原发性硬化性胆管炎(PSC)合并自身免疫性胆管炎(AIC)临床上较罕见。本文中报道的患者以口干、转氨酶升高为首发表现,经实验室检查及肝穿刺活检诊断为AIC。患者行磁共振胰胆管成像(MRCP)未提示肝外胆管病变,行经内镜逆行性胰胆管造影术(ERCP)可见肝内外胆管节段样狭窄,最终确诊为PSC合并AIC,现将此病例诊治过程报道如下。1病历资料患者女,57岁,因“口干、转氨酶升高2年,腹胀 Primary sclerosing cholangitis (PSC) with autoimmune cholangitis (AIC) is clinically rare. The patients reported in this article with dry mouth, elevated aminotransferases as the first performance, laboratory tests and liver biopsy diagnosed as AIC. Patients undergoing magnetic resonance cholangiopancreatography (MRCP) did not suggest extrahepatic bile duct disease, endoscopic retrograde cholangiopancreatography (ERCP) showed segmental intrahepatic and extrahepatic biliary strictures, the final diagnosis of PSC with AIC, the diagnosis and treatment of this case The process is reported below. A patient data, female, 57 years old, due to ”dry mouth, elevated transaminase for 2 years, bloating