Overview
Definition
A chronic, progressive autoimmune liver disease characterized by the destruction of the small intrahepatic bile ducts, leading to portal inflammation, cholestasis, and cirrhosis.
Epidemiology
Affects primarily women (9:1 female-to-male ratio) between 35-60 years of age.
Etiology & Risk Factors
- Autoimmune mediated destruction of cholangiocytes
- Strong genetic association and environmental triggers
Clinical Symptoms
- Fatigue
- Pruritus (intense itching, often worse at night)
- Hyperpigmentation
- Xanthelasma / Xanthomas (due to hypercholesterolemia)
- Dry eyes/mouth (Sjögren's association)
Clinical Approach
Diagnosis
- Elevated Alkaline Phosphatase (ALP) and GGT
- Positive Anti-Mitochondrial Antibodies (AMA) in >95% of cases
- Liver biopsy showing florid duct lesions if AMA is negative
Management
- Ursodeoxycholic acid (UDCA) to slow progression
- Obeticholic acid for non-responders
- Cholestyramine or Rifampin for pruritus
- Liver transplantation for end-stage disease
Complications
- Liver cirrhosis / Portal Hypertension
- Osteoporosis
- Malabsorption of fat-soluble vitamins (A, D, E, K)
- Hepatocellular Carcinoma