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Primary Biliary Cholangitis
Francesca Bolis1, Marco Carbone1
1Department of Medicine and Surgery, Centre for Autoimmune Liver Diseases, University of Milano-Bicocca, Via Cadore 48, 20900 Monza, Italy; Liver Unit, ASST Grande Ospedale Metropolitano Niguarda, Piazza Ospedale Maggiore 3, 20162 Milan, Italy.
Abstract:
Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease characterized by persistent cholestasis and progressive fibrosis. Diagnosis relies on cholestatic biochemistry with AMA or PBC-specific antinuclear antibodies positivity; biopsy is reserved for atypical cases. Ursodeoxycholic acid (UDCA) remains first-line, with on treatment biochemical response predicting long-term prognosis. Symptom management-particularly for pruritus, fatigue, and sicca-is crucial to address patient's quality of life. Long-term care includes surveillance for treatment response, development of fibrosis, portal hypertension, bone disease, and hepatocellular carcinoma. Liver transplantation (LT) remains definitive for end-stage or refractory disease, with post-LT UDCA recommended to reduce its recurrence.
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