Costedoat-Chalumeau N, Arnaud L, Saadoun D, Chastre J, Leroux G, Cacoub P, Amoura Z, Piette J-C
Service de médecine interne, centre de référence national pour le lupus systémique et le syndrome des antiphospholipides, faculté Paris 6, hôpital Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France.
Rev Med Interne. 2012 Apr;33(4):194-9. doi: 10.1016/j.revmed.2012.01.005. Epub 2012 Feb 16.
The catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition resulting from rapidly progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. Within a few days, the patients develop multiorgan failure with pulmonary distress, renal failure with severe hypertension, cerebral, cardiac, digestive or cutaneous involvement. CAPS develops in less than 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. CAPS may be precipitated by infectious diseases, surgical procedures or discontinuation of anticoagulation. CAPS overall mortality rate has decreased in the past decade and is now around 30%. Within our hospital, it has been reduced to 10%. The main differential diagnoses are other thrombotic microangiopathies, and heparin-induced thrombocytopenia. The treatment of CAPS consists of the empirical association of anticoagulation and corticosteroids, plus plasma exchange or intravenous immunoglobulins. Cyclophosphamide is added in patients with systemic lupus erythematosus. The prevention of CAPS is based upon the adequate management of the perioperative period when surgery cannot be avoided, the prompt treatment of infections and the education of patients with antiphospholipid syndrome.
灾难性抗磷脂综合征（CAPS）是一种危及生命的疾病，由快速进展的广泛血栓形成引起，主要影响存在抗磷脂抗体时的微血管系统。在几天内，患者会出现多器官功能衰竭，伴有肺部窘迫、伴有严重高血压的肾衰竭、脑部、心脏、消化系统或皮肤受累。CAPS在原发性抗磷脂综合征患者或与系统性红斑狼疮相关的抗磷脂综合征患者中发生率不到1%。约50%的病例中，CAPS可揭示抗磷脂综合征。CAPS可能由传染病、外科手术或停用抗凝治疗引发。在过去十年中，CAPS的总体死亡率有所下降，目前约为30%。在我们医院，已降至10%。主要鉴别诊断包括其他血栓性微血管病和肝素诱导的血小板减少症。CAPS的治疗包括经验性联合使用抗凝剂和皮质类固醇，加血浆置换或静脉注射免疫球蛋白。系统性红斑狼疮患者需加用环磷酰胺。CAPS的预防基于在无法避免手术时对围手术期的适当管理、对感染的及时治疗以及对抗磷脂综合征患者的教育。
