title: Fatal chronic active Epstein-Barr virus infection mimicking autoimmune hepatitis.
abstract: We report a 22-year-old female who presented with pyrexia, pancytopenia and liver dysfunction. The patient showed mild liver dysfunction with low-grade fever and mild hepatosplenomegaly 6 years previously, and autoimmune hepatitis (AIH) was diagnosed based on the examination of the laboratory data and liver biopsy. On admission, both markers of Epstein-Barr virus (EBV) and in-situ hybridisation from a liver biopsy specimen indicated chronic active EBV infection (CAEBV). The patient was administered an immunosuppressive agent and antiviral drug added to steroid therapy, but ultimately died from liver failure and virus-associated haemophagocytosis 10 months after the definite diagnosis. Retrospective examination of the serum at the diagnosis of AIH revealed extremely high titres of antibody to EBV, and EBV-DNA was also detectable by polymerase chain reaction. These results suggest the possibility that the patient may already have suffered from CAEBV at the initial diagnosis. We presume that hepatic involvement of CAEBV should be considered as differential diagnosis in cases showing liver dysfunction with clinical and biochemical features observed in AIH.
authors.affiliation: Department of Medicine and Clinical Oncology, Graduate School of Medicine, Chiba University, Chuo Ward, Chiba, Japan.
authors.forename: Tetsuhiro
authors.initials: T
authors.lastname: Chiba
authors.forename: Shigemasa
authors.initials: S
authors.lastname: Goto
authors.forename: Osamu
authors.initials: O
authors.lastname: Yokosuka
authors.forename: Fumio
authors.initials: F
authors.lastname: Imazeki
authors.forename: Masamichi
authors.initials: M
authors.lastname: Tanaka
authors.forename: Kenichi
authors.initials: K
authors.lastname: Fukai
authors.forename: Yoko
authors.initials: Y
authors.lastname: Takahashi
authors.forename: Hideki
authors.initials: H
authors.lastname: Tsujimura
authors.forename: Hiromitsu
authors.initials: H
authors.lastname: Saisho
mesh_terms: D000328:Adult; D000914:Antibodies, Viral / Q000097:blood; D002908:Chronic Disease; D004279:DNA, Viral / Q000097:blood; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis*; D015616:Histiocytosis, Non-Langerhans-Cell / Q000209:etiology; D006801:Humans; D008099:Liver / Q000473:pathology; D017093:Liver Failure / Q000209:etiology
chemical_list: D000914:Antibodies, Viral; D004279:DNA, Viral