopic ultrasonography-guided fine needle aspiration. Extra-pancreatic lesions were detected in 60% of the patients. Eighty-four % patients received the initial steroid therapy, and 85% received maintenance steroid therapy. Kaplan-Meier analysis revealed that the relapsed survival was 14% at 3 years, 25% at 5 years, 40% at 10 years, and 50% at 15 years. Mortality was favorable, but pancreatic cancer accounted for death in one quarter of fatal cases. CONCLUSION We clarified the current status of AIP in Japan.
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Affiliation(s)
- Atsushi Masamune
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
| | - Kazuhiro Kikuta
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan
| | - Shin Hamada
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan
| | - Ichiro Tsuji
- Division of Epidemiology, Department of Public Health and Forensic Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - Yoshifumi Takeyama
- Department of Surgery, Kindai University Faculty of Medicine, Osaka, Japan
| | - Tooru Shimosegawa
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan
| | - Kazuichi Okazaki
- Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Japan
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27
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Patil RP, Patil PD, Thakar AB. Panchakarma in autoimmune pancreatitis: A single-case study. Ayu 2019; 40:242-246. [PMID: 33935442 PMCID: PMC8078606 DOI: 10.4103/ayu.ayu_15_20] [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] [Received: 01/23/2020] [Revised: 03/05/2020] [Accepted: 07/20/2020] [Indexed: 11/10/2022] Open
Abstract
Autoimmune pancreatitis (AIP) is the pancreatic manifestation of a systemic fibro-inflammatory disorder. AIP is a unique form of pancreatitis in which autoimmune mechanisms are suspected to be involved in the pathogenesis. AIP is a rare disorder, its exact cause is unknown, but it is thought to be caused by the body’s immune system attacking the pancreas and it responds to steroid therapy only. In Ayurveda, although there is no synonym for AIP, but has a resemblance in clinical features of Grahani Dosha (derangement of duodenum and intestine). The cause of Grahani Dosha is Mandagni (hypofunctioning of Agni) and Panchakarma therapy increases Agni. As per Charaka Samhita, treatment for Grahani Dosha amongst the Panchakarma therapy is Virechana (therapeutic purgation) and Basti (medicated enema). The present case report is of a 30-year-old female, diagnosed as case of AIP with multisystem involvement with increased level of immunoglobulin G (IgG), glycosylated heamoglobin (HbA1c), cholesterol, triglycerides, low-density lipoprotein (LDL) and body mass index (BMI). The patient was on anticholinergic agents, antacids, levothyroxine, multivitamin along with iron and antihistamine drugs since 1 year, but with not much relief. Patient was treated with classical Virechana and MadhutailikaBasti. It was observed after the completion of therapy, that there was decrease in IgG, HbA1c, S. cholesterol, S. triglyceride, low density lipoprotein (LDL) and body mass index (BMI). This shows that Virechana and Basti play a significant role in patient with AIP associated with other disorders.
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Affiliation(s)
- Rajkala P Patil
- Department of Panchakarma, Central Ayurveda Research Institute for Cardiovascular Diseases, Punjabi Bagh, India
| | - Panchakshari D Patil
- Publication Section, Central Council for Research in Ayurvedic Sciences, Janakpuri, New Delhi, India
| | - Anup B Thakar
- Department of Panchakarma, Institute of Teaching and Research in Ayurveda, Jamnagar, Gujarat, India
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28
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Abstract
Autoimmune pancreatitis (AIP) is an entity that has been recognized since 1961. Prior to the discovery of elevated serum IgG4 as a useful biomarker for its diagnosis, Dr. Yoshida in 1995 first described the entity of AIP, which in retrospect closely resembles the current concept of type 1 AIP. Since the discovery of IgG4 as a biomarker (the IgG4-era), a novel concept of IgG4-related disease (IgG4-RD) has been accepted as being comprised of two subtypes of AIP: type 1 defined as the pancreatic manifestation of IgG4-RD, and type 2 characterized by granulocytic epithelial lesions. The characteristic features of type 1 AIP are increased serum IgG4 levels, lymphoplasmacytic sclerosing pancreatitis (abundant infiltration of IgG4+ plasmocytes and lymphocytes, storiform fibrosis, and obliterative phlebitis), extrapancreatic manifestations of IgG4-RD (e.g., sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis), and steroid responsiveness. These entities can be differentiated from mimickers by a combination of serum IgG4 level, imaging features, and histopathological findings. The current first-line therapy is corticosteroids, or rituximab in high-risk patients with steroid intolerance. Although relapse rates are high, treatment of relapsed disease remains experimental.
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Affiliation(s)
- Kazuichi Okazaki
- Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, Hirakata, Osaka, 573-1197, Japan.
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29
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Schreyer AG. [Inflammatory and infectious abdominal peritoneal and mesenterial processes]. Radiologe 2019; 58:10-18. [PMID: 29236138 DOI: 10.1007/s00117-017-0334-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022]
Abstract
BACKGROUND Mesenterial and peritoneal inflammation represents a major clinical challenge regarding differential diagnosis. In this review article, a systematic overview of the most common causes such as autoimmune reaction, infarction with consecutive necrosis and infection caused by protozoa or bacteria is provided. RESULTS The common clinical features of all inflammatory peritoneal and mesenterial diseases are the nonspecific abdominal symptoms. Even in radiological imaging an overlap of imaging features such as lymphadenopathy and nodular mesenteric structures is typical. Frequently the radiologist can narrow the differential diagnosis based on clinical information and anamnesis resulting in an optimized clinical outcome. CONCLUSION Having a huge spectrum of etiologies for inflammatory peritoneal and mesenterial conditions the radiologist has to consider autoimmune diseases (IgG4-associated disease), acute infarction with necrosis and bacterial or protozoan infection.
