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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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Yoon SB, Jeon TY, Moon SH, Shin DW, Lee SM, Choi MH, Min JH, Kim MJ. Differentiation of autoimmune pancreatitis from pancreatic adenocarcinoma using CT characteristics: a systematic review and meta-analysis. Eur Radiol 2023; 33:9010-9021. [PMID: 37466708 DOI: 10.1007/s00330-023-09959-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2022] [Revised: 05/15/2023] [Accepted: 05/23/2023] [Indexed: 07/20/2023]
Abstract
OBJECTIVES To determine informational CT findings for distinguishing autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma (PDAC) and to review their diagnostic accuracy. METHODS A systematic and detailed literature review was performed through PubMed, EMBASE, and the Cochrane library. Similar descriptors to embody the identical image finding were labeled as a single CT characteristic. We calculated the pooled diagnostic odds ratios (DORs) of each CT characteristic using a bivariate random-effects model. RESULTS A total of 145 various descriptors from 15 studies (including 562 AIP and 869 PDAC patients) were categorized into 16 CT characteristics. According to the pooled DOR, 16 CT characteristics were classified into three groups (suggesting AIP, suggesting PDAC, and not informational). Seven characteristics suggesting AIP were diffuse pancreatic enlargement (DOR, 48), delayed homogeneous enhancement (DOR, 46), capsule-like rim (DOR, 34), multiple pancreatic masses (DOR, 16), renal involvement (DOR, 15), retroperitoneal fibrosis (DOR, 13), and bile duct involvement (DOR, 8). Delayed homogeneous enhancement showed a pooled sensitivity of 83% and specificity of 85%. The other six characteristics showed relatively low sensitivity (12-63%) but high specificity (93-99%). Four characteristics suggesting PDAC were discrete pancreatic mass (DOR, 23), pancreatic duct cutoff (DOR, 16), upstream main pancreatic duct dilatation (DOR, 8), and upstream parenchymal atrophy (DOR, 7). CONCLUSION Eleven CT characteristics were informational to distinguish AIP from PDAC. Diffuse pancreatic enlargement, delayed homogeneous enhancement, and capsule-like rim suggested AIP with the highest DORs, whereas discrete pancreatic mass suggested PDAC. However, pooled sensitivities of informational CT characteristics were moderate. CLINICAL RELEVANCE STATEMENT This meta-analysis underscores eleven distinctive CT characteristics that aid in differentiating autoimmune pancreatitis from pancreatic adenocarcinoma, potentially preventing misdiagnoses in patients presenting with focal/diffuse pancreatic enlargement. KEY POINTS • Diffuse pancreatic enlargement (pooled diagnostic odds ratio [DOR], 48), delayed homogeneous enhancement (46), and capsule-like rim (34) were CT characteristics suggesting autoimmune pancreatitis. • The CT characteristics suggesting autoimmune pancreatitis, except delayed homogeneous enhancement, had a general tendency to show relatively low sensitivity (12-63%) but high specificity (93-99%). • Discrete pancreatic mass (pooled diagnostic odds ratio, 23) was the CT characteristic suggesting pancreatic ductal adenocarcinoma with the highest pooled DORs.
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Affiliation(s)
- Seung Bae Yoon
- Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, South Korea
| | - Tae Yeon Jeon
- Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea
| | - Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, 22 Gwanpyeong-ro 170 beon-gil, Dongan-gu, Anyang, Gyeonggi-do, 14068, South Korea.
| | - Dong Woo Shin
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, 22 Gwanpyeong-ro 170 beon-gil, Dongan-gu, Anyang, Gyeonggi-do, 14068, South Korea
| | - Sang Min Lee
- Department of Radiology, Cha Gangnam Medical Center, Seoul, South Korea
| | - Moon Hyung Choi
- Department of Radiology, College of Medicine, The Catholic University of Korea, Seoul, South Korea
| | - Ji Hye Min
- Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea
| | - Min-Jeong Kim
- Department of Radiology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea
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Liu B, Tang N, Yao Y, Li H, Xu L, Zhou B, Liu B. Steroid treatment response combined with serological mark in differentiating type-1 autoimmune pancreatitis from pancreatic cancer. Medicine (Baltimore) 2022; 101:e31660. [PMID: 36397434 PMCID: PMC9666219 DOI: 10.1097/md.0000000000031660] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/14/2022] [Accepted: 10/13/2022] [Indexed: 11/19/2022] Open
Abstract
Autoimmune pancreatitis (AIP) and pancreatic cancer (PC) are two different diseases. Their diagnosis, treatment, and prognosis are different, and it is difficult to differentiate them. This study aimed to explore the role of steroid treatment response combined with serological mark in distinguishing type-1 AIP from PC. Clinical data were collected and compared from 50 cases of AIP (group 1) and 100 cases of PC (group 2). The diagnostic value of serum IgG4, CA19-9, globulin, and eosinophil cell (EC) were evaluated. The response of steroid treatment of 28 patients with atypical imaging in group 1 was analyzed. After 2 weeks, the patients were classified as positive and negative steroid response according to the manifestations and/or the radiological changes. The positive response cases (n = 20) were confirmed as AIP, whereas negative ones (n = 8) were finally diagnosed as PC after complete resection. Serum globulin, IgG4, and EC levels in group 1 were significantly higher than those in group 2 (P < .01), and CA19-9 levels were distinctly lower in group 1 (P < .01). The level of serum IgG4 was related to the accuracy of diagnosis of AIP on the basis of the result of logistic regression analysis. Two-weeks steroid therapy response combined with serum IgG4 levels contribute to the differential diagnosis AIP and PC. However, regular and long-term follow-up were important for the differential diagnosis. There was an urgent need to explore the specific markers that distinguish these 2 entities.
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Affiliation(s)
- Bingqian Liu
- Department of Rheumatology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
| | - Ning Tang
- Department of Nutrition, Weifang People’s Hospital, Shandong Province, China
| | - Yuan Yao
- Department of Rheumatology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
| | - Hua Li
- Department of Rheumatology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
| | - Lishan Xu
- Department of Rheumatology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
| | - Bin Zhou
- Department of Biliary and Pancreatic Surgery, Department of Retroperitoneal Tumor Surgery, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
| | - Bin Liu
- Department of Rheumatology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
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Moon SH, Kim MH. Autoimmune Pancreatitis and Immunoglobulin G4-related Sclerosing Cholangitis: Past, Present, and Future. THE KOREAN JOURNAL OF GASTROENTEROLOGY = TAEHAN SOHWAGI HAKHOE CHI 2022; 80:107-114. [PMID: 36156034 DOI: 10.4166/kjg.2022.102] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/20/2022] [Revised: 08/15/2022] [Accepted: 08/16/2022] [Indexed: 06/16/2023]
Abstract
The emergence of glucocorticoid-responsive autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC), a new disease entity, has attracted considerable interest within the international gastroenterology community. The typical manifestations of AIP/IgG4-SC are obstructive jaundice and pancreatic enlargement in the elderly, which may mimic the presentations of pancreatobiliary malignancies. The timely diagnosis of AIP/IgG4-SC can lead to adequate glucocorticoid treatment, whereas a misdiagnosis can result in unnecessary major surgery. The diagnostic criteria used to diagnose AIP include several cardinal features of AIP that can be detected via pancreatic parenchymal imaging, ductal imaging, serum IgG4 levels, histopathology, other organ involvement, and response to glucocorticoid therapy. The differential diagnosis of AIP/IgG4-SC may include pancreatobiliary malignancies and primary sclerosing cholangitis. Although most patients with AIP/IgG4-SC respond well to glucocorticoid therapy, there is a frequent relapse of the disease in the long term. This review describes the evolution of the concept of AIP and IgG4-related disease, including the development of diagnostic criteria, discusses the current practice for diagnosis and treatment, and suggests prospects for research.
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Affiliation(s)
- Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Anyang, Korea
| | - Myung-Hwan Kim
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
- Department of Internal Medicine, Hanyang University Changwon Hanmaeum Hospital, Changwon, Korea
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Takagi T, Sugimoto M, Suzuki R, Konno N, Asama H, Sato Y, Irie H, Nakamura J, Takasumi M, Hashimoto M, Kato T, Kobashi R, Yanagita T, Hashimoto Y, Marubashi S, Hikichi T, Ohira H. Screening for hilar biliary invasion in ampullary cancer patients. World J Gastrointest Endosc 2022; 14:536-546. [PMID: 36186943 PMCID: PMC9516475 DOI: 10.4253/wjge.v14.i9.536] [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: 01/05/2022] [Revised: 06/28/2022] [Accepted: 08/07/2022] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND The treatment for ampullary cancer is pancreatoduodenectomy or local ampullectomy. However, effective methods for the preoperative investigation of hilar biliary invasion in ampullary cancer patients have not yet been identified.
