An autopsied case of chronic active Epstein-Barr virus infection complicated in systemic lupus erythematosus and antiphospholipid antibody syndrome

• Published in: Japanese Journal of Clinical Immunology Vol. 25; no. 6; pp. 458 - 465
• Main Authors: Koike, Ryuji, Ogawa, Jun, Hagiyama, Hiroyuki, Nishio, Junko, Kasahara, Ichiro, Sugihara, Takahiko, Kohsaka, Hitoshi, Kawachi, Hiroshi, Kubota, Tetsuo, Miyasaka, Nobuyuki
• Format: Journal Article
• Language: English; Japanese
• Published: Japan The Japan Society for Clinical Immunology 2002
• Subjects: Adult; Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - diagnosis; Antiphospholipid Syndrome - pathology; chronic active Epstein-Barr virus infection; Chronic Disease; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - diagnosis; Epstein-Barr Virus Infections - pathology; Fatal Outcome; Female; hemophagocytosis; Hemorrhage - complications; Hemorrhage - diagnosis; Hemorrhage - pathology; Histiocytosis, Non-Langerhans-Cell - complications; Histiocytosis, Non-Langerhans-Cell - diagnosis; Histiocytosis, Non-Langerhans-Cell - pathology; Humans; Lung Diseases - complications; Lung Diseases - diagnosis; Lung Diseases - pathology; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - diagnosis; Lupus Erythematosus, Systemic - pathology; Lupus Nephritis - complications; Lupus Nephritis - diagnosis; Lupus Nephritis - pathology; Pulmonary Embolism - complications; Pulmonary Embolism - diagnosis; Pulmonary Embolism - pathology; pulmonary hemorrhage; systemic lupus erythematosus
• ISSN: 0911-4300; 1349-7413
• DOI: 10.2177/jsci.25.458

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 SS-A. 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 30mg/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.