Clinical Manifestations
- Fatigue and pruritus
- Jaundice
- Hepatosplenomegaly
Investigations
- ALP always elevated (often very high), ALT may be mildly elevated
- Antimitochondrial antibodies are 95% sensitive
- ANA elevated in about 70%
Diagnosis
- No extrahepatic biliary obstruction, no comorbidities affecting the liver, and at least two of the following three criteria:
- ALP greater than 1.5 times the upper limit of normal
- Antimitochondrial antibody titre of at least 1:40
- Or sp100 or gp210 antibodies, if AMA negative
- Histologic evidence of BC showing nonsuppurative destructive cholangitis and destruction of interlobular bile ducts
Management