AIM To determine the necessity of and an appropriate method for investigating hilar biliary invasion of ampullary cancer.
METHODS Among 43 ampullary cancer patients, 34 underwent endoscopic treatment (n = 9) or surgery (n = 25). The use of imaging findings (thickening and enhancement of the bile duct wall on contrast-enhanced computed tomography, irregularity on endoscopic retrograde cholangiography, thickening of the entire bile duct wall on intraductal ultrasonography (IDUS), and partial thickening of the bile duct wall on IDUS) and biliary biopsy results for diagnosing hilar biliary invasion of ampullary cancer was compared.
RESULTS Hilar invasion was not observed in every patient. Among the patients who did not undergo biliary stent insertion, the combination of partial thickening of the bile duct wall on IDUS and biliary biopsy results showed the highest accuracy (100%) for diagnosing hilar biliary invasion. However, each imaging method and biliary biopsy yielded some false-positive results.
CONCLUSION Although some false-positive results were obtained with each method, the combination of partial thickening of the bile duct wall on IDUS and biliary biopsy results was useful for diagnosing hilar biliary invasion of ampullary cancer. However, hilar invasion of ampullary cancer is rare; therefore, the investigation of hilar biliary invasion of ampullary cancer might be unnecessary.
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Affiliation(s)
- Tadayuki Takagi
- Department of Gastroenterology, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Mitsuru Sugimoto
- Department of Gastroenterology, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Rei Suzuki
- Department of Gastroenterology, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Naoki Konno
- Department of Gastroenterology, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Hiroyuki Asama
- Department of Gastroenterology, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Yuki Sato
- Department of Gastroenterology, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Hiroki Irie
- Department of Gastroenterology, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Jun Nakamura
- Department of Endoscopy, Fukushima Medical University Hospital, Fukushima 960-1295, Japan
| | - Mika Takasumi
- Department of Gastroenterology, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Minami Hashimoto
- Department of Endoscopy, Fukushima Medical University Hospital, Fukushima 960-1295, Japan
| | - Tsunetaka Kato
- Department of Endoscopy, Fukushima Medical University Hospital, Fukushima 960-1295, Japan
| | - Ryoichiro Kobashi
- Department of Endoscopy, Fukushima Medical University Hospital, Fukushima 960-1295, Japan
| | - Takumi Yanagita
- Department of Gastroenterology, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Yuko Hashimoto
- Department of Pathological Diagnosis, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Shigeru Marubashi
- Department of Hepato-Biliary-Pancreatic and Transplant Surgery, Fukushima Medical University, Fukushima 960-1295, Japan
| | - Takuto Hikichi
- Department of Endoscopy, Fukushima Medical University Hospital, Fukushima 960-1295, Japan
| | - Hiromasa Ohira
- Department of Gastroenterology, Fukushima Medical University, Fukushima 960-1295, Japan
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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: 8] [Impact Index Per Article: 2.0] [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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Jearth V, Giri S, Sundaram S. Approach to management of pancreatic strictures: the gastroenterologist's perspective. Clin J Gastroenterol 2021; 14:1587-1597. [PMID: 34405382 DOI: 10.1007/s12328-021-01503-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/14/2021] [Accepted: 08/13/2021] [Indexed: 12/15/2022]
Abstract
Pancreatic strictures represent a complex clinical problem which often requires multidisciplinary management with a team of gastroenterologists, surgeons and radiologists. Dominant strictures are largely due to inflammatory processes of the pancreas like chronic pancreatitis. However, differentiating benign from malignant processes of the pancreas, leading to strictures is imperative and remains a challenge. With advances in endoscopic management, options for therapy include endoscopic retrograde cholangiopancreatography (ERCP), and endoscopic ultrasound-guided pancreatic drainage (EUS-PD) in situations where ERCP is not feasible or fails. However, endoscopic therapy is suited for a select group of patients and surgery remains key to management in many patients. In this narrative review, we look at the gastroenterologist's perspective and approach to pancreatic ductal strictures, including endoscopic and surgical management.
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Affiliation(s)
- Vaneet Jearth
- Department of Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Suprabhat Giri
- Department of Gastroenterology, Seth GS Medical College and King Edward Memorial Hospital, Mumbai, India
| | - Sridhar Sundaram
- Department of Digestive Diseases and Clinical Nutrition, Tata Memorial Hospital, Homi Bhabha National Institute, Dr. E Borges Road, Parel, Mumbai, 400012, India.
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Yoon SB, Moon SH, Song TJ, Kim JH, Kim MH. Endoscopic ultrasound-guided fine needle aspiration versus biopsy for diagnosis of autoimmune pancreatitis: Systematic review and comparative meta-analysis. Dig Endosc 2021; 33:1024-1033. [PMID: 33030283 DOI: 10.1111/den.13866] [Citation(s) in RCA: 26] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/22/2020] [Accepted: 10/01/2020] [Indexed: 12/17/2022]
Abstract
BACKGROUND Endoscopic ultrasound (EUS) is recommended for guiding the acquisition of pancreatic tissue in patients with suspected autoimmune pancreatitis (AIP). Data comparing EUS-guided fine needle aspiration (FNA) and fine needle biopsy (FNB) sampling in the diagnosis of AIP are limited. METHODS A comprehensive literature search of the PubMed, EMBASE, and Ovid MEDLINE databases was conducted until April 2020. The pooled rates of diagnostic yield for the histologic criteria of AIP, histologic tissue procurement, and adverse events were compared between FNA and FNB. Diagnostic yields were also compared between 19 gauge (G) and 22G needles. RESULTS This meta-analysis included nine studies comprising 309 patients with AIP who underwent FNA and seven studies comprising 131 patients who underwent FNB. The pooled diagnostic yields for level 1 or 2 histology criteria of AIP were 55.8% (95% confidence interval (CI) 37.0-73.9%, I2 = 91.1) for FNA and 87.2% (95% CI 68.8-98.1%, I2 = 69.4) for FNB (P = 0.030). The pooled histologic procurement rates for FNA and FNB were 91.3% (95% CI, 84.9-97.6%, I2 = 82.9) and 87.0% (95% CI, 77.8-96.1%, I2 = 40.0), respectively (P = 0.501). Adverse events were comparable between two groups. When analyzed by needle size, the diagnostic yield was better with a 19G needle than with a 22G needle (88.9% vs. 60.6%, P = 0.023). CONCLUSIONS The diagnostic yield may be better with FNB needles than with FNA needles for the diagnosis of AIP, despite the similar rate of histologic tissue procurement. A quantitative definition for the histologic sample adequacy for AIP may be warranted.
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Affiliation(s)
- Seung Bae Yoon
- Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, South Korea
| | - Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea.,Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea
| | - Tae Jun Song
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
| | - Jong Hyeok Kim
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea.,Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea
| | - Myung-Hwan Kim
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
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Diagnostic Performance of EUS-Guided Sampling in Indeterminate Radiological Diagnosis of Pancreatic Disease and Intra-Abdominal Lymphadenopathy. J Clin Med 2021; 10:jcm10173850. [PMID: 34501294 PMCID: PMC8432008 DOI: 10.3390/jcm10173850] [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] [Received: 08/06/2021] [Revised: 08/19/2021] [Accepted: 08/26/2021] [Indexed: 11/17/2022] Open
Abstract
BACKGROUND Endoscopic ultrasound (EUS)-guided sampling has been widely used for pathologic diagnosis of pancreatic lesions and intra-abdominal lymphadenopathy. However, its effectiveness for diagnostic decision making in indeterminate radiological diagnosis has not been well determined. MATERIALS AND METHODS From March 2012 to October 2015, 98 consecutive patients who underwent EUS-guided FNA for solid intra-abdominal lesions were retrospectively analyzed (100 procedures). The purpose of EUS-guided sampling was classified as (1) confirmation of a high-confidence radiological diagnosis (High-confidence group) or (2) decision making in the differential diagnostic dilemma for indeterminate radiological diagnosis (Indeterminate group). The accuracies of EUS-guided sampling according to the purpose were analyzed and then compared. RESULTS Of the 100 procedures, 22 procedures (22%) came under the Indeterminate group, whereas 78 came under the High-confidence group. The accuracies did not differ between the Indeterminate and the High-confidence groups (86.4% vs. 88.5%, p = 1.000). Clinical conditions that required EUS-guided sampling for indeterminate radiological diagnosis were (1) pancreatic cancer vs. benign disease (n = 8; e.g., pancreatic cancer vs. mass-forming pancreatitis), (2) recurrence of previous/pre-existing cancer vs. benign disease (n = 5; e.g., recurrent gastric cancer vs. reactive lymph node), (3) pathologic differentiation of presumed malignancy (n = 6; e.g., lymphadenopathies in the previous history of esophageal cancer and colon cancer), or (4) miscellaneous (n = 3; e.g., tuberculous lymphadenopathy vs. other condition). CONCLUSIONS EUS-guided sampling demonstrated an accuracy of 86.4% in the clinical setting of indeterminate radiological diagnosis, which was not different from that of the confirmation of high-confidence diagnosis.
