Primary biliary cholangitis

Abstract

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease predominantly affecting middle-aged women. It does not occur in children. PBC prevalence varies depending on the geographic location of the country. Over the past 30 years, there has been an increased incidence of PBC, while significant progress has been made in understanding the pathogenesis of PBC due to the development of innovative technologies in molecular biology, immunology and genetics. The presence of antimitochondrial antibodies and cholestasis on biochemical analysis is sufficient to make a diagnosis, without the need for liver biopsy. Small- and medium-sized bile ducts are the targets of PBC. In the first stage of the disease, granulomatous destruction of the bile ducts occurs; in the second stage, loss of bile ducts, their proliferation, increased size of the portal tracts with chronic inflammation; in the third stage - fibrosis with septal formation, loss of bile ducts and cholestasis; in the fourth stage - liver cirrhosis. Previously, the survival rate of PBC patients ranged from 7.5 to 16 years. However, it has improved significantly with ursodeoxycholic acid and obeticholic acid treatment. If there is no effect from treatment and end-stage liver failure sets in, liver transplantation is performed.

PPAR agonists in Primary Biliary Cholangitis: A Systematic Review and Meta-Analysis of Randomized Controlled Trials

“…Due to epithelium in ammation, bile excretion is impaired, causing bile accumulation in the gallbladder and re ux of substances to the liver. [40] Among the main substances are bile salts with hepatotoxic potential, which, when returning to the liver, stimulate the synthesis of ALP, re ecting hepatocyte and ductal cell distress. Recent studies demonstrated the bene ts of PPAR analogs in laboratory markers by reducing bile acid toxicity and associated lesions.…”