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Affiliation(s)
- A G Schreyer
- Institut für Röntgendiagnostik, Universitätsklinikum Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Deutschland.
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30
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Abstract
Chronic pancreatitis (CP) is an important gastrointestinal cause of morbidity worldwide. It can severely impair the quality of life besides life-threatening acute and long-term complications. Pain and pancreatic exocrine insufficiency (leading to malnutrition) impact the quality of life. Acute complications include pseudocysts, pancreatic ascites, and vascular complications. Long-term complications are diabetes mellitus and pancreatic cancer. Early diagnosis of CP is crucial to alter the natural course of the disease. However, majority of the cases are diagnosed in the advanced stage. The role of various imaging techniques in the diagnosis of CP is discussed in this review.
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Affiliation(s)
- Rohan Kamat
- Department of Radiodiagnosis and Imaging, PGIMER, Chandigarh, India
| | - Pankaj Gupta
- Department of Gastroenterology, PGIMER, Chandigarh, India
| | - Surinder Rana
- Department of Gastroenterology, PGIMER, Chandigarh, India
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31
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Native T1 mapping of autoimmune pancreatitis as a quantitative outcome surrogate. Eur Radiol 2019; 29:4436-4446. [DOI: 10.1007/s00330-018-5987-9] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/06/2018] [Revised: 12/01/2018] [Accepted: 12/18/2018] [Indexed: 12/19/2022]
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Sandrasegaran K, Menias CO. Imaging in Autoimmune Pancreatitis and Immunoglobulin G4-Related Disease of the Abdomen. Gastroenterol Clin North Am 2018; 47:603-619. [PMID: 30115440 DOI: 10.1016/j.gtc.2018.04.007] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
Autoimmune pancreatitis (AIP) is steroid-responsive fibroinflammatory disorder of the pancreas. There are 2 distinct subtypes of AIP, types 1 and 2. Type 1 is associated with systemic immunoglobulin (Ig)G4 disease and may affect multiple organs in the body. Type 2 is confined to the pancreas and shows an association with ulcerative colitis. This article describes the imaging findings of AIP and IgG4 disease in the liver, bile ducts, kidneys, and the retroperitoneal regions. The imaging differentiation of AIP from pancreas cancer is discussed.
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Affiliation(s)
- Kumaresan Sandrasegaran
- Department of Radiology, Indiana University School of Medicine, 550 North University Boulevard, UH0279, Indianapolis, IN 46202, USA.
| | - Christine O Menias
- Department of Radiology, Mayo Clinic School of Medicine, Mayo Clinic Hospital, 5777 East Mayo Boulevard, Phoenix, AZ 85054, USA
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33
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Pancreatic Pseudocysts and Parenchymal Necrosis in Patients With Autoimmune Pancreatitis: A Systematic Review. Pancreas 2018; 47:952-957. [PMID: 30028447 DOI: 10.1097/mpa.0000000000001121] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
OBJECTIVE To investigate the prevalence and natural history of pancreatic pseudocysts (PCs) and parenchymal necrosis in autoimmune pancreatitis (AIP). METHOD A search using PubMed, Embase, Scopus, and Cochrane was performed. Search terms were AIP, PC, acute fluid collection, and pancreatic necrosis. RESULTS Fifteen studies with 17 patients were included. In 8 of 17 patients, PC was noted concurrently with the AIP diagnosis, whereas in the other half, PC appeared months or years after. In 10 of 17 cases, PC appeared as solitary. The location was variable. Pseudocysts were small (<3 cm) in 4 cases and large (>3 cm) in 13 cases. A normal pancreatic duct was observed in 6 of 17 cases, whereas 9 of 17 had pancreatic duct stenosis. Steroids were given to 4 of 4 small and 10 of 13 large PC. All small PC resolved with steroids, whereas only 4 of 10 large PC treated had some response. Most (9/13) of large PC underwent endoscopic or surgical procedures. None of the 17 cases developed necrosis. CONCLUSIONS Pseudocysts in AIP are rare. Pancreatic pseudocyst can present in variable number, size, and location. Small PC resolved with steroids. Large PC had poor response to steroids requiring invasive interventions. Necrosis in AIP has not been reported.
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34
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Hafezi-Nejad N, Singh VK, Fung C, Takahashi N, Zaheer A. MR Imaging of Autoimmune Pancreatitis. Magn Reson Imaging Clin N Am 2018; 26:463-478. [PMID: 30376982 DOI: 10.1016/j.mric.2018.03.008] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Autoimmune pancreatitis (AIP) is characterized by autoimmune inflammatory destruction of the pancreatic tissue. Imaging plays an essential role in the diagnosis. AIP type 1 is the pancreatic manifestation of immunoglobulin G4 (IgG4)-related disease and is associated with IgG4-positive plasma cell infiltration and fibrosis of multiple organ systems. Type 2 is a related disease with pancreatic inflammation with or without concurrent inflammatory bowel disease. The authors demonstrate the imaging findings that are associated with the pancreatic and extra-pancreatic manifestations of AIP. They emphasize the common MR imaging and magnetic resonance cholangiopancreatography findings to help make the diagnosis of AIP.
