Kuntzen Thomas, Friedrichs Nicolaus, Fischer Hans Peter, Eis-Hübinger Anna Maria, Sauerbruch Tilman, Spengler Ulrich
Department of Internal Medicine I, Rheinische Friedrich-Wilhelms-Universitaet Bonn, Bonn, Germany.
Eur J Gastroenterol Hepatol. 2005 Oct;17(10):1131-4. doi: 10.1097/00042737-200510000-00020.
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.