Abstract: Objective Due to the similarity of clinical and imaging manifestations and lack of awareness of this disease, IgG4-related sclerosing cholangitis (IgG4-SC) is often misdiagnosed as primary sclerosing cholangitis (PSC), cholangiocarcinoma (CC) or other cholestatic diseases, and unnecessary surgical treatment is performed. The accurate understanding of IgG4-SC is very important. However, available clinical studies about IgG4-SC are comparatively insufficient, and most literatures are reports of sporadic cases, there is a need of systematic review of the entity. We need to know the clinical characteristics and misdiagnosis causes of IgG4-SC, and to improve the clinical diagnosis. Methods With "IgG4-related sclerosing cholangitis" as the key word, the literature published before September 2019 was searched on CNKI database, WANFANG database and VIP database, including general information, clinical manifestations, laboratory examinations, imaging findings, pathological features, misdiagnosis, treatment and prognosis of relevant cases. Results A total of 25 articles, including 100 patients, were obtained. The sex ratio of them was 4:1, with the mean age of illness onset of (58.8±12.3) years; the most common symptoms were jaundice (85%, 85/100) and abdominal pain (53%, 53/100); the serological tests showed that serum IgG4 level increased in 94.2% (81/86) of patients, and serum CA19-9 level increased in 33% of patients; the imaging findings included bile duct stenosis in the lower common bile duct in 57 cases and bile duct stricture in the hilar region with intrahepatic bile duct dilatation in 15 cases; the pathological findings in 34 cases examined showed the hyperplasia of fibrous tissue in the wall of gallbladder/bile duct with numerous lymphocyte, plasma cell infiltration, and obliterative phlebitis or storiform fibrosis; the immunohistochemistry in 15 cases showed the IgG4⁺ plasma cell were >10/HPF; out of the 100 patients, 12 cases were misdiagnosed as CC and surgical operation were inappropriately underwent, and 61 cases were treated with glucocorticoids, with remission in 58 cases (95%).Conclusion The main manifestations of patients with IgG4-SC are obstructive jaundice and abdominal pain, which are very similar to primary sclerosing cholangitis, cholangiocarcinoma, pancreatic cancer and other diseases, so it is necessary to pay attention to the differential diagnosis in this setting.

Key words: IgG4-related sclerosing cholangitis, Clinical features, Systemic analysis