Zar T, Kaplan A A
Department of Medicine, Division of Nephrology, University of Connecticut Health Center, Farmington, CT 06030, USA.
Clin Nephrol. 2008 Jul;70(1):77-81. doi: 10.5414/cnp70077.
Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare life-threatening variant of antiphospholipid antibody syndrome (APS), with an associated mortality rate of > 50%. Treatment recommendations are aggressive and consist of intravenous heparin, steroids, immunoglobulins and/or therapeutic plasma exchange (TPE). At present, insufficient data exist to make precise recommendations regarding the most effective therapy for CAPS. Accumulating evidence over recent years is encouraging and may lead to future guidelines. We report predictive and effective removal of pathological anticardiolipin antibody (aCL AB) in a patient with CAPS. The case report and discussion provide valuable insight into aCL AB production and its removal by first- order kinetics using TPE.
灾难性抗磷脂抗体综合征（CAPS）是抗磷脂抗体综合征（APS）一种罕见的危及生命的变异型，相关死亡率>50%。治疗建议积极，包括静脉注射肝素、类固醇、免疫球蛋白和/或治疗性血浆置换（TPE）。目前，尚无足够数据就CAPS最有效的治疗方法给出精确建议。近年来不断积累的证据令人鼓舞，可能会催生未来的指南。我们报告了1例CAPS患者中病理性抗心磷脂抗体（aCL AB）的预测性和有效清除情况。该病例报告及讨论为aCL AB的产生及其通过TPE一级动力学清除提供了有价值的见解。
