Autoimmune Hepatitis in Two Patients with Behçet’s Disease: A Case Report

BACKGROUND: Behçet's disease (BD) is a multisystem inflammatory disorder with variable manifestations. Although hepatic involvement is rare, abnormal liver enzymes may occasionally be observed. We describe two patients with BD who developed autoimmune hepatitis (AIH), confirmed by liver biopsy. CASE PRESENTATION: We report two Iranian patients-one male and one female-diagnosed with BD, who later developed persistently elevated liver enzymes. Both had elevated IgG levels, negative viral hepatitis markers, and liver biopsy findings compatible with autoimmune hepatitis (AIH). Due to limited retrospective serologic data, the diagnosis was established based on multidisciplinary clinicopathological assessment and response to immunosuppressive therapy. Sonography showed fatty liver in both cases. Treatment with azathioprine and prednisolone led to normalization of liver enzymes over a long-term follow-up. CONCLUSIONS: Although uncommon, AIH can co-occur with BD and should be considered in patients with persistently elevated transaminases. Liver biopsy is crucial for definitive diagnosis. Early immunosuppressive therapy can prevent disease progression.