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Naitoh I, Nakazawa T. Classification and Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis. Gut Liver 2021; 16:28-36. [PMID: 34380781 PMCID: PMC8761932 DOI: 10.5009/gnl210116] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/12/2021] [Revised: 05/06/2021] [Accepted: 06/14/2021] [Indexed: 11/20/2022] Open
Abstract
IgG4-related sclerosing cholangitis (IgG4-SC) can be classified into four types based on cholangiographic findings and regions of biliary stricture. This cholangiographic classification is useful to differentiate IgG4-SC from mimickers including cholangiocarcinoma, primary sclerosing cholangitis, and pancreatic cancer. Autoimmune pancreatitis (AIP) is a valuable clue for the diagnosis of IgG4-SC because the two are frequently found in association with each other. Two sets of diagnostic criteria for IgG4-SC have been proposed. In Japan, the clinical diagnostic criteria 2020 were recently developed. These clinical diagnostic criteria include narrowing of the intrahepatic and/or extrahepatic bile duct, thickening of the bile duct wall, serological findings, pathological findings, other organ involvement, and effectiveness of steroid therapy. When these criteria are applied, IgG4-SC is initially classified as associated or not associated with AIP, and cholangiographic classification is used for differential diagnosis. In most instances, IgG4-SC can be diagnosed on the basis of clinical diagnostic criteria. However, it is challenging to diagnose isolated IgG4-SC or IgG4-SC not associated with AIP. Here, we review the classification and diagnostic criteria for IgG4-SC, specifically focusing on the clinical diagnostic criteria 2020 and a large IgG4-SC case series from a nationwide survey in Japan.
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Affiliation(s)
- Itaru Naitoh
- Department of Gastroenterology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Takahiro Nakazawa
- Department of Gastroenterology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
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Endoscopic retrograde cholangiopancreatography and intraductal ultrasonography in the diagnosis of autoimmune pancreatitis and IgG4-related sclerosing cholangitis. J Med Ultrason (2001) 2021; 48:573-580. [PMID: 34331625 DOI: 10.1007/s10396-021-01114-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2021] [Accepted: 06/10/2021] [Indexed: 12/17/2022]
Abstract
Endoscopic retrograde cholangiopancreatography is used to evaluate the narrowing of the main pancreatic duct in autoimmune pancreatitis (AIP) and biliary stricture in IgG4-related sclerosing cholangitis (IgG4-SC). Intraductal ultrasonography enables detailed visualization of the thickening of the bile duct wall in IgG4-SC. Pancreatic cancer, cholangiocarcinoma, and primary sclerosing cholangitis are important mimicking conditions of AIP and IgG4-SC. Diffuse or segmental stricture without marked upstream dilatation is a typical pancreatographic finding in AIP. By contrast, a single, short stricture with marked upstream dilatation is a typical finding in pancreatic cancer. The cholangiogram of IgG4-SC is classified into four types based on biliary stricture location, and this cholangiogram classification is useful for the differential diagnosis of IgG4-SC. Endoscopic retrograde cholangiography can be used to distinguish between IgG4-SC and primary sclerosing cholangitis. A segmental/long and intrapancreatic stricture is a characteristic finding of IgG4-SC, whereas band-like strictures, a beaded or pruned-tree appearance, and diverticulum-like outpouching are characteristic of primary sclerosing cholangitis. The characteristic intraductal ultrasonographic findings of circular-symmetrical wall thickening, smooth outer and inner margins, and homogeneous internal echo at the biliary stricture site are useful for diagnosis of IgG4-SC. Thickening of the bile duct wall at non-stricture sites is also a typical intraductal ultrasonographic finding of IgG4-SC and can be used for differential diagnosis from cholangiocarcinoma. Transpapillary bile duct and duodenal papilla biopsy during endoscopic retrograde cholangiopancreatography are also useful in the diagnosis of IgG4-SC.
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12
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Jung YJ, Moon SH, Kim MH. Role of Endoscopic Procedures in the Diagnosis of IgG4-Related Pancreatobiliary Disease. Chonnam Med J 2021; 57:44-50. [PMID: 33537218 PMCID: PMC7840337 DOI: 10.4068/cmj.2021.57.1.44] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2020] [Revised: 12/20/2020] [Accepted: 12/21/2020] [Indexed: 12/16/2022] Open
Abstract
The emergence of the disease entity of glucocorticoid-responsive systemic immunoglobulin G4 (IgG4)-related pancreatobiliary disease has generated substantial attention among the international gastroenterology society. IgG4-related pancreatobiliary disease includes type 1 autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC). The typical manifestations of IgG4-related pancreatobiliary disease are cholestatic liver dysfunction, obstructive jaundice, and weight loss, although it may present with no clinical symptoms. Since it mimics tumors on imaging, AIP/IgG4-SC may often be misdiagnosed as pancreatic or biliary cancer. The endoscopic armamentarium for the diagnosis of IgG4-related pancreatobiliary disease includes endoscopic ultrasonography, intraductal ultrasonography, endoscopic retrograde cholangiopancreatography, and cholangioscopy. The role of endoscopic tissue acquisition is two-fold in the diagnosis of IgG4-related pancreatobiliary disease: exclusion of cancer and procurement of histopathological proof for diagnosis of AIP/IgG4-SC, which can also be achieved by adding the immunohistochemistry for IgG4. Our review article addresses the role of various endoscopic examinations in diagnosing IgG4-related pancreatobiliary disease, focusing on the differentiation of this condition from pancreatobiliary malingnancies.
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Affiliation(s)
- Ye-Ji Jung
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea
| | - Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea.,Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, Korea
| | - Myung-Hwan Kim
- Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Biliary inflammation scoring for immunoglobulin G4-related sclerosing cholangitis: an endoscopic approach with endoscopic ultrasound. Surg Endosc 2021; 35:7068-7073. [PMID: 33492512 DOI: 10.1007/s00464-020-08222-y] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2020] [Accepted: 12/03/2020] [Indexed: 12/21/2022]
Abstract
BACKGROUND AND AIMS The differential diagnosis of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) and cholangiocarcinoma (CC) remains a clinical challenge. Imaging modalities play critical roles in the diagnosis of IgG4-SC. The present study aimed to evaluate the differential diagnosis of IgG4-SC and CC based on images of endoscopic ultrasound (EUS). METHODS The biliary inflammation scoring (BIS) method for EUS was developed based on the comparison between images of IgG4-SC and that of cholangiocarcinoma (CC) and other acute or chronic cholangitis. In the BIS diagnostic phase, the EUS images from 66 IgG4-SC patients and 44 CC patients were blindly evaluated using the BIS methods. RESULTS The sensitivity, specificity, and accuracy of the newly established BIS in distinguishing IgG4-SC from CC were 86% [95% confidence interval (CI) 75-93%], 95% (95% CI 83-99%), and 90% (95% CI 83-94%), respectively. CONCLUSION EUS should be considered to be added to the workup algorithm in patients with suspected IgG4-SC as a useful diagnostic procedure. BIS is a promising diagnostic method to discriminate IgG4-SC during the ongoing endoscopy.
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14
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Yoon SB, Moon SH, Kim JH, Song TJ, Kim MH. The use of immunohistochemistry for IgG4 in the diagnosis of autoimmune pancreatitis: A systematic review and meta-analysis. Pancreatology 2020; 20:1611-1619. [PMID: 33060017 DOI: 10.1016/j.pan.2020.10.028] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/22/2020] [Revised: 08/01/2020] [Accepted: 10/08/2020] [Indexed: 02/07/2023]
Abstract
BACKGROUND The diagnosis of autoimmune pancreatitis (AIP) remains challenging, especially when serum IgG4 is normal or imaging features are indeterminate. We performed a systematic review and meta-analysis to evaluate the performance of IgG4 immunostaining of pancreatic, biliary, and ampullary tissues as a diagnostic aid for AIP. METHODS A comprehensive literature search of the PubMed, EMBASE, and Ovid MEDLINE databases was conducted until February 2020. The methodological quality of each study was assessed according to the Quality Assessment of Diagnostic Accuracy Studies checklist. A random-effects model was used to summarize the diagnostic odds ratio and other measures of accuracy. RESULTS The meta-analysis included 20 studies comprising 346 patients with AIP and 590 patients with other pancreatobiliary diseases, including 371 pancreatobiliary malignancies. The summary estimates for tissue IgG4 in discriminating AIP and controls were as follows: diagnostic odds ratio 38.86 (95% confidence interval (CI), 18.70-80.75); sensitivity 0.64 (95% CI, 0.59-0.69); specificity 0.93 (95% CI, 0.91-0.95). The area under the curve was 0.939 for tissue IgG4 in discriminating AIP and controls. Subgroup analysis revealed no significant difference in diagnostic accuracy according to control groups (pancreatobiliary cancer versus other chronic pancreatitis) and sampling site (pancreas versus bile duct/ampulla). CONCLUSIONS Current data demonstrate that IgG4 immunostaining of pancreatic, biliary, and ampullary tissue has a high specificity but moderate sensitivity for diagnosing AIP. IgG4 immunostaining may be useful in supporting a diagnosis of AIP when AIP is clinically suspected, but a combination of imaging and serology does not provide a conclusive diagnosis.
