2021 Volume 35 Issue 1 Pages 54-61
IgG4-related sclerosing cholangitis (IgG4-SC) is a distinct type of cholangitis frequently associated with autoimmune pancreatitis around 70-90% of all and currently recognized as a biliary manifestation of IgG4-related disease. The therapeutic strategy for IgG4-SC, which sometimes presented without AIP, is applied to that of AIP in real situation. A clinical practical guideline 2019 for IgG4-SC was established, while there has been devoid of evidence such as characteristics of isolated type, long-term prognosis included cancerization, the feasibility and safety of the steroid and its pathogen.This guideline proceeded by experts was developed based on the consensus, which was decided by the modified Delphi approach to enhance its low evidence level. As such, hopefully, this guide could be a permanently reliable standard which would lead to the world consensus concerning the therapeutic strategy for IgG4-SC by making frequent revises with plausible evidence and promising biomarkers without cease our dedications.
