Postinfantile giant cell hepatitis with autoimmune features following a human herpesvirus 6-induced adverse drug reaction
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Published on: February 3, 2012
Thomas Kuntzen1, Nicolaus Friedrichs, Hans Peter Fischer
1Department of Internal Medicine I, Rheinische Friedrich-Wilhelms-Universitaet Bonn, Bonn, Germany.
Insights
Giant cell hepatitis (GCH) in adults is rare and often fatal. This case highlights a novel association with HHV6-induced drug reactions and autoimmune features, successfully treated with immunosuppression.
Area of Science:
- Hepatology
- Immunology
- Virology
Background:
- Giant cell hepatitis (GCH) is a rare liver disease, primarily affecting neonates, characterized by multinucleated giant cells in the liver.
- Adult GCH is uncommon, often presents fulminantly, and its etiology is poorly understood, with associations to autoimmune disorders, viral infections, and drug reactions.
- Current therapeutic strategies for GCH remain controversial due to unclear pathogenesis.
Observation:
- A rare case of adult GCH was observed with concurrent autoimmune features.
- The patient experienced GCH following an adverse drug reaction triggered by human herpesvirus 6 (HHV6).
- This specific combination of GCH, HHV6-induced drug reaction, and autoimmune features has not been previously reported.
Findings:
- The patient presented with GCH and autoimmune manifestations.
- A causative link was established between HHV6 infection, an adverse drug reaction, and the subsequent development of GCH.
- High-dose immunosuppressive therapy resulted in significant clinical improvement.
Implications:
- This case expands the known etiological spectrum of adult GCH.
- It suggests a potential role for HHV6 and drug reactions in GCH pathogenesis, particularly in patients with autoimmune predispositions.
- The successful use of immunosuppression offers a potential therapeutic avenue for similar complex GCH cases.
Abstract:
Giant cell hepatitis (GCH) is frequently found in neonates, but rarely in adults. Diagnosis is made on the basis of the presence of hepatocellular multinucleate giant cells. The disease often takes a fulminant course with the development of cirrhosis within months, requiring transplantation or leading to death in a high percentage of cases. The aetiology and pathogenesis are unclear. Association with autoimmune disorders, viral infections and drug reactions, but also with congenital metabolic diseases such as alpha1-antitrypsin deficiency or haemosiderosis has been described. In some cases, no causative event has been found. Therefore, therapeutic options are controversially discussed. We present a patient with GCH with autoimmune features after a human herpesvirus 6 (HHV6)-induced adverse drug reaction, a combination that has not been reported before. High-dose immunosuppression led to dramatic improvements over the past year.
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