New developments in the treatment of primary biliary cholangitis

Summary: Primary biliary cholangitis is a chronic, immunologically mediated, progressive liver dis ease characterized by progressive destruction of small bile ducts, resulting in cholestasis, liver fi brosis with the development of liver cirrhosis and all of its complications. These ultimately lead to liver failure, resulting in death or the need for a liver transplant. Early and eff ective treatment is a key factor in infl uencing the prognosis. The dis ease mainly aff ects women, accounting for 90% of cases. Dia gnosis is possible based on an elevation of serum alkaline phosphatase (ALP) above 1.5-times the normal level for 6 months and in combination with the presence of antimitochondrial antibodies (AMA) ≥ 1 : 40 or specifi c antinuclear antibodies (ANA) sp100, gp210, or a combination of elevated serum ALP with typical histological fi ndings found during liver biopsy. Although ursodeoxycholic acid (UDCA) remains the treatment of choice, unfortunately some patients do not tolerate this treatment or do not respond adequately to it. Second-line treatment is currently available – peroxisome proliferator-activated receptor (PPAR) agonists, delta (PPAR-) or dual agonists (PPAR-/ ). Seladelpar (PPAR-) and elafi branor (PPAR-/ ) are promising treatment alternatives due to their anti- -inflammatory and antifi brotic properties. Key words: primary biliary cholangitis – alkaline phosphatase – AMA antibodies – ANA antibodies – ursodeoxycholic acid – elafi branor – seladelpar