Differentiating Primary Sclerosing Cholangitis (PSC), Autoimmune Hepatitis (AIH) and Primary Biliary Cholangitis (PBC) Introduction
PSC, AIH and PBC are immune‑mediated liver diseases that can overlap clinically and biochemically but have different natural histories, complications and long‑term treatments.
In emergency practice the goals are to recognise life‑threatening complications (ascending cholangitis, biliary sepsis, acute liver failure, decompensated cirrhosis), determine the dominant pattern of liver injury, stabilise the patient, and expedite appropriate specialist investigation and referral.
Definitive diagnosis and disease‑specific management are specialist‑led and often require MRCP and/or liver biopsy.
Note: PBC = primary biliary cholangitis (formerly called primary biliary cirrhosis).
Epidemiology and high‑yield associations
- Primary sclerosing cholangitis (PSC)
- More common in men.
- Strong association with inflammatory bowel disease (especially ulcerative colitis); IBD may be asymptomatic.
- Increased lifetime risk of cholangiocarcinoma and colorectal cancer when IBD is present.
- Autoimmune hepatitis (AIH)
- More common in women, often young to middle‑aged, but can occur at any age.
- Frequently associated with other autoimmune diseases.
- Typically responds to immunosuppression.
- Primary biliary cholangitis (PBC)
- Predominantly affects middle‑aged women.
- Associated autoimmune conditions include Sjögren’s syndrome and autoimmune thyroid disease.