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Affiliation(s)
- Nima Hafezi-Nejad
- The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University, 600 North Wolfe Street, Baltimore, MD 21287, USA
| | - Vikesh K Singh
- Department of Internal Medicine, Pancreatitis Center, Johns Hopkins Medical Institutions, 1800 Orleans Street, Sheikh Zayed Tower, Baltimore, MD 21287, USA; Division of Gastroenterology, Johns Hopkins University, School of Medicine, 1800 Orleans Street, Sheikh Zayed Tower, Baltimore, MD 21287, USA
| | - Christopher Fung
- Department of Radiology and Diagnostic Imaging, University of Alberta, 2J2.00 WC Mackenzie Health Sciences Centre, 8440 112 Street Northwest, Edmonton, Alberta T6G 2R7, Canada
| | - Naoki Takahashi
- Department of Radiology, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA
| | - Atif Zaheer
- The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University, 600 North Wolfe Street, Baltimore, MD 21287, USA; Department of Internal Medicine, Pancreatitis Center, Johns Hopkins Medical Institutions, 1800 Orleans Street, Sheikh Zayed Tower, Baltimore, MD 21287, USA.
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35
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Hamano H, Tanaka E, Ishizaka N, Kawa S. IgG4-related Disease - A Systemic Disease that Deserves Attention Regardless of One's Subspecialty. Intern Med 2018; 57:1201-1207. [PMID: 29279491 PMCID: PMC5980798 DOI: 10.2169/internalmedicine.9533-17] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/24/2022] Open
Abstract
IgG4-related disease (IgG4-RD) is an inflammatory condition characterized by a high serum IgG4 concentration and the abundant infiltration of lymphocytes and IgG4-positive plasma cells in the tissue, as well as spatial (diverse clinical manifestations) and temporal (the possibility of recurrence) multiplicities. Since the initial documentation of IgG4-related disease in patients with autoimmune pancreatitis in 2001, a growing body of evidence has been accumulating to suggest that various-virtually all-organs can be affected by IgG4-RD. In general, steroid therapy is effective and is considered to be the first-line treatment for IgG4-RD. The precise mechanism underlying this systemic disorder has remained unknown. Considering that IgG4-RD was specified as being an intractable disease in 2015, further studies are needed to clarify whether IgG4-RD is indeed a distinct disease entity or a complex of disorders of different etiologies and clinical conditions.
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Affiliation(s)
- Hideaki Hamano
- Division of Medical Informatics, Shinshu University Hospital, Japan
- Department of Internal Medicine, Gastroenterology, Shinshu University School of Medicine, Japan
| | - Eiji Tanaka
- Department of Internal Medicine, Gastroenterology, Shinshu University School of Medicine, Japan
| | | | - Shigeyuki Kawa
- Department of Internal Medicine, Matsumoto Dental University, Japan
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36
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OKAZAKI K, UCHIDA K. Current perspectives on autoimmune pancreatitis and IgG4-related disease. PROCEEDINGS OF THE JAPAN ACADEMY. SERIES B, PHYSICAL AND BIOLOGICAL SCIENCES 2018; 94:412-427. [PMID: 30541967 PMCID: PMC6374139 DOI: 10.2183/pjab.94.027] [Citation(s) in RCA: 26] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/12/2023]
Abstract
IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder recognized as a novel clinical entity with either synchronous or metachronous multi-organ involvement. Patients with IgG4-RD show diffuse or focal organ enlargement and mass-forming or nodular/thickened lesions with abundant infiltration of IgG4-positive plasmacytes and fibrosis, and such patients respond well to steroid treatment. It should be differentiated from mimics by a combination of serum IgG4 level, imaging features, and histopathological findings. The current first-line drug is corticosteroids, or rituximab in high-risk patients for steroid intolerance. Although relapse rates are high, standardized protocols for relapsed cases have not been approved yet. Based on genetic factors, disease-specific or -related antigens, abnormal innate and adaptive immunity may be involved, although the precise pathogenic mechanism and long-term outcome still remain unclear.
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Affiliation(s)
- Kazuichi OKAZAKI
- Department of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
- Correspondence should be addressed: K. Okazaki, Department of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010, Japan (e-mail: )
| | - Kazushige UCHIDA
- Department of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
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37
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Weindorf SC, Frederiksen JK. IgG4-Related Disease: A Reminder for Practicing Pathologists. Arch Pathol Lab Med 2017; 141:1476-1483. [DOI: 10.5858/arpa.2017-0257-ra] [Citation(s) in RCA: 44] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease that produces sclerotic, tumefactive masses containing dense lymphoplasmacytic infiltrates rich in immunoglobulin (Ig) G4+ plasma cells. Initially characterized as a form of autoimmune pancreatitis, the distinctive histopathology of IgG4-RD has now been described in almost every organ system. However, because the clinical manifestations of IgG4-RD are diverse and nonspecific, the disease may go unsuspected until a biopsy or resection specimen is obtained to diagnose a presumed malignancy. Pathologists thus play a key role in the diagnosis of IgG4-RD, and familiarity with its histopathologic features is essential to preventing the irreversible comorbidities associated with this treatable disease. This brief review outlines the epidemiology, clinical manifestations, and histopathology of IgG4-RD, with the aim of furthering pathologists' awareness of and ability to diagnose this disorder.
