APASL clinical practice guidance: the diagnosis and management of patients with primary biliary cholangitis
Hong You, Xiong Ma, Cumali Efe, Guiqiang Wang, Sook‐Hyang Jeong, Kazumichi Abe, Weijia Duan, Sha Chen, Yuanyuan Kong, Dong Zhang, Lai Wei, Fu‐Sheng Wang, Han‐Chieh Lin, Jin Mo Yang, Tawesak Tanwandee, Rino Alvani Gani, Diana A. Payawal, Barjesh Chander Sharma, Jinlin Hou, Osamu Yokosuka, A. Kadir Dökmeci, Dorothy H. Crawford, Jia‐Horng Kao, Teerha Piratvisuth, Dong Jin Suh, Laurentius A. Lesmana, Jose D. Sollano, George Lau, Shiv Kumar Sarin, Masao Omata, Atsushi Tanaka, Jidong Jia
Abstract
Primary biliary cholangitis (PBC) is a chronic intrahepatic cholestatic disease with not fully elucidated pathogenesis. Immunological dysfunction triggered by environmental factors may render autoimmunity against the interlobular bile ducts in genetically predisposed hosts. PBC typically affects middle-aged women, commonly presents with fatigue and pruritus, or with an asymptomatic elevation of serum alkaline phosphatase (ALP)/glutamyl transpeptidase (GGT). The pathological features are progressive, non-suppurative, destructive intrahepatic cholangitis, leading to fibrosis and eventually cirrhosis. Antimitochondrial antibodies (AMAs), especially the M2 subtype (AMA-M2), are highly sensitive and specific for PBC in clinical settings. Currently, ursodeoxycholic acid (UDCA) is the treatment of choice for this disease.