Autoimmune hepatitis — diagnosis and stepwise management

Diagnosis: autoimmune hepatitis, type 1 serology, acute icteric presentation.^[12][18]

The picture is hepatitic (ALT/AST-dominant, ALP only mildly raised). EASL 2025 notes that AIH may affect any patient irrespective of age, sex, or ethnicity and spans asymptomatic disease to acute liver failure. Childhood (and exam) serology still splits type 1 (SMA and/or ANA) from type 2 (anti-LKM1); this patient is type 1.^[12][18]

The simplified diagnostic criteria (Hennes 2008) score autoantibodies, immunoglobulin G, histology, and exclusion of viral hepatitis. In the validation set a cutoff of 6 or more had 88% sensitivity and 97% specificity; 7 or more had 81% sensitivity and 99% specificity. The authors proposed probable AIH at greater than 6 points and definite AIH at 7 or higher. Early diagnosis matters because immunosuppression is life-saving.^[10]

Why not PBC or PSC? Those are cholestatic (ALP-dominant) bile-duct diseases; here transaminases lead. This is a hepatitic disease — AIH.^[10][12]

Stepwise management.^[11][19]

1. Induction. A prospective adult cohort used prednisolone 0.5 to 1 mg/kg/day alone or combined with azathioprine 1 to 2 mg/kg/day (mycophenolate was an alternative first-line in that series). In non-cirrhotic AIH, Manns 2010 showed budesonide 3 mg three times daily (or twice daily) plus azathioprine 1 to 2 mg/kg/day induced complete biochemical remission more often than prednisone 40 mg/day tapered to 10 mg/day plus azathioprine, with fewer steroid-specific side effects — cirrhosis was an exclusion, so do not use budesonide if she is cirrhotic.^[11][19]
2. Maintenance. Treatment usually needs to be maintained for life, as relapses are common after azathioprine cessation. In childhood series, steroids plus azathioprine induce remission in 80%; only some 20% of type 1 patients discontinue therapy successfully.^[18][19]
3. Watch for acute severe AIH. If INR is 1.5 or above without cirrhosis or encephalopathy, that cohort definition of AS-AIH received high-dose corticosteroids; delay beyond 5 days predicted non-response and the need for transplant.^[13]
4. If cirrhosis develops: EASL directs surveillance for portal hypertension and hepatocellular carcinoma, and liver transplantation in decompensated cirrhosis.^[12]