Abstract:Objective To analyze the clinical features of patients with primary sclerosing cholangitis (PSC) and refine the diagnostic accuracy and treatment. Methods The clinical data of 25 PSC patients hospitalized from May 2009 to May 2019 were analyzed retrospectively,and the clinical manifestations,laboratory and imaging features,concurrent diseases and therapeutic options were summarized. Results Out of 25 patients diagnosed with large-duct PSC (including 1 case complicated with small bile duct damage),there were 16 males and 9 females,aged 19-79 years with a median age of 57 years.The main clinical presentations were jaundice in 10 cases,abdominal distention in 8 cases,abdominal pain in 7 cases and fatigue in 7 cases.Liver cirrhosis was found in 8 cases,including 7 confirmed and 1 suspected;ulcerative colitis occurred in 4 cases,including 2 cases concurrently,1 case before PSC and 1 case after PSC diagnosis;autoimmune hepatitis,ankylosing spondylitis and psoriatic arthritis occurred in one case,respectively.There were no patients complicated with cholangiocarcinoma. In liver function test, γ-GT, ALP and T-Bil increased most obviously. Elevations of γ-GT were found in 22 cases (median value:215 U/L),ALP in 19 cases (median value:284 U/L),T-bil in 14 cases (median value:27.4 umol/L).Out of 22 cases who completed quantitative analysis of immunoglobulin Ig,IgG increased in 10 cases (median value:14.5 g/L),IgM increased in 6 cases (median value:1.205 g/L).In 10 cases who received ANCA tests,3 cases were positive(1 case of perinuclear ANCA,1 case of antiproteinase 3 antibody and 1 case of positive for both).For ANA tests in 15 cases,elevation was found in 9 cases (median value 1∶100).Out of 17 cases who received IgG4 tests,elevation was found in 5 cases (median value:0.862 g/L).All the patients underwent MRCP examination,and 13 cases showed involvement of both extrahepatic and intrahepatic bile duct,7 cases showed extrahepatic bile duct involvement alone,5 cases showed intrahepatic bile duct involvement alone.After biopsy was conducted in 3 patients,1 case presented with findings consistent with small-duct PSC,and 1 case was in line with the characteristics of autoimmune hepatitis.Endoscopic ultrasound was performed in one patient and supported the diagnosis of large-duct PSC.Out of 25 cases,20 cases were treated with ursodeoxycholic acid（UDCA）and 9 cases with glucocorticoid (7 of which were also with UDCA), and 1 case perfomed with bile duct drainage, so that to relieve biliary obstruction, and other 2 cases were treated with symptomatic treatment. Conclusions PSC usually occurs in the middle-aged adults with a slight male predominance.Liver fumtion and immunological tests could be suggestive of the disease.MRCP is the first-line diagnostic modality for PSC.UDCA is widely used in the treatment of PSC,but its efficacy is still to be validated.