Nayer Ali, Ortega Luis M
Division of Nephrology and Hypertension, University of Miami, Miami, FL, USA.
Division of Nephrology and Hypertension, Allegheny General Hospital, Temple University School of Medicine, Pittsburg, PA, USA.
J Nephropathol. 2014 Jan;3(1):9-17. doi: 10.12860/jnp.2014.03. Epub 2014 Jan 1.
Catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening autoimmune disease characterized by disseminated intravascular thrombosis resulting in multiorgan failure.
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CAPS is due to antiphospholipid antibodies directed against a heterogeneous group of proteins that are associated with phospholipids. These autoantibodies activate endothelial cells, platelets, and immune cells, thereby promoting a proinflammatory and prothrombotic phenotype. Furthermore, antiphospholipid antibodies inhibit anticoagulants, impair fibrinolysis, and activate complements. Although CAPS can affect a variety of organs and tissues, the kidneys, lungs, central nervous system, heart, skin, liver, and gastrointestinal tract are most commonly affected. The systemic inflammatory response syndrome, likely to extensive tissue damage, accompanies CAPS. The most frequent renal manifestations are hypertension, proteinuria, hematuria, and acute renal failure.In the majority of patients with CAPS, a precipitating factor such as infection, surgery, or medication can be identified. Antiphospholipid antibodies such as lupus anticoagulant and antibodies against cardiolipin, β2-glycoprotein I, and prothrombin are serological hallmark of CAPS. Laboratory tests often reveal antinuclear antibodies, thrombocytopenia, and anemia. Despite widespread intravascular coagulation, blood films reveal only a small number of schistocytes. In addition, severe thrombocytopenia is uncommon.
Histologically, CAPS is characterized by acute thrombotic microangiopathy. CAPS must be distinguished from other forms of thrombotic microangiopathies such as hemolytic-uremic syndrome, thrombotic thrombocytopenic purpura, disseminated intravascular coagulation, and heparin-induced thrombocyt openia. CAPS is associated with high morbidity and mortality. Therefore, an aggressive multidisciplinary treatment strategy is indicated. Anticoagulation, immunosuppression, plasma exchange, intravenous immunoglobulins, and anti-platelet agents, used in various combinations, have resulted in improved patient outcome.
灾难性抗磷脂综合征（CAPS）是一种罕见的危及生命的自身免疫性疾病，其特征为弥漫性血管内血栓形成，导致多器官功能衰竭。
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CAPS是由针对与磷脂相关的一组异质性蛋白质的抗磷脂抗体引起的。这些自身抗体激活内皮细胞、血小板和免疫细胞，从而促进促炎和促血栓形成表型。此外，抗磷脂抗体抑制抗凝剂、损害纤维蛋白溶解并激活补体。虽然CAPS可累及多种器官和组织，但肾脏、肺、中枢神经系统、心脏、皮肤、肝脏和胃肠道最常受累。CAPS伴有全身炎症反应综合征，可能导致广泛的组织损伤。最常见的肾脏表现为高血压、蛋白尿、血尿和急性肾衰竭。在大多数CAPS患者中，可识别出感染、手术或药物等诱发因素。狼疮抗凝物以及抗心磷脂、β2糖蛋白I和凝血酶原的抗体等抗磷脂抗体是CAPS的血清学标志。实验室检查常显示抗核抗体、血小板减少和贫血。尽管存在广泛的血管内凝血，但血涂片仅显示少量裂体细胞。此外，严重血小板减少并不常见。
组织学上，CAPS的特征为急性血栓性微血管病。CAPS必须与其他形式的血栓性微血管病如溶血尿毒综合征、血栓性血小板减少性紫癜、弥漫性血管内凝血和肝素诱导的血小板减少症相鉴别。CAPS与高发病率和死亡率相关。因此，需要积极的多学科治疗策略。抗凝、免疫抑制、血浆置换、静脉注射免疫球蛋白和抗血小板药物联合使用，已改善了患者的预后。
