Autoimmune post-infantile giant cell hepatitis: a case report and review of the literature

Abstract

Giant cell hepatitis (GCH) is a rare entity in adults that is characterized by large multinucleated hepatocyte formation and parenchymal inflammation. We present a case of acute liver failure in a 33-year-old woman secondary to autoimmune hepatitis (AIH). A liver biopsy revealed submassive hepatocyte necrosis consistent with GCH. We conducted a literature review of 187 reported cases of post-infantile GCH in adults. AIH was the most commonly reported cause of GCH, but GCH was associated with a wide spectrum of etiologies, including infections, rheumatological diseases, hematological diseases, malignancies, and medications. The severity of disease can range from mild hepatitis to fulminant hepatic failure. The mortality rate among the cases in the literature was 18.82%. GCH is managed by treating the underlying cause, and ribavirin has been proposed as a treatment option for idiopathic GCH. A small number of patients progress to requiring orthotopic liver transplant, but recurrence is possible post-transplant.

Postinfantile Giant Cell Hepatitis in Native and Allograft Livers: A Multi-Institutional Clinicopathologic Study of 70 Cases

“…10,13 Also consistent with previous reports, we confirmed that autoimmune liver disease, especially AIH, was the most common etiology associated with PIGCH, accounting for 40% of cases. 1,3,14 Among the 5 PIGCH cases associated with medication, both methamphetamine and Bactrim (trimethoprimsulfamethoxazole) had known liver toxicity; methamphetamine has been previously reported to induce giant cell transformation in hepatocyte. 15,16 .…”

Facing the Unknown: Idiopathic Giant Cell Hepatitis

“…Idiopathic cases, in particular, represent only 25%–30% of 200 reported cases. 1 , 2 GCH is defined by giant, multinucleated cells believed to arise from an abnormal hepatocyte response to various stimuli. Pathophysiology remains poorly understood, although a multitude of medications, viruses, autoimmune conditions, and malignancies have been implicated.…”