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Affiliation(s)
- Steven C. Weindorf
- From the Department of Pathology, University of Michigan, Ann Arbor. Dr Frederiksen is now with the Department of Pathology, Jackson Memorial Hospital, Miami, Florida
| | - John Karl Frederiksen
- From the Department of Pathology, University of Michigan, Ann Arbor. Dr Frederiksen is now with the Department of Pathology, Jackson Memorial Hospital, Miami, Florida
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38
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Abstract
BACKGROUND IgG4-related diseases are rare systemic multiorgan diseases and can thus affect any organ system. The incidence of diagnosis has significantly increased due to increasing awareness. OBJECTIVE In the abdomen the hepatopancreaticobiliary system provides an essential organ system for the expression of IgG4-associated autoimmune diseases. The focus here is autoimmune pancreatitis type 1 but IgG4-associated sclerosing cholangitis and IgG4-associated hepatopathy, which can also occur in combination are less well-known. METHODS Various mostly Asiatic, histologically-based diagnostic systems, such as HISORt or international consensus diagnostic criteria (ICDC) are available for the diagnostics of hepatopancreaticobiliary IgG4-related autoimmune diseases, in which imaging techniques playing an increasingly important role. RESULTS In addition to generalized organ swelling further morphological and also functional imaging criteria have become increasingly well-known for autoimmune pancreatitis, such as late enhancement or the imaging response to steroid therapy. Magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERP) can provide valuable information for the diagnostics of IgG4-related diseases in the hepatopancreaticobiliary system. CONCLUSION IgG4-related autoimmune diseases of the hepatopancreaticobiliary system are a rare group of diseases in which increasing knowledge of the radiological appearance also leads to an increasingly frequency of diagnosis. IgG4-related diseases must be distinguished from non-necrotizing pancreatitis and pancreatic cancer, which is often difficult but has significant therapeutic consequences for the patients.
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Affiliation(s)
- L Grenacher
- Diagnostik München, Augustenstraße 115, 80798, München, Deutschland.
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39
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Unlu O, Schulman E, Wolff SD, Narula N, Stone JH, Bass A, Erkan D. Immunoglobulin G4-related Aortitis: A Clinical Pathology Conference Held by the Division of Rheumatology at the Hospital for Special Surgery. HSS J 2017; 13:307-312. [PMID: 28983226 PMCID: PMC5617822 DOI: 10.1007/s11420-017-9565-5] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/12/2016] [Accepted: 05/16/2017] [Indexed: 02/07/2023]
Affiliation(s)
- Ozan Unlu
- 0000 0001 2285 8823grid.239915.5Division of Rheumatology, Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021 USA ,000000041936877Xgrid.5386.8Division of Rheumatology, Weill Cornell Medicine, New York, NY USA
| | - Elizabeth Schulman
- 0000 0001 2285 8823grid.239915.5Division of Rheumatology, Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021 USA ,000000041936877Xgrid.5386.8Division of Rheumatology, Weill Cornell Medicine, New York, NY USA
| | | | - Navneet Narula
- 0000 0000 8499 1112grid.413734.6Clinical Pathology and Laboratory Medicine, New York Presbyterian Hospital, New York, NY USA ,000000041936877Xgrid.5386.8Clinical Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY USA
| | - John H. Stone
- 000000041936754Xgrid.38142.3cDivision of Rheumatology, Allergy & Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA USA
| | - Anne Bass
- 0000 0001 2285 8823grid.239915.5Division of Rheumatology, Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021 USA ,000000041936877Xgrid.5386.8Division of Rheumatology, Weill Cornell Medicine, New York, NY USA
| | - Doruk Erkan
- 0000 0001 2285 8823grid.239915.5Division of Rheumatology, Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021 USA ,000000041936877Xgrid.5386.8Division of Rheumatology, Weill Cornell Medicine, New York, NY USA
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40
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Chen YC, Chan HH, Lai KH, Tsai TJ, Hsu PI. Type 1 (IgG4-related) autoimmune pancreatitis: Experiences in a medical center in southern Taiwan within the past 10 years. ADVANCES IN DIGESTIVE MEDICINE 2017. [DOI: 10.1002/aid2.12061] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/07/2022]
Affiliation(s)
- Yen-Chun Chen
- Division of Gastroenterology & Hepatology; Department of Internal Medicine, Kaohsiung Veterans General Hospital; Kaohsiung Taiwan
- Division of Gastroenterology; Department of Internal Medicine, Dalin Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation; Chiayi Taiwan
| | - Hoi-Hung Chan
- Division of Gastroenterology & Hepatology; Department of Internal Medicine, Kaohsiung Veterans General Hospital; Kaohsiung Taiwan
- Department of Biological Sciences; National Sun Yat-sen University; Kaohsiung Taiwan
- College of Pharmacy and Health Care; Tajen University; Pingtung Taiwan
- School of Medicine; National Yang-Ming University; Taipei Taiwan
