Clinical practice guidelines for IgG4-related sclerosing cholangitis

Abstract

IgG4-related sclerosing cholangitis 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-related sclerosing cholangitis were established in 2012, differential diagnosis from primary sclerosing cholangitis and cholangiocarcinoma is sometimes difficult. Because the evidence level of most retrived articles was low (below C), we developed consensus guidelines. 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 with clinical statements were developed regarding diagnosis and treatment. 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-related sclerosing cholangitis.