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Affiliation(s)
- Seung Bae Yoon
- Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, South Korea
| | - Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea; Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea.
| | - Jong Hyeok Kim
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea; Institute for Liver and Digestive Diseases, Hallym University, Chuncheon, South Korea
| | - Tae Jun Song
- Department of Internal Medicine, University of Ulsan, College of Medicine, Asan Medical Center, Seoul, South Korea
| | - Myung-Hwan Kim
- Department of Internal Medicine, University of Ulsan, College of Medicine, Asan Medical Center, Seoul, South Korea
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15
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Kanno A, Ikeda E, Ando K, Nagai H, Miwata T, Kawasaki Y, Tada Y, Yokoyama K, Numao N, Ushio J, Tamada K, Lefor AK, Yamamoto H. The Diagnosis of Autoimmune Pancreatitis Using Endoscopic Ultrasonography. Diagnostics (Basel) 2020; 10:diagnostics10121005. [PMID: 33255660 PMCID: PMC7760882 DOI: 10.3390/diagnostics10121005] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2020] [Revised: 11/18/2020] [Accepted: 11/20/2020] [Indexed: 12/14/2022] Open
Abstract
Autoimmune pancreatitis (AIP) is characterized by enlargement of the pancreas and irregular narrowing of the main pancreatic duct. It is often associated with IgG4-related sclerosing cholangitis (IgG4-SC), in which the bile duct narrows. Although characteristic irregular narrowing of the pancreatic duct caused by endoscopic retrograde cholangiopancreatography is noted in AIP, it is difficult to differentiate between localized AIP and pancreatic carcinoma based on imaging of the pancreatic duct. While stenosis of the bile duct in IgG4-SC is characterized by longer-length stenosis than in cholangiocarcinoma, differentiation based on bile duct imaging alone is challenging. Endoscopic ultrasound (EUS) can characterize hypoechoic enlargement of the pancreas or bile duct wall thickening in AIP and IgG4-SC, and diagnosis using elastography and contrast-enhanced EUS are being evaluated. The utility of EUS-guided fine needle aspiration for the histological diagnosis of AIP has been reported and is expected to improve diagnostic performance for AIP. Findings in the bile duct wall from endoscopic retrograde cholangiopancreatography followed by intraductal ultrasonography are useful in differentiating IgG4-SC from cholangiocarcinoma. Diagnoses based on endoscopic ultrasonography play a central role in the diagnosis of AIP.
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Affiliation(s)
- Atsushi Kanno
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
- Correspondence: ; Tel.: +81-285-58-7348; Fax: 81-285-44-8297
| | - Eriko Ikeda
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Kozue Ando
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Hiroki Nagai
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Tetsuro Miwata
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Yuki Kawasaki
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Yamato Tada
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Kensuke Yokoyama
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Norikatsu Numao
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Jun Ushio
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Kiichi Tamada
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
| | - Alan Kawarai Lefor
- Department of Surgery, Jichi Medical University, Shimotsuke 329-0498, Japan;
| | - Hironori Yamamoto
- Department of Medicine, Division of Gastroenterology, Jichi Medical University, Shimotsuke 329-0498, Japan; (E.I.); (K.A.); (H.N.); (T.M.); (Y.K.); (Y.T.); (K.Y.); (N.N.); (J.U.); (K.T.); (H.Y.)
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16
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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 EF, 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 DOI: 10.1177/2050640620934911] [Citation(s) in RCA: 128] [Impact Index Per Article: 25.6] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [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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- See list at the end of this article
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17
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Chapman MH, Thorburn D, Hirschfield GM, Webster GGJ, Rushbrook SM, Alexander G, Collier J, Dyson JK, Jones DE, Patanwala I, Thain C, Walmsley M, Pereira SP. British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. Gut 2019; 68:1356-1378. [PMID: 31154395 PMCID: PMC6691863 DOI: 10.1136/gutjnl-2018-317993] [Citation(s) in RCA: 172] [Impact Index Per Article: 28.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2018] [Revised: 02/21/2019] [Accepted: 03/24/2019] [Indexed: 12/11/2022]
Abstract
These guidelines on the management of primary sclerosing cholangitis (PSC) were commissioned by the British Society of Gastroenterology liver section. The guideline writing committee included medical representatives from hepatology and gastroenterology groups as well as patient representatives from PSC Support. The guidelines aim to support general physicians, gastroenterologists and surgeons in managing adults with PSC or those presenting with similar cholangiopathies which may mimic PSC, such as IgG4 sclerosing cholangitis. It also acts as a reference for patients with PSC to help them understand their own management. Quality of evidence is presented using the AGREE II format. Guidance is meant to be used as a reference rather than for rigid protocol-based care as we understand that management of patients often requires individual patient-centred considerations.
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Affiliation(s)
- Michael Huw Chapman
- GI Division, UCL Hospitals NHS Foundation Trust, London, UK
- Liver Unit, Royal Free London NHS Foundation Trust, London, UK
| | | | - Gideon M Hirschfield
- Toronto Centre for Liver Disease, University Health Network and University of Toronto, Toronto, Canada
| | | | - Simon M Rushbrook
- Department of Hepatology, Norfolk and Norwich University Hospitals NHS Trust, Norwich, UK
| | | | | | - Jessica K Dyson
- Hepatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle, UK
- Institute of Cellular Medicine, Newcastle University, Newcastle, UK
| | - David Ej Jones
- Institute of Cellular Medicine, Newcastle University, Newcastle, UK
| | - Imran Patanwala
- Gastroenterology, Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, UK
- University of Liverpool, Liverpool, UK
| | | | | | - Stephen P Pereira
- GI Division, UCL Hospitals NHS Foundation Trust, London, UK
- Institute for Liver & Digestive Health, University College London, London, UK
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18
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Kamisawa T, Nakazawa T, Tazuma S, Zen Y, Tanaka A, Ohara H, Muraki T, Inui K, Inoue D, Nishino T, Naitoh I, Itoi T, Notohara K, Kanno A, Kubota K, Hirano K, Isayama H, Shimizu K, Tsuyuguchi T, Shimosegawa T, Kawa S, Chiba T, Okazaki K, Takikawa H, Kimura W, Unno M, Yoshida M. Clinical practice guidelines for IgG4-related sclerosing cholangitis. JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES 2019; 26:9-42. [PMID: 30575336 PMCID: PMC6590186 DOI: 10.1002/jhbp.596] [Citation(s) in RCA: 94] [Impact Index Per Article: 15.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
IgG4‐related sclerosing cholangitis (IgG4‐SC) is a distinct type of cholangitis frequently associated with autoimmune pancreatitis and currently recognized as a biliary manifestation of IgG4‐related disease. Although clinical diagnostic criteria of IgG4‐SC were established in 2012, differential diagnosis from primary sclerosing cholangitis and cholangiocarcinoma is sometimes difficult. Furthermore, no practical guidelines for IgG4‐SC are available. Because the evidence level of most articles retrieved through searching the PubMed, Cochrane Library, and Igaku Chuo Zasshi databases was below C based on the systematic review evaluation system of clinical practice guidelines MINDS 2014, we developed consensus guidelines using the modified Delphi approach. Three committees (a guideline creating committee, an expert panelist committee for rating statements according to the modified Delphi method, and an evaluating committee) were organized. Eighteen clinical questions (CQs) with clinical statements were developed regarding diagnosis (14 CQs) and treatment (4 CQs). Recommendation levels for clinical statements were set using the modified Delphi approach. The guidelines explain methods for accurate diagnosis, and safe and appropriate treatment of IgG4‐SC.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan, Komagome Hospital, Tokyo, Japan
| | - Takahiro Nakazawa
- Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan
| | - Susumu Tazuma
- Department of General Internal Medicine, Hiroshima University Graduate School of Biomedical & Health Science, Hiroshima, Japan
| | - Yoh Zen
- Department of Diagnostic Pathology, Kobe University, Kobe, Japan
| | - Atsushi Tanaka
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
| | - Hirotaka Ohara
- Department of Community-Based Medical Education, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Takashi Muraki
- Department of Medicine, Gastroenterology, Shinshu University, Matsumoto, Nagano, Japan
| | - Kazuo Inui
- Department of Gastroenterology, Second Teaching Hospital, Fujita Health University, Nagoya, Japan
| | - Dai Inoue