- Department of Business Management; National Sun Yat-sen University; Kaohsiung Taiwan
| | - Kwok-Hung Lai
- Division of Gastroenterology & Hepatology; Department of Internal Medicine, Kaohsiung Veterans General Hospital; Kaohsiung Taiwan
- School of Medicine; National Yang-Ming University; Taipei Taiwan
| | - Tzung-Jiun Tsai
- Division of Gastroenterology & Hepatology; Department of Internal Medicine, Kaohsiung Veterans General Hospital; Kaohsiung Taiwan
| | - Ping-I Hsu
- Division of Gastroenterology & Hepatology; Department of Internal Medicine, Kaohsiung Veterans General Hospital; Kaohsiung Taiwan
- School of Medicine; National Yang-Ming University; Taipei Taiwan
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Schmocker RK, Vanness DJ, Greenberg CC, Havlena JA, LoConte NK, Weiss JM, Neuman HB, Leverson G, Smith MA, Winslow ER. Utilization of preoperative endoscopic ultrasound for pancreatic adenocarcinoma. HPB (Oxford) 2017; 19:465-472. [PMID: 28237627 PMCID: PMC5695546 DOI: 10.1016/j.hpb.2017.01.017] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/27/2016] [Revised: 12/21/2016] [Accepted: 01/04/2017] [Indexed: 12/12/2022]
Abstract
BACKGROUND Endoscopic ultrasound (EUS) is used for pancreatic adenocarcinoma staging and obtaining a tissue diagnosis. The objective was to determine patterns of preoperative EUS and the impact on downstream treatment. METHODS The Surveillance, Epidemiology, and End Results (SEER) Medicare-linked database was used to identify patients with pancreatic adenocarcinoma. The staging period was the first staging procedure within 6 months of surgery until surgery. Logistic regression was used to determine factors associated with preoperative EUS. The main outcome was EUS in the staging period, with secondary outcomes including number of staging tests and time to surgery. RESULTS 2782 patients were included, 56% were treated at an academic hospital (n = 1563). 1204 patients underwent EUS (43.3%). The factors most associated with receipt of EUS were: earlier year of diagnosis, SEER area, and a NCI or academic hospital (all p < 0.0001). EUS was associated with a longer time to surgery (17.8 days; p < 0.0001), and a higher number of staging tests (40 tests/100 patients; p < 0.0001). CONCLUSIONS Factors most associated with receipt of EUS are geographic, temporal, and institutional, rather than clinical/disease factors. EUS is associated with a longer time to surgery and more preoperative testing, and additional study is needed to determine if EUS is overused.
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Affiliation(s)
- Ryan K Schmocker
- Department of Surgery, University of Wisconsin School of Medicine and Public Health, USA
| | - David J Vanness
- Department of Population Health Sciences, University of Wisconsin School of Medicine and Public Health, USA
| | - Caprice C Greenberg
- Department of Surgery, University of Wisconsin School of Medicine and Public Health, USA; Department of Population Health Sciences, University of Wisconsin School of Medicine and Public Health, USA
| | - Jeff A Havlena
- Department of Surgery, University of Wisconsin School of Medicine and Public Health, USA
| | - Noelle K LoConte
- Department of Medicine, Division of Oncology, University of Wisconsin School of Medicine and Public Health, USA
| | - Jennifer M Weiss
- Department of Medicine, Division of Gastroenterology & Hepatology, University of Wisconsin School of Medicine and Public Health, USA
| | - Heather B Neuman
- Department of Surgery, University of Wisconsin School of Medicine and Public Health, USA
| | - Glen Leverson
- Department of Surgery, University of Wisconsin School of Medicine and Public Health, USA
| | - Maureen A Smith
- Department of Surgery, University of Wisconsin School of Medicine and Public Health, USA; Department of Family Medicine, University of Wisconsin School of Medicine and Public Health, USA
| | - Emily R Winslow
- Department of Surgery, University of Wisconsin School of Medicine and Public Health, USA.
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Macinga P, Pulkertova A, Bajer L, Maluskova J, Oliverius M, Smejkal M, Heczkova M, Spicak J, Hucl T. Simultaneous occurrence of autoimmune pancreatitis and pancreatic cancer in patients resected for focal pancreatic mass. World J Gastroenterol 2017; 23:2185-2193. [PMID: 28405146 PMCID: PMC5374130 DOI: 10.3748/wjg.v23.i12.2185] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/28/2016] [Revised: 01/31/2017] [Accepted: 03/02/2017] [Indexed: 02/06/2023] Open
Abstract
AIM To assess the occurrence of autoimmune pancreatitis (AIP) in pancreatic resections performed for focal pancreatic enlargement.
METHODS We performed a retrospective analysis of medical records of all patients who underwent pancreatic resection for a focal pancreatic enlargement at our tertiary center from January 2000 to July 2013. The indication for surgery was suspicion of a tumor based on clinical presentation, imaging findings and laboratory evaluations. The diagnosis of AIP was based on histology findings. An experienced pathologist specialized in pancreatic disease reviewed all the cases and confirmed the diagnosis in pancreatic resection specimens suggestive of AIP. The histological diagnosis of AIP was set according to the international consensus diagnostic criteria.
