A Case of Post Infantile Giant Cell Hepatitis

Abstract

Introduction: Post infantile giant cell hepatitis (PGCH) is a rare entity. It is purely a histomorphological diagnosis and does not establish the etiology. It has been associated mostly with autoimmune liver diseases but various viruses, drugs, post-transplant conditions and other causes have been reported. The natural course is usually a fulminant, progressive course to cirrhosis within months, leading to death or a requirement for transplantation. We present an interesting case of giant cell hepatitis in an adult patient. Case Presentation: A 57-year-old female with a past history of “hepatitis” presented for evaluation of worsening liver function. She reports being told that she had hepatitis at the time of cholecystectomy 10 years prior and no treatment or follow up was needed. On presentation, she was noted to have a mixed pattern hepatitis with predominant cholestatic features as a work up prior to antifungal therapy for toe nail fungal-infection. Subsequent testing revealed a mildly prolonged INR and thrombocytopenia. Work up for etiologies of chronic liver disease was relevant for a mildly increased ANA titer (1:40). Liver biopsy showed post-infantile giant cell hepatitis and cirrhosis. Steroids were initiated for presumed autoimmune hepatitis (AIH) and due to a rapid deterioration of liver function, liver transplant evaluation was recommended and steroids discontinued. While awaiting liver transplantation, she developed new onset muscle weakness and neuropathy diagnosed as Guillain-Barré syndrome (GBS). Rare viral etiologies were excluded and she was successfully treated with IVIG. After recovery from her GBS she was successfully transplanted with normalization of her liver function. Explant confirmed the diagnosis of post-infantile giant cell hepatitis induced cirrhosis. Discussion: Adult or post-infantile giant-cell hepatitis has been shown to be progressive and often fatal disease process, with a survival rate of only approximately 50% without orthotopic liver transplantation. Our case emphasizes the progressive nature of the disease and the need for prompt evaluation and treatment.