<p>Primary biliary cholangitis (PBC) is a&#xa0;chronic inflammatory, autoimmune-mediated liver disease that progresses to fibrosis and cirrhosis if left untreated. In addition to preventing complications, the management of burdensome symptoms, particularly pruritus, represents a&#xa0;key therapeutic goal. Ursodeoxycholic acid (UDCA) is the established first-line treatment; however, up to 40% of patients show an inadequate response and require second-line treatment. Bezafibrate, a&#xa0;pan-peroxisome proliferator–activated receptor (PPAR) agonist, has long been used off-label as an effective option to improve parameters of cholestasis and relieve pruritus. More recently, two new PPAR agonists, elafibranor (PPAR alpha/delta) and seladelpar (PPAR delta), have received conditional approval in Germany. Both demonstrated strong biochemical efficacy and a&#xa0;favorable safety profile in clinical trials. This article provides a&#xa0;comprehensive update on the diagnostics, risk stratification and treatment of PBC, with a&#xa0;particular focus on the role of novel PPAR agonists in second-line treatment. Practical strategies for the treatment of pruritus are also discussed. The aim is to support guideline-based care and to improve the treatment of patients with PBC through timely treatment adjustment and individualized treatment concepts.</p>

错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

Primär biliäre Cholangitis – Update zu Diagnostik, Risikostratifizierung und neuen Therapieoptionen

  • Toni Herta,
  • Thomas Berg

摘要

Primary biliary cholangitis (PBC) is a chronic inflammatory, autoimmune-mediated liver disease that progresses to fibrosis and cirrhosis if left untreated. In addition to preventing complications, the management of burdensome symptoms, particularly pruritus, represents a key therapeutic goal. Ursodeoxycholic acid (UDCA) is the established first-line treatment; however, up to 40% of patients show an inadequate response and require second-line treatment. Bezafibrate, a pan-peroxisome proliferator–activated receptor (PPAR) agonist, has long been used off-label as an effective option to improve parameters of cholestasis and relieve pruritus. More recently, two new PPAR agonists, elafibranor (PPAR alpha/delta) and seladelpar (PPAR delta), have received conditional approval in Germany. Both demonstrated strong biochemical efficacy and a favorable safety profile in clinical trials. This article provides a comprehensive update on the diagnostics, risk stratification and treatment of PBC, with a particular focus on the role of novel PPAR agonists in second-line treatment. Practical strategies for the treatment of pruritus are also discussed. The aim is to support guideline-based care and to improve the treatment of patients with PBC through timely treatment adjustment and individualized treatment concepts.