RESULTS Two hundred ninety-five pancreatic resections were performed in 201 men and 94 women. AIP was diagnosed in 15 patients (5.1%, 12 men and 3 women) based on histology of the resected specimen. Six of them had AIP type 1, nine were diagnosed with AIP type 2. Pancreatic adenocarcinoma (PC) was also present in six patients with AIP (40%), all six were men. Patients with AIP + PC were significantly older (60.5 vs 49 years of age, P = 0.045), more likely to have been recently diagnosed with diabetes (67% vs 11%, P = 0.09), and had experienced greater weight loss (15.5 kg vs 8.5 kg, P = 0.03) than AIP patients without PC. AIP was not diagnosed in any patients prior to surgery; however, the diagnostic algorithm was not fully completed in every case.
CONCLUSION The possible co-occurrence of PC and AIP suggests that preoperative diagnosis of AIP does not rule out simultaneous presence of PC.
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Ibrahim U, Saqib A, Nalluri N, Raza MR, Goldstein M. IgG4-related Disease Presenting as a Pancreatic Mass and Bilateral Lacrimal Gland Swelling. Cureus 2017; 9:e1054. [PMID: 28367391 PMCID: PMC5364085 DOI: 10.7759/cureus.1054] [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] [Indexed: 11/10/2022] Open
Abstract
IgG4-related disease is characterized by multi-system inflammation and possible elevation in serum immunoglobulin-G4 level. Treatment regimens include glucocorticoids, disease-modifying anti-rheumatic agents and recently, rituximab infusions have been reported to be effective in treatment-refractory disease. We present a case of a 64-year-old male presenting with acute abdominal pain and scleral icterus. An abdominal ultrasound demonstrated extensive biliary ductal dilatation. A computed tomography (CT) scan and a magnetic resonance cholangiopancreatography (MRCP) revealed a 4.8 cm pancreatic head mass. He underwent an exploratory laparotomy, and a pathologic examination of the mass revealed a dense lymphoplasmacytic infiltrate. The patient’s IgG subclass 2 level was elevated. A review of the patient’s medical records revealed that, in 1992, he presented with bilateral periorbital discomfort not severe enough to require intervention. In 2006, he presented with bilateral periorbital swelling and discomfort. A magnetic resonance imaging (MRI) scan showed gross enlargement of the lacrimal glands, and a biopsy revealed a dense lymphoplasmacytic infiltrate. He was treated with corticosteroids, cyclosporine and methotrexate. The regimen was repeated in 2009 for recurrent symptoms. Hence, on our encounter, a diagnosis of IgG4-related disease was made and he was treated with rituximab that resulted in complete remission.
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Affiliation(s)
- Uroosa Ibrahim
- Department of Hematology/Oncology, Staten Island University Hospital
| | - Amina Saqib
- Pulmonary/Critical Care, Staten Island University Hospital
| | - Nikhil Nalluri
- Department of Internal Medicine, Staten Island University Hospital
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Autoimmune Pancreatitis Masquerading as Pancreatic Cancer: when in Doubt, Cut It Out. J Gastrointest Cancer 2017; 49:365-372. [PMID: 28197777 DOI: 10.1007/s12029-017-9924-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
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Martin CS, Sirbu AE, Betivoiu MA, Florea S, Barbu CG, Fica SV. Serum immunoglobulin G4 levels and Graves' disease phenotype. Endocrine 2017; 55:478-484. [PMID: 27819113 DOI: 10.1007/s12020-016-1157-5] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/07/2016] [Accepted: 10/24/2016] [Indexed: 12/24/2022]
Abstract
PURPOSE We investigated, at diagnosis, the relationship between serum immunoglobulin G4 levels and the main characteristics of Graves' disease: hyperthyroidism severity, goiter size, presence of active Graves' ophthalmopathy, antithyroid antibodies status, and titer. METHODS This prospective study included 80 newly diagnosed Graves' disease patients. The main parameters measured at diagnosis: thyroid-stimulating hormone, free thyroxine, free triiodothyronine, total triiodothyronine, thyroglobulin, antithyroid peroxidase antibodies, anti-thyroglobulin antibodies, thyroid-stimulating hormone receptor antibodies, immunoglobulin G4. RESULTS In Graves' disease patients, serum immunoglobulin G4 levels were higher than in general population (p = 0.028) and higher in men compared to women (p = 0.002). Only one female patient with intense hypoechoic goiter, high anti-thyroglobulin antibody, and antithyroid peroxidase antibody titers had an elevated serum immunoglobulin G4 level at diagnosis. Patients with immunoglobulin G4 levels above the 75th percentile (>237.52 mg/dl, N = 20) were younger at Graves' ophthalmopathy onset (p < 0.001), had higher antithyroid peroxidase antibody (p = 0.01), and anti-thyroglobulin antibody levels (p = 0.006) and required shorter duration of the first methimazole treatment cycle (p = 0.041) than patients with immunoglobulin G4 below the 75th percentile. At diagnosis, patients with immunoglobulin G4 levels above the 90th percentile (>286.28 mg/dl, N = 8) had lower total triiodothyronine values (p = 0.001) than patients with IgG below the 90th percentile. No significant correlations were found between smoking status (p = 0.58), goiter size (p = 0.50), the presence of ophthalmopathy (p = 0.42) or thyroid-stimulating hormone receptor antibody titers (p = 0.45) and the mean value of immunoglobulin G4 levels at diagnosis. CONCLUSIONS Our data suggest that Graves' disease patients with elevated immunoglobulin G4 levels at diagnosis have a phenotype characterized by higher anti-thyroglobulin antibody and antithyroid peroxidase antibody titers, less severe T3 hyperthyroidism, younger age at ophthalmopathy onset and require a shorter duration of the first methimazole treatment cycle.
