2025 Volume 66 Issue 6 Pages 249-256
A man in his 80s with bronchial asthma and hypereosinophilic syndrome had been on steroid therapy (prednisolone 5 mg daily) for about 7 years. He was referred to our hospital 2 months after developing hepatitis while still on therapy. Severe jaundice developed 3 months later. A liver biopsy revealed chronic active hepatitis with marked lymphocyte and plasma cell infiltration in the portal area and lobular remodeling. Based on elevated serum immunoglobulin G and positivity for anti-LKM-1 antibodies, this patient was diagnosed with type 2 autoimmune hepatitis. Despite an increase in the steroid dose, the disease rapidly progressed to cirrhosis, and complications including infections and pulmonary edema set in. The patient died one year after disease onset. The clinical features of type 2 autoimmune hepatitis in Japan remain unclear, but the disease can sometimes progress quickly. In cases of unexplained liver dysfunction, autoimmune hepatitis must be considered in the differential diagnosis, even if the patient is an older, man and on immunosuppressive therapy.
