Clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2020: (Revision of the clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2012).

Published on Mar 1, 2021in Journal of Hepato-biliary-pancreatic Sciences4.16
· DOI :10.1002/JHBP.913
Takahiro Nakazawa29
Estimated H-index: 29
(Nagoya City University),
Terumi Kamisawa77
Estimated H-index: 77
+ 13 AuthorsTosiaki Unno (Tohoku University)
Background Several years have passed since the clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2012 were published. New findings and knowledge have accumulated since then. Methods The Research Committees for IgG4-related Diseases and for Intractable Diseases of the Liver and Biliary Tract in association with the Ministry of Health, Labor and Welfare of Japan and the Japan Biliary Association, have established a working group consisting of researchers specializing in IgG4-SC and have drawn up new clinical diagnostic criteria for IgG4-SC 2020. Results The diagnosis of IgG4-SC is based on a combination of the following six criteria: (1) narrowing of the intra- or extrahepatic bile duct, (2) thickening of the bile duct wall, (3) serological findings, (4) pathological findings, (5) other organ involvement, and (6) effectiveness of steroid therapy. Conclusion These new diagnostic criteria for IgG4-SC are useful in practice for general physicians and other non-specialists.
#1Shigeyuki Kawa (Matsumoto Dental University)H-Index: 67
#2Terumi KamisawaH-Index: 77
Last. Kazuichi OkazakiH-Index: 85
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17 CitationsSource
#1Terumi KamisawaH-Index: 77
#2Takahiro NakazawaH-Index: 29
Last. Masahiro Yoshida (International University of Health and Welfare)H-Index: 43
view all 27 authors...
: 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 r...
43 CitationsSource
#1Terumi KamisawaH-Index: 77
#2Yoh Zen (Kobe University)H-Index: 87
Last. Kazuichi Okazaki (Kansai Medical University)H-Index: 85
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Summary Autoimmune pancreatitis is an unusual form of pancreatitis that is histologically characterised by a massive infiltration of lymphocytes and IgG4-positive plasma cells and storiform fibrosis. The disease is recognised as a pancreatic manifestation of IgG4-related disease. IgG4-related sclerosing cholangitis is a biliary counterpart that is typically associated with autoimmune pancreatitis. Two parallel immunological responses are thought to underlie the pathophysiology of these diseases:...
19 CitationsSource
#1Atsushi Tanaka (Teikyo University)H-Index: 33
#2Susumu Tazuma (Hiroshima University)H-Index: 29
Last. Hajime Takikawa (Teikyo University)H-Index: 33
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Background & Aims Immunoglobulin G4 sclerosing cholangitis (IgG4-SC) is a biliary tract manifestation of IgG4-related systemic disease. We investigated the demographics, clinical features at presentation, treatment response, and outcomes of IgG4-SC using data from a large-scale survey in Japan. Methods We performed a retrospective cohort study of 527 patients with IgG4-SC (436 female; median age, 66.2 years; range, 23–89 years) in Japan from 2000 to 2015. Data on patient demographics, presentati...
45 CitationsSource
#1Takahiro NakazawaH-Index: 29
#2Shuya ShimizuH-Index: 13
Last. Itaru Naitoh (Nagoya City University)H-Index: 22
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Autoimmune pancreatitis (AIP) is frequently associated with IgG4-related sclerosing cholangitis (IgG4-SC). 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) (Nakazawa et al., Pancreas 30:20–25, 2005). IgG4-SC responds well to steroid therapy, whereas PSC is effectively treated only with liver transplantation, and cholangiocarcinoma requires sur...
14 CitationsSource
#1M. Yata (Nagoya University)H-Index: 1
#2K. Suzuki (Nagoya University)H-Index: 6
Last. Shinji Naganawa (Nagoya University)H-Index: 61
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Aim To compare the multidetector-row computed tomography (MDCT) findings of IgG4-related sclerosing cholangitis (IgG4-SC) and extrahepatic cholangiocarcinoma (EH-CCA). Materials and methods Two radiologists who had no knowledge of the patients' clinical information retrospectively evaluated the CT findings of patients with IgG4-SC (n=33) and EH-CCA (n=39) on a consensus basis. Another radiologist measured the biliary lesions. IgG4-SC was diagnosed using the Japan Biliary Association criteria (20...
17 CitationsSource
#1Takahiro Nakazawa (Nagoya City University)H-Index: 29
#2Yushi Ikeda (Nagoya City University)H-Index: 1
Last. Atsushi Tanaka (Nagoya City University)H-Index: 1
view all 10 authors...
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is frequently associated with type 1 autoimmune pancreatitis (AIP). Association with AIP can be utilized in the diagnosis of IgG4-SC. However, some cases of IgG4-SC are isolated from AIP, which complicates the diagnosis. Most of the reported cases of isolated IgG4-SC displayed hilar biliary strictures, whereas isolated IgG4-SC with intrapancreatic biliary stricture is very rare. Recently, we have encountered 5 isolated intrapancreatic Ig...
18 CitationsSource
#1Rondell P. Graham (Mayo Clinic)H-Index: 26
#2Thomas C. Smyrk (Mayo Clinic)H-Index: 116
Last. Lizhi Zhang (Mayo Clinic)H-Index: 54
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Summary Extrahepatic bile ducts are the most commonly involved extrapancreatic organ site in patients with type 1 autoimmune pancreatitis. IgG4-related sclerosing cholangitis (IgG4-SC) alone, without evidence of pancreatic or other organ involvement, is uncommon and is difficult to distinguish from cholangiocarcinoma preoperatively. We describe 9 patients with isolated IgG4-SC over an approximate 10-year period, each clinically suspected to have cholangiocarcinoma. We examined the clinical, radi...
34 CitationsSource
#1Ajay B. Tokala (Women's College Hospital)H-Index: 1
#2Korosh KhaliliH-Index: 26
Last. Kartik S. Jhaveri (UHN: University Health Network)H-Index: 22
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OBJECTIVE. The purpose of this study was to perform a retrospective MRI-based comparative analysis of the morphologic patterns of bile duct disease in IgG4-related systemic disease (ISD, also called autoimmune pancreatitis) compared with primary sclerosing cholangitis (PSC) and the autoimmune liver diseases autoimmune hepatitis and primary biliary cirrhosis. MATERIALS AND METHODS. This study included 162 consecutively registered patients (47 with ISD, 73 with PSC, and 42 with autoimmune liver di...
47 CitationsSource
#1Hirotaka Ohara (Nagoya City University)H-Index: 47
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 85
Last. Hajime Takikawa (Teikyo University)H-Index: 33
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Background IgG4-sclerosing cholangitis (IgG4-SC) patients have an increased level of serum IgG4, dense infiltration of IgG4-positive plasma cells with extensive fibrosis in the bile duct wall, and a good response to steroid therapy. However, it is not easy to distinguish IgG4-SC from primary sclerosing cholangitis, pancreatic cancer, and cholangiocarcinoma on the basis of cholangiographic findings alone because various cholangiographic features of IgG4-SC are similar to those of the above progre...
213 CitationsSource
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