- Department of Radiology, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan
| | - Takayoshi Nishino
- Department of Gastroenterology, Tokyo Womens' Medical University Yachiyo Medical Center, Yachiyo, Japan
| | - Itaru Naitoh
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Takao Itoi
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo, Japan
| | - Kenji Notohara
- Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan
| | - Atsushi Kanno
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - Kensuke Kubota
- Department of Endoscopy, Yokohama City University Hospital, Yokohama, Japan
| | - Kenji Hirano
- Department of Gastroenterology, Tokyo Takanawa Hospital, Tokyo, Japan
| | - Hiroyuki Isayama
- Department of Gastroenterology, Graduate School of Medicine, Juntendo University, Tokyo, Japan
| | - Kyoko Shimizu
- Department of Gastroenterology, Tokyo Womens' Medical University, Tokyo, Japan
| | | | - Tooru Shimosegawa
- Division of Gastroenterology, South-Miyagi Medical Center, Ohgawara, Japan
| | - Shigeyuki Kawa
- Department of Internal Medicine, Matsumoto Dental University, Matsumoto, Japan
| | | | - Kazuichi Okazaki
- The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Moriguchi, Japan
| | - Hajime Takikawa
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
| | - Wataru Kimura
- Faculty of Medicine, Departments of Gastroenterology and Gastroenterological, General, Breast, and Thyroid Surgery, Yamagata University, Yamagata, Japan
| | - Michiaki Unno
- Division of Hepato-Biliary Pancreatic Surgery, Tohoku University Graduate School, of Medicine, Sendai, Japan
| | - Masahiro Yoshida
- Department of Hepato-Biliary-Pancreatic and Gastrointestinal Surgery, School of Medicine, International University of Health and Welfare, Ichikawa, Japan
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19
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Xiao X, Lian M, Zhang W, Eric Gershwin M, Ma X. The Immunologic Paradoxes of IgG4-Related Disease. Clin Rev Allergy Immunol 2018; 54:344-351. [PMID: 29460058 DOI: 10.1007/s12016-018-8679-y] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
IgG4-related disease (IgG4-RD), which usually occurs in middle-aged and elderly men, is a newly recognized fibroinflammatory condition characterized by swelling and sclerosis of involved organs, increased IgG4-positive plasma cell infiltration in lesions, and elevated IgG4 concentration in serum. Despite growing interest in the research, the pathophysiological mechanism remains elusive. Most IgG4-RD patients respond well to steroid therapy initially, but recurrent and refractory cases are common, especially in advanced fibrotic stage. Recent studies have documented the heterogeneity of the B cell lineages, which suggests their multiple functions in IgG4-RD beyond IgG4 production, such as cytokine secretion, antigen presentation, autoantibody production, and modulation of T and B cell interactions. Thus, a critical balance exists between pathogenic and regulatory B subsets to prevent immunopathology. A prompt response to B cell depletion therapy reported in recent cases strongly suggests the imbalance within B cell lineages in IgG4-RD. A more precise understanding of the pathogenesis of IgG4-RD will open up new perspectives for therapeutic strategy. With a particular emphasis on the novel B cell-targeted therapeutic strategies, this review highlights the immunologic features of IgG4-RD and the possible roles of B cell lineages in the pathogenesis of IgG4-RD.
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Affiliation(s)
- Xiao Xiao
- Division of Gastroenterology and Hepatology, Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China.,Key Laboratory of Gastroenterology and Hepatology, Ministry of Health, Shanghai JiaoTong University; Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China.,State Key Laboratory for Oncogenes and Related Genes, Shanghai JiaoTong University; Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China.,Renji Hospital, School of Medicine, Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China
| | - Min Lian
- Division of Gastroenterology and Hepatology, Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China.,Key Laboratory of Gastroenterology and Hepatology, Ministry of Health, Shanghai JiaoTong University; Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China.,State Key Laboratory for Oncogenes and Related Genes, Shanghai JiaoTong University; Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China.,Renji Hospital, School of Medicine, Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China
| | - Weici Zhang
- Department of Internal Medicine, Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 Health Sciences Drive, Suite 6510, Davis, CA, 95616, USA
| | - M Eric Gershwin
- Department of Internal Medicine, Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 Health Sciences Drive, Suite 6510, Davis, CA, 95616, USA.
| | - Xiong Ma
- Division of Gastroenterology and Hepatology, Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China. .,Key Laboratory of Gastroenterology and Hepatology, Ministry of Health, Shanghai JiaoTong University; Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China. .,State Key Laboratory for Oncogenes and Related Genes, Shanghai JiaoTong University; Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China. .,Renji Hospital, School of Medicine, Shanghai JiaoTong University, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China.
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Dong Y, D'Onofrio M, Hocke M, Jenssen C, Potthoff A, Atkinson N, Ignee A, Dietrich CF. Autoimmune pancreatitis: Imaging features. Endosc Ultrasound 2018; 7:196-203. [PMID: 28836516 PMCID: PMC6032703 DOI: 10.4103/eus.eus_23_17] [Citation(s) in RCA: 39] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Background and Objectives Autoimmune pancreatitis (AIP) remains a difficult disease to diagnose before treatment, particularly if presenting as a focal mass lesion. The purpose of this multicenter retrospective study is to analyze imaging features of histologically confirmed AIP to determine the additional diagnostic value of contrast-enhanced ultrasound (CEUS), contrast-enhanced endoscopic ultrasound (CE-EUS), and elastography to B-mode features. Patients and Methods We report on a retrospective data collection of 60 histologically confirmed cases of AIP in comparison to 16 patients with pancreatic adenocarcinomas (PDAC). All CE (-E) US examinations were assessed by two independent readers in consensus. The role of CEUS and CE-EUS for pancreatic evaluation was defined according to the 2011 European Federation of Societies for Ultrasound in Medicine and Biology guidelines. Results After injection of ultrasound (US) contrast agents, most AIP lesions displayed focal or diffuse isoenhancement (86.6%) in the arterial phase, while most of the PDAC lesions (93.7%) were hypoenhancing (P < 0.01). During the late phase, most AIP lesions were hyper-(65%) or iso-enhancing (35%), while most PDAC lesions were hypoenhancing (93.7%). CE-EUS was performed in a subset of ten patients and showed hyperenhancement in all AIP cases. Most focal AIP lesions (n = 27, 79.4%) were stiffer than the surrounding pancreatic parenchyma. Conclusions In this study, percutaneous and endoscopic contrast enhanced harmonic US techniques consistently revealed diffuse and focal types of AIP to have features consistent with vascularized lesions. Differentiation from the typically hypovascularized pancreatic adenocarcinoma was possible with CE (-E) US evaluation.
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Affiliation(s)
- Yi Dong
- Department of Ultrasound, Zhongshan Hospital, Fudan University, 200032 Shanghai, China
| | - Mirko D'Onofrio
- Department of Radiology, GB Rossi University Hospital, University of Verona, Verona, Italy
| | - Michael Hocke
- Medical Department, Helios Klinikum Meiningen, Meiningen, Germany
| | - Christian Jenssen
- Department of Internal Medicine, Krankenhaus Märkisch Oderland, Strausberg, Germany
| | - Andrej Potthoff
- Department of Gastroenterology, Hepatology and Endocrinology, Medizinische Hochschule Hannover, Hannover, Germany
| | - Nathan Atkinson
- Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford University Hospitals NHS Trust, Oxford, UK
| | - Andre Ignee
- Medical Department, Caritas Krankenhaus, Uhlandstr. 7, D-97980, Bad Mergentheim, Germany
| | - Christoph F Dietrich
- Medical Department, Caritas Krankenhaus, Uhlandstr. 7, D-97980, Bad Mergentheim, Germany
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21
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Fujii-Lau LL, Levy MJ. The Role of Endoscopic Ultrasound in the Diagnosis of Autoimmune Pancreatitis. Gastrointest Endosc Clin N Am 2017; 27:643-655. [PMID: 28918803 DOI: 10.1016/j.giec.2017.06.005] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
Autoimmune pancreatitis (AIP) is increasingly being recognized due to improved understanding of the disease and its criteria for diagnosis. The classic type 1 AIP can be diagnosed on clinical data, but type 2 AIP requires histologic confirmation. Current criteria incorporate cross-sectional imaging and endoscopic retrograde cholangiopancreatography in the diagnosis of AIP. However, endoscopic ultrasound (EUS) will likely have an increasing role in the diagnosis through its characteristic imaging, image-enhancing techniques, and its ability to acquire tissue through either fine needle aspiration or biopsy. This article will review the diagnostic challenges of AIP and the current role of EUS.
