Moccia F, Greco G
Dipartimento d'Emergenza, Ospedale S. Martino, Genova.
Clin Ter. 1994 Jul;145(7):65-9.
The antiphospholipid antibody syndrome may develop both in patients with Systemic Lupus Erythematosus and as a primary disease, i.e. in the absence of both clinical manifestations and specific antibodies of Systemic Lupus Erythematosus. The primary syndrome is characterized by one of the following clinical manifestations: recurrent thromboembolic events, spontaneous abortions, immune thrombocytopenia, associated with laboratory signs such as the presence of anticardiolipin antibodies, lupus anticoagulant and/or antiphospholipid antibodies. Thrombosis by antiphospholipid antibodies is due to endothelial damage rather than to vasculitis. Several mechanisms have been postulated to explain the thrombotic effect of these antibodies. Anticoagulants, both dicumarol and heparin, associated with prednisone, are the classic therapy. High doses of immunoglobulins, plasmapheresis, immunoadsorbent plasmapheresis have also been used. Corticosteroids in bolus, possibly associated with cyclophosphamide also in bolus and plasmapheresis can be tried in more severe cases.
抗磷脂抗体综合征可在系统性红斑狼疮患者中发生，也可作为一种原发性疾病出现，即在既无系统性红斑狼疮的临床表现也无特异性抗体的情况下发生。原发性综合征具有以下临床表现之一：复发性血栓栓塞事件、自然流产、免疫性血小板减少症，并伴有实验室指标，如抗心磷脂抗体、狼疮抗凝物和/或抗磷脂抗体的存在。抗磷脂抗体导致的血栓形成是由于内皮损伤而非血管炎。已经提出了几种机制来解释这些抗体的血栓形成作用。双香豆素和肝素这两种抗凝剂与泼尼松联合使用是经典疗法。也使用过高剂量免疫球蛋白、血浆置换、免疫吸附血浆置换。在更严重的病例中，可以尝试大剂量使用皮质类固醇，可能还联合大剂量环磷酰胺和血浆置换。
