DISEASE SCANNER
Global Incurable Diseases Tracker
Primary Biliary Cholangitis (PBC)
A chronic autoimmune liver disease characterized by progressive destruction of small intrahepatic bile ducts, leading to cholestasis and cirrhosis. Predominantly affects middle-aged women. Previously called primary biliary cirrhosis.
120.0K
15
Symptoms
Treatment Options
Risk Factors
Diagnostic Methods
- 1Anti-mitochondrial antibodies (AMA)
- 2Liver function tests (elevated ALP)
- 3Liver biopsy (not always needed)
- 4Imaging to exclude obstruction
- 5IgM elevation
Prognosis
Variable. With UDCA response, 10-year transplant-free survival >80%. Non-responders have worse prognosis. Regular monitoring for osteoporosis and thyroid disease.
Prevention
- No primary prevention
- Early diagnosis and treatment
- Regular screening in families
Research Status
Ursodeoxycholic acid (UDCA) first-line, delays progression. Obeticholic acid for UDCA non-responders. Bezafibrate or fenofibrate (PPAR agonists) showing efficacy. Liver transplant for end-stage disease.
Affected Countries
Sources
- https://rarediseases.org
- https://www.ncbi.nlm.nih.gov/books
- https://www.mayoclinic.org/diseases-conditions
- https://rarediseases.org/rare-diseases
Medical Disclaimer
This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.