title: A case of catastrophic antiphospholipid antibody syndrome complicated with systemic lupus erythematosus, double positive for anti-cardiolipin/β₂ glycoprotein I and anti-phosphatidylserine/prothrombin autoantibodies.
abstract: A 16-year-old male with severe thrombocytopenia and progressive multiple organ infarctions was diagnosed as having catastrophic antiphospholipid syndrome (CAPS) complicated with systemic lupus erythematosus, and was successfully treated with combination of anticoagulants, corticosteroids, plasma exchange, and intravenous cyclophosphamide. Antibodies to phosphatidylserine/prothrombin (PS/PT) complex and cardiolipin (CL)/β(2)-glycoprotein I (β(2)GPI) were simultaneously detected, indicating that the different pathways of both PS/PT and CL/β(2)GPI might be associated with the radical manifestation of CAPS.
authors: The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu 807-0855, Japan.
authors: Eri
authors: E
authors: Hirakawa
authors: Kazuyoshi
authors: K
authors: Saito
authors: Shintaro
authors: S
authors: Hirata
authors: Tatsuya
authors: T
authors: Atsumi
authors: Takao
authors: T
authors: Koike
authors: Yoshiya
authors: Y
authors: Tanaka
mesh_terms: D000293:Adolescent; D017153:Antibodies, Anticardiolipin / Q000097:blood; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000150:complications* / Q000276:immunology / Q000628:therapy; D015415:Biomarkers / Q000097:blood; D002308:Cardiolipins / Q000276:immunology*; D002388:Catastrophic Illness; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications* / Q000276:immunology / Q000628:therapy; D008297:Male; D010718:Phosphatidylserines / Q000097:blood / Q000276:immunology*; D010951:Plasma Exchange; D011516:Prothrombin / Q000276:immunology* / Q000378:metabolism; D016896:Treatment Outcome; D053482:beta 2-Glycoprotein I / Q000097:blood / Q000276:immunology*
chemical_list: D017153:Antibodies, Anticardiolipin; D000925:Anticoagulants; D015415:Biomarkers; D002308:Cardiolipins; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D010718:Phosphatidylserines; D053482:beta 2-Glycoprotein I; D003520:Cyclophosphamide; D011516:Prothrombin