Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain.
Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Mútua de Terrassa, Terrassa, Catalonia, Spain.
J Autoimmun. 2018 Aug;92:1-11. doi: 10.1016/j.jaut.2018.05.007. Epub 2018 May 18.
The catastrophic antiphospholipid syndrome (CAPS) is a life-threating variant of the antiphospholipid syndrome characterized by the development of multiple thrombosis in a short period of time, usually ending up in the failure of function of several vital organs. Most CAPS episodes are related to a prothrombotic situation or precipitating factor such as infections, surgical procedures or malignant diseases. In patients with CAPS, the development of multiple thrombosis leads to an important cytokine release that worsens the already critical patient's situation. The disease usually involves the kidneys, the lungs and the heart, although any organ system can be affected. Although occasionally the disease affects large vessels, in the majority of cases it affects small vessels, leading to a disseminated microangiopathic syndrome resembling thrombotic thrombocytopenic purpura. Treatment is based on the administration of anticoagulants, corticosteroids, plasma exchange and/or intravenous immunoglobulins. Cyclophosphamide is recommended in those CAPS cases associated to systemic lupus erythematosus. Additionally, rituximab and eculizumab have been used in refractory cases. Mortality is still around 30% despite current treatment.
灾难性抗磷脂综合征（CAPS）是一种危及生命的抗磷脂综合征变体，其特征是在短时间内发生多种血栓形成，通常导致多个重要器官功能衰竭。大多数 CAPS 发作与促血栓形成情况或促发因素有关，如感染、手术或恶性疾病。在 CAPS 患者中，多种血栓形成的发展导致重要细胞因子的释放，从而使已经处于危急状态的患者病情恶化。该疾病通常涉及肾脏、肺部和心脏，尽管任何器官系统都可能受到影响。尽管该疾病偶尔会影响大血管，但在大多数情况下，它会影响小血管，导致类似于血栓性血小板减少性紫癜的弥散性微血管病综合征。治疗基于抗凝剂、皮质类固醇、血浆置换和/或静脉注射免疫球蛋白的给药。对于与系统性红斑狼疮相关的 CAPS 病例，建议使用环磷酰胺。此外，在难治性病例中已使用利妥昔单抗和依库珠单抗。尽管目前有治疗方法，但死亡率仍约为 30%。
