Clinical diagnostic criteria of IgG4‐related sclerosing cholangitis 2012

• DOI: 10.1007/s00534-012-0521-y
• Publication Date: 2012-06-20T06:51:59Z
• Authors: Hirotaka Ohara; Kazuichi Okazaki; Hirohito Tsubouchi; Kazuo Inui; Shigeyuki Kawa; Terumi Kamisawa; Susumu Tazuma; Kazushige Uchida; Kenji Hirano; Hitoshi Yoshida; Takayoshi Nishino; Shigeru B.H. Ko; Nobumasa Mizuno; Hideaki Hamano; Atsushi Kanno; Kenji Notohara; Osamu Hasebe; Takahiro Nakazawa; Yasuni Nakanuma; Hajime Takikawa
• Keywords: Diagnosis, Differential; Pancreatic Neoplasms; 03 medical and health sciences; Biliary Tract Neoplasms; 0302 clinical medicine; Immunoglobulin G; Cholangitis, Sclerosing; Humans; Bile Ducts; 3. Good health

AbstractBackgroundIgG4‐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 progressive or malignant diseases.MethodsThe Research Committee of IgG4‐related Diseases and the Research Committee of Intractable Diseases of Liver and Biliary Tract in association with the Ministry of Health, Labor and Welfare, Japan and the Japan Biliary Association have set up a working group consisting of researchers specializing in IgG4‐SC, and established the new clinical diagnostic criteria of IgG4‐SC 2012.ResultsThe diagnosis of IgG4‐SC is based on the combination of the following 4 criteria: (1) characteristic biliary imaging findings, (2) elevation of serum IgG4 concentrations, (3) the coexistence of IgG4‐related diseases except those of the biliary tract, and (4) characteristic histopathological features. Furthermore, the effectiveness of steroid therapy is an optional extra diagnostic criterion to confirm accurate diagnosis of IgG4‐SC.ConclusionThese diagnostic criteria for IgG4‐SC are useful in practice for general physicians and other nonspecialists.