Primary biliary cholangitis. (December 2019)

• Record Type: Journal Article
• Authors: Mells, George
• Is Part Of: Medicine. Volume 47:Number 12(2019)
• Page Start: 796
• Page End: 798
• Publication Date: 2019-12
• Subjects: Autoimmune liver disease -- cholestatic liver disease -- cholestatic pruritus -- colestyramine -- liver transplantation -- modafinil -- obeticholic acid -- PBC fatigue symptom complex -- rifampicin -- ursodeoxycholic acid; Medicine -- Periodicals; Medicine -- Periodicals; Médecine -- Périodiques; Médecine factuelle -- Périodiques; Medicine; Periodicals; Electronic journals; Electronic journals; 610
• DOI: 10.1016/j.mpmed.2019.09.012
• Languages: English
• ISSNs: 1357-3039

Abstract

Abstract: Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by lymphocytic destruction of the small, intrahepatic bile ducts, causing chronic cholestasis and progressive fibrosis that eventually leads to biliary cirrhosis. It is a complex disorder resulting from the interaction of genetic and environmental factors and is strongly predominant in female patients (female:male 9:1). Typical symptoms include cholestatic pruritus and the PBC fatigue symptom complex. Features of end-stage liver disease (ESLD) from PBC are the same as those of ESLD from other causes, i.e. variceal haemorrhage, ascites, jaundice and hepatic encephalopathy. First-line disease-modifying treatment for PBC is with ursodeoxycholic acid (UDCA). Patients with an inadequate biochemical response to UDCA (e.g. defined as serum alkaline phosphatase >1.67 times the upper limit of normal measured after 12 months of treatment) are prioritized for addition of second-line treatment with obeticholic acid or a fibrate, i.e. bezafibrate or fenofibrate. First-line treatment for PBC-related pruritus is with colestyramine. If colestyramine is unpalatable or ineffective, second-line treatment of pruritus is with rifampicin. Patients with the fatigue symptom complex and prominent somnolence can benefit from modafinil. Liver transplantation is indicated for PBC patients with chronic liver failure or intractable pruritus.