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Affiliation(s)
| | - Michael J Levy
- Division of Gastroenterology and Hepatology, Mayo Clinic, 200 1st Street Southwest, Rochester, MN 55902, USA.
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Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis. J Gastroenterol 2017; 52:483-493. [PMID: 27470434 DOI: 10.1007/s00535-016-1246-5] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/10/2016] [Accepted: 07/17/2016] [Indexed: 02/04/2023]
Abstract
BACKGROUND Recent research has shown that a substantial number of patients with primary sclerosing cholangitis (PSC) can also have elevated serum/tissue IgG4. The aim of our study was to develop a simple scoring system for the discrimination of IgG4-related sclerosing cholangits (IgG4-SC) from PSC. METHODS Patients with IgG4-SC (n = 39) and PSC (n = 76) who had intrahepatic/hilar strictures were included. Candidate-differentiating variables included patient age, other organ involvement (OOI), inflammatory bowel disease, serum IgG4, and cholangiographic features. A scoring system was developed on the basis of these variables, and its performance was internally validated using a bootstrapping-based method. RESULTS The scoring system in the final model included age (<30 years, 0 points; 30-39 years, 1 point; 40-49 years, 2 points; 50-59 years, 3 points; ≥60 years, 4 points), OOI (no, 0 points; yes, 3 points), and beaded appearance (yes, 0 points; no, 2 points). The patients were classified according to their total score into three categories: 0-4 points, probable PSC; 5-6 points, indicating diagnostic steroid trial; 7-9 points, probable IgG4-SC. The discrimination between IgG4-SC and PSC using the scoring system was excellent (area under the receiver operating characteristic curve, 0.986). CONCLUSIONS A reliable differentiation of IgG4-SC from PSC can be made using the scoring system presented here. We suggest the diagnosis of IgG4-SC at a cutoff of 7 points or higher and the indication of diagnostic steroid trial at 5 or 6 points. External validation of our scoring system is warranted.
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From Pathogenesis, Clinical Manifestation, and Diagnosis to Treatment: An Overview on Autoimmune Pancreatitis. Gastroenterol Res Pract 2017; 2017:3246459. [PMID: 28197205 PMCID: PMC5288542 DOI: 10.1155/2017/3246459] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/12/2016] [Revised: 11/01/2016] [Accepted: 12/27/2016] [Indexed: 02/06/2023] Open
Abstract
Autoimmune pancreatitis (AIP) is a special type of chronic pancreatitis which is autoimmune mediated. The international consensus diagnostic criteria (ICDC) 2011 proposed two types of AIP: type I is associated with histological pattern of lymphoplasmacytic sclerosing pancreatitis (LPSP), characterized by serum IgG4 elevation, whereas type 2 is named idiopathic duct-centric pancreatitis (IDCP), with granulocytic epithelial lesion (GEL) and immunoglobulin G4 (IgG4) negative. The pathogenic mechanism is unclear now; based on genetic factors, disease specific or related antigens, innate and adaptive immunity may be involved. The most common clinical manifestations of AIP are obstructive jaundice and upper abdominal pain. The diagnosis can be made by a combination of parenchymal and ductal imaging, serum IgG4 concentrations, pancreatic histology, extrapancreatic disease, and glucocorticoid responsiveness according to ICDC 2011. Because of the clinical and imaging similarities with pancreatic cancer, general work-up should be done carefully to exclude pancreatic malignant tumor before empirical trial of glucocorticoid treatment. Glucocorticoid is the most common drug for AIP to induce remission, while there still exists controversy on steroid maintenance and treatment for relapse. Further studies should be done to identify more specific serum biomarkers for AIP, the pathogenic mechanisms, and the treatment for relapse.
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Jani N, Buxbaum J. Autoimmune pancreatitis and cholangitis. World J Gastrointest Pharmacol Ther 2015; 6:199-206. [PMID: 26558153 PMCID: PMC4635159 DOI: 10.4292/wjgpt.v6.i4.199] [Citation(s) in RCA: 5] [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: 04/28/2015] [Revised: 06/22/2015] [Accepted: 08/31/2015] [Indexed: 02/06/2023] Open
Abstract
Autoimmune pancreatitis (AIP) is part of a systemic fibrosclerotic process characterized by lymphoplasmacytic infiltrate with immunoglobulin G subtype-4 (IgG4) positive cells. It characteristically presents with biliary obstruction due to mass-like swelling of the pancreas. Frequently AIP is accompanied by extra-pancreatic manifestations including retroperitoneal fibrosis, thyroid disease, and salivary gland involvement. Auto-antibodies, hypergammaglobulemia, and prompt resolution of pancreatic and extrapancreatic findings with steroids signify its autoimmune nature. Refractory cases are responsive to immunomodulators and rituximab. Involvement of the biliary tree, termed IgG4 associated cholangiopathy, mimics primary sclerosing cholangitis and is challenging to manage. High IgG4 levels and swelling of the pancreas with a diminutive pancreatic duct are suggestive of autoimmune pancreatitis. Given similarities in presentation but radical differences in management and outcome, differentiation from pancreatic malignancy is of paramount importance. There is controversy regarding the optimal diagnostic criterion and steroid trials to make the diagnosis. Additionally, the retroperitoneal location of the pancreas and requirement for histologic sampling, makes tissue acquisition challenging. Recently, a second type of autoimmune pancreatitis has been recognized with similar clinical presentation and steroid response though different histology, serologic, and extrapancreatic findings.
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26
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Bor R, Madácsy L, Fábián A, Szepes A, Szepes Z. Endoscopic retrograde pancreatography: When should we do it? World J Gastrointest Endosc 2015; 7:1023-1031. [PMID: 26322155 PMCID: PMC4549659 DOI: 10.4253/wjge.v7.i11.1023] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/16/2015] [Revised: 06/30/2015] [Accepted: 08/14/2015] [Indexed: 02/05/2023] Open
Abstract
Endoscopic retrograde pancreatography (ERP) is an accurate imaging modality in the diagnosis of pancreatobiliary diseases. However, its use has been substantially reduced due to the invasiveness of procedure, the risk of complications and the widespread availability of non-invasive cross-section imaging techniques (computed tomography, magnetic resonance imaging, and endoscopic ultrasound). Since the introduction of endoscopic sphincterotomy, ERP has transformed from diagnostic method to an almost exclusively therapeutic procedure. Pancreatic duct injection substantially increased the risk of post-ERP pancreatitis (1.6%-15.7%); therefore, according to international guidelines ERP is recommended only in cases where biliary intervention is required. However, the role of ERP in the management of pancreatic diseases is currently not clearly defined, but in some cases the filling of pancreatic duct may provide essential information complementing the results of non-invasive imaging techniques. The aim of this publication is to systematically summarize the literature dealing with the diagnostic yield of ERP. We would like to define the precise indications of ERP and overview a diagnostic protocol of pancreatic diseases depending on international guidelines and the opinion of Hungarian experts, because it may improve the diagnostic accuracy, minimize of burden of patients and reduce the risk of procedure related complications.
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27
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Chandrasekhara V, Chathadi KV, Acosta RD, Decker GA, Early DS, Eloubeidi MA, Evans JA, Faulx AL, Fanelli RD, Fisher DA, Foley K, Fonkalsrud L, Hwang JH, Jue TL, Khashab MA, Lightdale JR, Muthusamy VR, Pasha SF, Saltzman JR, Sharaf R, Shaukat A, Shergill AK, Wang A, Cash BD, DeWitt JM. The role of endoscopy in benign pancreatic disease. Gastrointest Endosc 2015; 82:203-14. [PMID: 26077456 DOI: 10.1016/j.gie.2015.04.022] [Citation(s) in RCA: 56] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/14/2015] [Accepted: 04/14/2015] [Indexed: 02/06/2023]
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Comparison of endoscopic retrograde cholangiopancreatography with papillary biopsy and endoscopic ultrasound-guided pancreatic biopsy in the diagnosis of autoimmune pancreatitis. Pancreatology 2015; 15:259-64. [PMID: 25891790 DOI: 10.1016/j.pan.2015.03.011] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/04/2014] [Revised: 01/06/2015] [Accepted: 03/23/2015] [Indexed: 12/11/2022]
Abstract
BACKGROUND International consensus diagnostic criteria (ICDC) have been proposed for the diagnostic criteria and algorithm of autoimmune pancreatitis (AIP). Although endoscopy is important in the diagnosis of AIP, practical patterns of its usage vary considerably worldwide. This study aimed to compare endoscopic retrograde cholangiopancreatography (ERCP) with papillary biopsy and endoscopic ultrasound (EUS)-guided pancreatic biopsy for diagnosing AIP using ICDC. METHODS We retrospectively reviewed and classified 165 Korean patients diagnosed by Korean criteria from June 2007 to October 2013. Among them, 61 patients underwent ERCP with duodenal papillary biopsy (group A) and 62 patients underwent EUS-guided pancreatic biopsy (group B). We analyzed the diagnostic criteria and levels of each criterion, and type of AIP before and after endoscopic procedures. RESULTS ERCP with papillary biopsy increased the diagnostic sensitivity from 65.6% (40/61) to 95.1% (58/61) (P < 0.01). EUS-guided pancreatic biopsy increased the diagnostic sensitivity from 50.0% (27/62) to 88.7% (55/62) (P < 0.01). The increases of diagnostic sensitivity in two endoscopic methods were not different statistically. In diagnosing definite AIP, EUS-guided pancreatic biopsy was more useful than ERCP with papilla biopsy (sensitivity; 79.0% vs. 65.6%, P < 0.01). EUS-guided pancreatic biopsy was helpful to classify type 1 and type 2 AIP in some patients. Procedure-related complication (mild pancreatitis) developed in one patient (1.6%) in group A and two patients (3.2%) in group B. ERCP with papillary biopsy was less expensive than EUS-guided pancreatic biopsy. CONCLUSIONS Both ERCP with papillary biopsy and EUS-guided pancreatic biopsy are safe and play important roles in diagnosing AIP according to the ICDC.
