Autoimmune Liver Diseases

Two of the classic autoimmune liver diseases, typically taught alongside primary biliary cholangitis as a trio.

Autoimmune hepatitis

Autoimmune chronic active hepatitis. Demographics: young females (10–40 years).

History: progressive fatigue, anorexia, and jaundice. Associated with autoimmune thyroid disease, coeliac disease, and ulcerative colitis.

Examination: jaundice.

Investigations: LFTs: abnormal — raised transaminases plus globulins. Autoantibodies: smooth muscle antibodies positive and/or antinuclear antibodies positive, plus other positive autoantibodies. Typical findings on liver biopsy.

Management: specialist referral. Oral prednisone, monitored according to serum ALT levels; azathioprine or mercaptopurine may be required. ~80% respond, while 20% develop chronic liver disease. If untreated, most patients die within 3–5 years. Provide vaccination against hepatitis A and B.

Primary sclerosing cholangitis

A chronic cholestatic liver disease characterised by inflammation, destruction, fibrosis, and progressive narrowing and saccular dilatation of the bile ducts. Uncommon; typically affects middle-aged patients with inflammatory bowel disease (particularly ulcerative colitis).

History and examination: patients are generally asymptomatic. Progressive jaundice and features of cholestasis — pruritus, fatigue, weight loss, right upper quadrant pain. Examination often reveals nothing to find.

Investigations: LFTs (cholestatic pattern): elevated alkaline phosphatase; elevated ALT (2–3× the normal upper limit); elevated GGT; hypergammaglobulinaemia. Most patients don’t have elevated bilirubin at diagnosis.

Cholangiography is recommended for diagnosis — MRCP is the diagnostic method of choice.

Management: no specific therapy exists — refer to a specialist. Endoscopic dilatation for dominant bile duct strictures; liver transplant may be required. Colonoscopy is recommended given the link with inflammatory bowel disease and increased colorectal cancer risk.