title: [An autopsied case of chronic active Epstein-Barr virus infection complicated in systemic lupus erythematosus and antiphospholipid antibody syndrome].
abstract: We have experienced a case of chronic active Epstein-Barr virus infection (CAEBV) complicated in systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS). A 35-year-old woman was admitted to our hospital with complaints of fever and dyspnea on exertion. She was diagnosed as having SLE on the basis of arthritis, oropharyngeal ulcer, lymphopenia, and positive autoantibodies against DNA, RNP and SSA. The diagnosis of APS was also made because of positive anti-cardiolipin IgG antibodies and the existence of multiple pulmonary infarction with pulmonary hypertension. The administration of 30 mg/day of prednisolone and anti-coagulation significantly improved clinical symptoms. However, she was again admitted to the hospital four months later because of progressive liver damage and pancytopenia. Increment of prednisolone did not improve the clinical situation and she expired because of pulmonary hemorrhage. At autopsy, there were a significant increase of histiocytes with hemophagocytosis and a dense infiltration of atypical lymphocytes in the liver, spleen, lymph nodes and bone marrow. Infiltrated lymphocytes were positive for CD 3 and EBER 1 in immunohistochemical staining and EBVmRNA was detected by in situ hybridization. Final pathological diagnosis was CAEBV with hemophagocytic syndrome in association with lupus nephritis, pulmonary hemorrhage and pulmonary infarction.
authors.affiliation: Department of Bioregulatory Medicine and Rheumatology, Graduate School, Tokyo Medical & Dental University.
authors.forename: Jun
authors.initials: J
authors.lastname: Ogawa
authors.forename: Ryuji
authors.initials: R
authors.lastname: Koike
authors.forename: Takahiko
authors.initials: T
authors.lastname: Sugihara
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Hagiyama
authors.forename: Junko
authors.initials: J
authors.lastname: Nishio
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Kohsaka
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Kubota
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kawachi
authors.forename: Ichiro
authors.initials: I
authors.lastname: Kasahara
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Miyasaka
mesh_terms: D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D002908:Chronic Disease; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006470:Hemorrhage / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D008171:Lung Diseases / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D008181:Lupus Nephritis / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D011655:Pulmonary Embolism / Q000150:complications / Q000175:diagnosis / Q000473:pathology