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Kanno A, Masamune A, Shimosegawa T. Endoscopic approaches for the diagnosis of autoimmune pancreatitis. Dig Endosc 2015; 27:250-8. [PMID: 25115499 DOI: 10.1111/den.12343] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/08/2014] [Accepted: 08/04/2014] [Indexed: 12/21/2022]
Abstract
Autoimmune pancreatitis (AIP) is characterized by diffuse pancreatic enlargement and irregular narrowing of the main pancreatic duct (MPD). Immunoglobulin (Ig)G4-related sclerosing cholangitis (IgG4-SC) associated with AIP frequently appears as a bile duct stricture. Therefore, it is important to differentiate AIP and IgG4-SC from pancreatic cancer and cholangiocarcinoma or primary sclerosing cholangitis, respectively. Endoscopy plays a central role in the diagnosis of AIP and IgG4-SC because it provides imaging of the MPD and bile duct strictures as well as the ability to obtain tissue samples for histological evaluations. Diffuse irregular narrowing of MPD on endoscopic retrograde cholangiopancreatography (ERCP) is rather specific to AIP, but localized narrowing of the MPD is often difficult to differentiate from MPD stenosis caused by pancreatic cancer. A long stricture (>1/3 the length of the MPD) and lack of upstream dilatation from the stricture (<5 mm) might be key features of AIP on ERCP. Some cholangiographic features, such as segmental strictures, stric tures of the lower bile duct, and long strictures with prestenotic dilatation, are more common in IgG4-SC than in cholangiocarcinoma. Endoscopic ultrasonography (EUS) reveals diffuse hypoechoic pancreatic enlargement, sometimes with hypoechoic inclusions, in patients with AIP. In addition, EUS-elastography and contrast-enhanced harmonic EUS have been developed with promising results. The usefulness of EUS-guided fine-needle aspiration has been increasingly recognized for obtaining adequate tissue samples for the histological diagnosis of AIP. Further improvement of endoscopic procedures and devices will contribute to more accurate diagnosis of AIP and IgG4-SC.
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Affiliation(s)
- Atsushi Kanno
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan
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Kamisawa T, Chari ST, Lerch MM, Kim MH, Gress TM, Shimosegawa T. Republished: recent advances in autoimmune pancreatitis: type 1 and type 2. Postgrad Med J 2014; 90:18-25. [PMID: 24336310 DOI: 10.1136/postgradmedj-2012-304224rep] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis characterised clinically by frequent presentation with obstructive jaundice, histologically by a lymphoplasmacytic infiltrate with fibrosis, and therapeutically by a dramatic response to steroids. When so defined, AIP can be sub-classified into two subtypes, 1 and 2. Recent international consensus diagnostic criteria for AIP have been developed for diagnosis of both forms of AIP. Type 1 AIP is the pancreatic manifestation of a multiorgan disease, recently named IgG4-related disease. Little is known about the pathogenesis of either form of AIP. Despite frequent association of type 1 AIP with elevated serum IgG4 levels and infiltration with IgG4-positive plasma cells, it is unlikely that IgG4 plays a pathogenic role in AIP. Type 1 AIP responds to steroids, but there needs to be consensus on treatment regimens for induction and therapeutic end points. Relapses are common, but can be reduced by long-term use of low-dose steroids. Recent reports suggest that immunomodulators (azathioprine, 6-mercaptopurine and mycophenolate mofetil), as well biological agents (the antibody to CD20, rituximab) may have a role in maintaining remission in relapsing type 1 AIP. Future studies should clarify the best management options for treatment of relapses and maintenance of remission. Type 2 AIP is a pancreas-specific disorder not associated with IgG4. It presents in younger individuals equally with obstructive jaundice and pancreatitis. The inflammatory process responds to steroid therapy; relapses are uncommon. The clinical spectrum and long-term outcomes of medically treated type 2 AIP are still being evaluated.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, , Tokyo, Japan
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Kamisawa T, Ohara H, Kim MH, Kanno A, Okazaki K, Fujita N. Role of endoscopy in the diagnosis of autoimmune pancreatitis and immunoglobulin G4-related sclerosing cholangitis. Dig Endosc 2014; 26:627-35. [PMID: 24712522 DOI: 10.1111/den.12289] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/07/2014] [Accepted: 02/19/2014] [Indexed: 12/13/2022]
Abstract
Autoimmune pancreatitis (AIP) must be differentiated from pancreatic carcinoma, and immunoglobulin (Ig)G4-related sclerosing cholangitis (SC) from cholangiocarcinoma and primary sclerosing cholangitis (PSC). Pancreatographic findings such as a long narrowing of the main pancreatic duct, lack of upstream dilatation, skipped narrowed lesions, and side branches arising from the narrowed portion suggest AIP rather than pancreatic carcinoma. Cholangiographic findings for PSC, including band-like stricture, beaded or pruned-tree appearance, or diverticulum-like outpouching are rarely observed in IgG4-SC patients, whereas dilatation after a long stricture of the bile duct is common in IgG4-SC. Transpapillary biopsy for bile duct stricture is useful to rule out cholangiocarcinoma and to support the diagnosis of IgG4-SC with IgG4-immunostaining. IgG4-immunostaining of biopsy specimens from the major papilla advances a diagnosis of AIP. Contrast-enhanced endoscopic ultrasonography (EUS) and EUS elastography have the potential to predict the histological nature of the lesions. Intraductal ultrasonographic finding of wall thickening in the non-stenotic bile duct on cholangiography is useful for distinguishing IgG4-SC from cholangiocarcinoma. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) is widely used to exclude pancreatic carcinoma. To obtain adequate tissue samples for the histological diagnosis of AIP, EUS-Tru-cut biopsy or EUS-FNA using a 19-gauge needle is recommended, but EUS-FNA with a 22-gauge needle can also provide sufficient histological samples with careful sample processing after collection and rapid motion of the FNA needles within the pancreas. Validation of endoscopic imaging criteria and new techniques or devices to increase the diagnostic yield of endoscopic tissue sampling should be developed.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
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Recent advances in the diagnosis and management of autoimmune pancreatitis. AJR Am J Roentgenol 2014; 202:1007-21. [PMID: 24758653 DOI: 10.2214/ajr.13.11247] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
OBJECTIVE Autoimmune pancreatitis (AIP) is a rare chronic relapsing steroid-responsive fibroinflammatory disorder of the pancreas that is likely caused by immune dysregulation. It is now thought that AIP consists of two distinct clinicopathologic syndromes currently designated as types 1 and 2. CONCLUSION A current update on etiopathogenesis, pathology, and clinical and imaging findings of AIP is provided with an emphasis on diagnosis and management.
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Song TJ, Kim MH, Kim MJ, Moon SH, Han JM. Clinical validation of the international consensus diagnostic criteria and algorithms for autoimmune pancreatitis: combined IAP and KPBA meeting 2013 report. Pancreatology 2014; 14:233-7. [PMID: 25062869 DOI: 10.1016/j.pan.2014.04.033] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/08/2014] [Revised: 04/28/2014] [Accepted: 04/29/2014] [Indexed: 12/11/2022]
Abstract
There have been great developments in the diagnosis and treatment of autoimmune pancreatitis (AIP) in the last decade. Most significantly, the international consensus diagnostic criteria (ICDC) proposed in 2011 were the first attempt to provide unified diagnostic criteria incorporating most features of the previously existing national criteria. However, the ICDC have not yet been prospectively validated using evidence-based studies since their introduction. An international symposium on the diagnosis of AIP was held in Seoul, South Korea on September 6, 2013, in cooperation with the International Association of Pancreatology and the Korean Pancreatobiliary Association meeting. In contrast to other symposia in the past, which had primarily focused on the diagnostic criteria themselves, expert panels in this symposium discussed how the diagnostic criteria and algorithms had been embraced in clinical settings to diagnose AIP in each country and conducted a comprehensive evaluation of these criteria and algorithms. It was acknowledged that there was a room for improvement in the ICDC and their algorithms and that further modifications might be required in the future. Prospective clinical validation in larger series is needed for confirmation.