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Affiliation(s)
- Carmen Sorina Martin
- Endocrinology Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
- Endocrinology Department, Elias Hospital, Marasti Avenue 17, 011461, Bucharest, Romania.
| | - Anca Elena Sirbu
- Endocrinology Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
- Endocrinology Department, Elias Hospital, Marasti Avenue 17, 011461, Bucharest, Romania
| | | | - Suzana Florea
- Laboratory Department, Elias Hospital, Marasti Avenue 17, 011461, Bucharest, Romania
| | - Carmen Gabriela Barbu
- Endocrinology Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
- Endocrinology Department, Elias Hospital, Marasti Avenue 17, 011461, Bucharest, Romania
| | - Simona Vasilica Fica
- Endocrinology Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
- Endocrinology Department, Elias Hospital, Marasti Avenue 17, 011461, Bucharest, Romania
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Zhang XY, Li DX. Diagnosis and treatment of autoimmune pancreatitis: A case report. Shijie Huaren Xiaohua Zazhi 2017; 25:204-208. [DOI: 10.11569/wcjd.v25.i2.204] [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] [Indexed: 02/06/2023] Open
Abstract
Autoimmune pancreatitis (AIP) is a particular type of chronic pancreatic inflammatory disease which is caused by an autoimmune process, accounting for 2% to 6% of chronic pancreatitis cases. Because of its low incidence and similar clinical presentation and radiographic manifestations to chronic pancreatitis and pancreatic cancer, AIP is often misdiagnosed clinically. This paper reports a case of autoimmune pancreatitis treated at Weifang People's Hospital.
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Okazaki K, Chari ST, Frulloni L, Lerch MM, Kamisawa T, Kawa S, Kim MH, Lévy P, Masamune A, Webster G, Shimosegawa T. International consensus for the treatment of autoimmune pancreatitis. Pancreatology 2016; 17:1-6. [PMID: 28027896 DOI: 10.1016/j.pan.2016.12.003] [Citation(s) in RCA: 156] [Impact Index Per Article: 17.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/07/2016] [Revised: 11/24/2016] [Accepted: 12/11/2016] [Indexed: 12/11/2022]
Abstract
BACKGROUND AND AIMS The International Consensus Diagnostic Criteria (ICDC) for AIP has proposed two distinctive type of AIP, type 1 and type 2, and enabled us first to differentiate two types of AIP each other. By initial steroid treatment for induction of remission, remission can be successfully induced in almost all subjects with type 1 and type 2 AIP. As relapse rate in type 1 AIP is significantly higher than in type 2 AIP, there has been ongoing debate on how to treat effectively relapse of type 1 AIP. METHODS By a modified Delphi approach, a panel of international experts has proposed an international consensus on the treatment of AIP after intense discussion and deliberation during an international consensus symposium of the International Association of Pancreatology (IAP) 2016. RESULTS Individual statements for nine clinical questions with recommendation levels and the therapeutic strategy have been proposed. CONCLUSION The recommendations are based on the available evidence, and eastern and western experts' opinions to find standard treatment of AIP worldwide. These recommendations can be tailored according to the local expertise and context in the management of individual patients.
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Affiliation(s)
- Kazuichi Okazaki
- Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Japan.
| | - Suresh T Chari
- Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
| | - Luca Frulloni
- Department of Medicine, Pancreas Institute, University of Verona, Verona, Italy
| | - Markus M Lerch
- Department of Medicine A, University Medicine Greifswald, Germany
| | - Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
| | - Shigeyuki Kawa
- Center for Health, Safety, and Environmental Management, Shinshu University, Matsumoto, Japan
| | - Myung-Hwan Kim
- Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea
| | - Philippe Lévy
- Service de Pancréatologie-Gastroentérologie, Pôle des Maladies de l'Appareil Digestif, Université Denis Diderot-Paris VII Hôpital Beaujon, APHP, Clichy, France
| | - Atsushi Masamune
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - George Webster
- Pancreaticobiliary Medicine Unit, University College London Hospitals, London, UK
| | - Tooru Shimosegawa
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan
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Shi ZD. Recent advances in research of immunoglobulin G4-related autoimmune pancreatits. Shijie Huaren Xiaohua Zazhi 2016; 24:3946-3952. [DOI: 10.11569/wcjd.v24.i28.3946] [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] [Indexed: 02/06/2023] Open
Abstract
Autoimmune pancreatitis (AIP) is a rare type of chronic pancreatitis that belongs to the spectrum of immunoglobulin G4 (IgG4) related diseases. AIP is characterized by lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, and increased IgG4+ plasma cells. Patients with AIP present clinically with recurrent pancreatitis and obstructive jaundice, symptoms involving bile ducts and salivary glands and so on. Serum IgG4 level is often elevated. The main imaging manifestation of AIP is a "sausage-shaped pancreas" and multiple strictures of the main pancreatic duct. AIP responds sensitively to glucocorticosteroids but relapses easily. In relapsed cases, re-administration of glucocorticosteroids alone or in combination with immune modulators is effective.
