Consensus statements of the Hellenic Autoimmune Liver Diseases Study Group on the diagnosis and current management of primary biliary cholangitis - PubMed
4 hours ago
- #Autoimmune liver disease
- #PPAR agonists
- #Primary biliary cholangitis
- Primary biliary cholangitis (PBC) is an autoimmune disease affecting small intrahepatic bile ducts, predominantly in females.
- PBC is characterized by chronic cholestasis, PBC-related autoantibodies, and progressive histological damage.
- Common symptoms include pruritus, fatigue, hyperpigmentation, dry-gland syndrome, xanthelasmas, and concurrent extrahepatic autoimmune diseases, though many patients are asymptomatic at diagnosis.
- Diagnosis relies on antimitochondrial antibodies (AMA) and PBC-specific antinuclear antibodies (anti-gp210, anti-sp100).
- Risk stratification involves demographic, clinical, biochemical, serological, and fibrosis stage assessments.
- First-line treatment is ursodeoxycholic acid (UDCA) at 13-15 mg/kg/day, with response evaluated after 6-12 months using GLOBE or UK-PBC scores.
- Non-responders should receive add-on therapy with PPAR agonists (elafibranor or seladelpar).
- Treatment targets include normal bilirubin (<0.6× ULN) and alkaline phosphatase levels, along with symptom management (pruritus, fatigue, cognitive dysfunction).
- PBC significantly impacts health-related quality of life, necessitating holistic management.