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Affiliation(s)
- Tae Jun Song
- Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea
| | - Myung-Hwan Kim
- Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
| | - Min Jae Kim
- Department of Physician Education and Training, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea
| | - Sung Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Anyang, South Korea
| | - Ji Min Han
- Department of Internal Medicine, Daegu Catholic University Medical Center, Daegu, South Korea
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Yamashita H, Naitoh I, Nakazawa T, Hayashi K, Miyabe K, Shimizu S, Kondo H, Yoshida M, Umemura S, Hori Y, Ohara H, Takahashi S, Joh T. A comparison of the diagnostic efficacy in type 1 autoimmune pancreatitis based on biopsy specimens from various organs. Pancreatology 2014; 14:186-92. [PMID: 24854614 DOI: 10.1016/j.pan.2014.03.003] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/11/2013] [Revised: 02/26/2014] [Accepted: 03/03/2014] [Indexed: 12/11/2022]
Abstract
BACKGROUND Comprehensive immunostaining evaluation of the biopsy specimens from various organs with type 1 autoimmune pancreatitis (AIP) has not been elucidated. Our aim was to clarify which of these biopsy specimens and counting method could be a useful tool for supporting the diagnosis of AIP. METHODS We retrospectively evaluated biopsy specimens from pancreas (n = 19), stomach (n = 28), duodenum (n = 27), duodenal papilla (n = 25), colon (n = 19), liver (n = 11), bile duct (n = 24), and minor salivary gland (n = 13) in 36 patients with AIP. Positive IgG4 immunostaining (>10 plasma cells/high-power field [HPF]) and positive IgG4/IgG ratio (>40%) of biopsy specimens from 8 sites of 6 organs in one HPF and an average from 3 HPFs were compared between AIP and controls. RESULTS The sensitivity of IgG4 immunostaining for AIP in one HPF were 16% in pancreas, 14% in stomach, 15% in duodenum, 52% in duodenal papilla, 11% in colon, 27% in liver, 21% in bile duct and 8% in minor salivary gland, respectively. The positive IgG4 immunostaining of the duodenal papilla in one HPF showed the highest sensitivity (52%) and accuracy (73%) among the 8 sites. It also showed the highest sensitivity among 4 different counting methods (IgG4 immunostaining in one HPF and 3 HPFs, both IgG4 immunostaining and IgG/IgG4 ratio in one HPF and 3 HPFs), but there were no significant differences with respect to specificity and accuracy. CONCLUSIONS IgG4 immunostaining of swollen duodenal papilla with more than 10 IgG4-positive plasma cells in at least one HPF is useful for supporting the diagnosis of AIP.
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Affiliation(s)
- Hiroaki Yamashita
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Itaru Naitoh
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.
| | - Takahiro Nakazawa
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Kazuki Hayashi
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Katsuyuki Miyabe
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Shuya Shimizu
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Hiromu Kondo
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Michihiro Yoshida
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Shuichiro Umemura
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Yasuki Hori
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Hirotaka Ohara
- Department of Community-based Medical Education, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Satoru Takahashi
- Department of Experimental Pathology and Tumor Biology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Takashi Joh
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
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Nakazawa T, Naitoh I, Hayashi K, Miyabe K, Simizu S, Joh T. Diagnosis of IgG4-related sclerosing cholangitis. World J Gastroenterol 2013; 19:7661-7670. [PMID: 24282356 PMCID: PMC3837265 DOI: 10.3748/wjg.v19.i43.7661] [Citation(s) in RCA: 68] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/20/2013] [Revised: 07/23/2013] [Accepted: 09/29/2013] [Indexed: 02/06/2023] Open
Abstract
IgG4-related sclerosing cholangitis (IgG4-SC) is often associated with autoimmune pancreatitis. However, the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis (PSC), and the presence of segmental stenosis suggests cholangiocarcinoma (CC). IgG4-SC responds well to steroid therapy, whereas PSC is only effectively treated with liver transplantation and CC requires surgical intervention. Since IgG4-SC was first described, it has become a third distinct clinical entity of sclerosing cholangitis. The aim of this review was to introduce the diagnostic methods for IgG4-SC. IgG4-SC should be carefully diagnosed based on a combination of characteristic clinical, serological, morphological, and histopathological features after cholangiographic classification and targeting of a disease for differential diagnosis. When intrapancreatic stenosis is detected, pancreatic cancer or CC should be ruled out. If multiple intrahepatic stenoses are evident, PSC should be distinguished on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining. Associated inflammatory bowel disease is suggestive of PSC. If stenosis is demonstrated in the hepatic hilar region, CC should be discriminated by ultrasonography, intraductal ultrasonography, bile duct biopsy, and a higher cutoff serum IgG4 level of 182 mg/dL.
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Moon SH, Kim MH. Autoimmune pancreatitis: role of endoscopy in diagnosis and treatment. Gastrointest Endosc Clin N Am 2013; 23:893-915. [PMID: 24079796 DOI: 10.1016/j.giec.2013.06.005] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
This review addresses the role of endoscopy in the diagnosis and treatment of autoimmune pancreatitis (AIP) and provides a diagnostic process for patients with suspected AIP. When should AIP be suspected? When can it be diagnosed without endoscopic examination? Which endoscopic approaches are appropriate in suspected AIP, and when? What are the roles of diagnostic endoscopic retrograde pancreatography, endoscopic biopsies, and IgG4 immunostaining? What is the proper use of the steroid trial in the diagnosis of AIP in patients with indeterminate computed tomography imaging? Should biliary stenting be performed in patients with AIP with obstructive jaundice?
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Affiliation(s)
- Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, 896 Pyeongchon-dong, Dongan-gu, Anyang 431-070, South Korea
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Kamisawa T, Chari ST, Lerch MM, Kim MH, Gress TM, Shimosegawa T. Recent advances in autoimmune pancreatitis: type 1 and type 2. Gut 2013; 62:1373-80. [PMID: 23749606 DOI: 10.1136/gutjnl-2012-304224] [Citation(s) in RCA: 115] [Impact Index Per Article: 9.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis characterised clinically by frequent presentation with obstructive jaundice, histologically by a lymphoplasmacytic infiltrate with fibrosis, and therapeutically by a dramatic response to steroids. When so defined, AIP can be sub-classified into two subtypes, 1 and 2. Recent international consensus diagnostic criteria for AIP have been developed for diagnosis of both forms of AIP. Type 1 AIP is the pancreatic manifestation of a multiorgan disease, recently named IgG4-related disease. Little is known about the pathogenesis of either form of AIP. Despite frequent association of type 1 AIP with elevated serum IgG4 levels and infiltration with IgG4-positive plasma cells, it is unlikely that IgG4 plays a pathogenic role in AIP. Type 1 AIP responds to steroids, but there needs to be consensus on treatment regimens for induction and therapeutic end points. Relapses are common, but can be reduced by long-term use of low-dose steroids. Recent reports suggest that immunomodulators (azathioprine, 6-mercaptopurine and mycophenolate mofetil), as well biological agents (the antibody to CD20, rituximab) may have a role in maintaining remission in relapsing type 1 AIP. Future studies should clarify the best management options for treatment of relapses and maintenance of remission. Type 2 AIP is a pancreas-specific disorder not associated with IgG4. It presents in younger individuals equally with obstructive jaundice and pancreatitis. The inflammatory process responds to steroid therapy; relapses are uncommon. The clinical spectrum and long-term outcomes of medically treated type 2 AIP are still being evaluated.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
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Abstract
Autoimmune pancreatitis (AIP) is a rare, heterogeneous, fibroinflammatory disorder of the pancreas. It has gained increasing recognition due to a presentation that can mimic difficult-to-treat disorders such as pancreatic cancer, cholangiocarcinoma and primary sclerosing cholangitis. In contrast, autoimmune pancreatitis is a benign disease that is very responsive to therapy with corticosteroids. There are two types of AIP. Type 1 disease is the most common worldwide and is associated with extrapancreatic manifestations and elevated levels of IgG4-positive cells. Type 2 AIP is characterized by a paucity of IgG4-positive cells and is more difficult to diagnose. This review provides an update on the diagnosis, pathophysiology and treatment of AIP, with special emphasis on the two subtypes.
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Affiliation(s)
- Gyanprakash A. Ketwaroo
- Department of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA
| | - Sunil Sheth
- Department of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA
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