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49
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Woo YJ, Kim JW, Yoon JS. Clinical implications of serum IgG 4 levels in patients with IgG 4-related ophthalmic disease. Br J Ophthalmol 2016; 101:256-260. [PMID: 27215743 DOI: 10.1136/bjophthalmol-2016-308592] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2016] [Revised: 04/04/2016] [Accepted: 05/01/2016] [Indexed: 12/24/2022]
Abstract
AIMS The present study aimed to investigate the clinical implications of serum IgG4 levels in patients with IgG4-related ophthalmic disease (ROD). METHODS The medical records of 31 patients who met the diagnostic criteria for IgG4-ROD were retrospectively reviewed. Twenty-five patients whose serum IgG4 levels could be identified were included. Clinical manifestations and serum IgG4 levels before and after corticosteroid treatment were obtained. Factors associated with relapse were evaluated by comparing the features of patients with disease relapse with those of patients without relapse. RESULTS Twenty-four patients were 'definite' and one was 'probable' for IgG4-ROD according to the diagnostic criteria. Serum IgG4 levels were higher in patients with systemic involvement (p=0.046). All patients improved clinically after corticosteroid treatment. Serum IgG4 levels decreased after steroid treatment (p=0.005) and normalised in nine patients. In cases of relapse, serum IgG4 levels increased along with the aggravation of symptoms (p=0.047). Serum IgG4 levels that were still elevated (≥135 mg/dL) after steroid treatment (p=0.034) and cessation of steroid treatment during disease remission (p=0.043) were predictive factors for IgG4-ROD relapse. CONCLUSIONS Serum IgG4 level can be considered an adjunctive marker for treatment response in IgG4-ROD. Patients with serum IgG4 levels that remain elevated after steroid treatment should be carefully observed for relapse. A continuing maintenance dose of oral steroid is recommended to prevent relapse, even when clinical remission is achieved.
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Affiliation(s)
- Young Jun Woo
- Department of Ophthalmology, Institute of Vision Research, Yonsei University College of Medicine, Seoul, South Korea
| | - Ji Won Kim
- Department of Ophthalmology, Institute of Vision Research, Yonsei University College of Medicine, Seoul, South Korea
| | - Jin Sook Yoon
- Department of Ophthalmology, Institute of Vision Research, Yonsei University College of Medicine, Seoul, South Korea
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López-Serrano A, Crespo J, Pascual I, Salord S, Bolado F, Del-Pozo-García AJ, Ilzarbe L, de-Madaria E, Moreno-Osset E. Diagnosis, treatment and long-term outcomes of autoimmune pancreatitis in Spain based on the International Consensus Diagnostic Criteria: A multi-centre study. Pancreatology 2016; 16:382-90. [PMID: 26944001 DOI: 10.1016/j.pan.2016.02.006] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/22/2015] [Revised: 12/18/2015] [Accepted: 02/12/2016] [Indexed: 12/11/2022]
Abstract
OBJECTIVES Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that has been reported worldwide for the last two decades. The aim of this study is to analyse the clinical profile of patients from Spain with AIP, as well as treatments, relapses and long-term outcomes. METHODS Data from 59 patients with suspected AIP that had been diagnosed in 15 institutions are retrospectively analysed. Subjects are classified according to the International Consensus Diagnostic Criteria (ICDC). Patients with type 1 AIP (AIP1) and type 2 AIP (AIP2) are compared. Kaplan-Meier methodology is used to estimate the overall survival without relapses. RESULTS Fifty-two patients met ICDC, 45 patients were AIP1 (86.5%). Common manifestations included abdominal pain (65.4%) and obstructive jaundice (51.9%). Diffuse enlargement of pancreas was present in 51.0%; other organ involvement was present in 61.5%. Serum IgG4 increased in 76.7% of AIP1 patients vs. 20.0% in AIP2 (p = 0.028). Tissue specimens were obtained in 76.9%. Initial successful treatment with steroids or surgery was achieved in 79.8% and 17.3%, respectively. Maintenance treatment was given in 59.6%. Relapses were present in 40.4% of AIP1, with a median of 483 days. Successful long-term remission was achieved in 86.4%. CONCLUSIONS AIP1 is the most frequent form of AIP in Spain in our dataset. Regularly, ICDC allows AIP diagnosis without the need for surgery. Steroid and chirurgic treatments were effective and safe in most patients with AIP, although maintenance was required many times because of their tendency to relapse. Long-term serious consequences were uncommon.
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Affiliation(s)
- Antonio López-Serrano
- Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain.
| | - Javier Crespo
- Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain
| | - Isabel Pascual
- Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain
| | - Silvia Salord
- Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain
| | - Federico Bolado
- Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain
| | - Andrés J Del-Pozo-García
- Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain
| | - Lucas Ilzarbe
- Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain
| | - Enrique de-Madaria
- Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain
| | - Eduardo Moreno-Osset
- Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain
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