Mizuno R, Fujimoto S, Fujimoto T, Nishino T, Shiiki H, Hashimoto T, Nakamura S, Dohi K
First Department of Internal Medicine, Nara Medical University.
Intern Med. 2000 Oct;39(10):856-9. doi: 10.2169/internalmedicine.39.856.
Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of antiphospholipid syndrome (APS) characterized by disseminated microangiopathy that results in multiorgan failure. CAPS mainly occurs in association with systemic lupus erythematosus (SLE). Clinically, CAPS mimics disseminated SLE vasculitis, intravascular coagulation (DIC), and particularly thrombotic thrombocytopenic purpura (TTP). We describe an autopsy case of young woman with CAPS in SLE, which is difficult to differentiate from TTP secondary to SLE.
灾难性抗磷脂综合征(CAPS)是抗磷脂综合征(APS)的一种严重变体,其特征为弥漫性微血管病,可导致多器官功能衰竭。CAPS主要与系统性红斑狼疮(SLE)相关。临床上,CAPS类似于弥漫性SLE血管炎、弥散性血管内凝血(DIC),尤其是血栓性血小板减少性紫癜(TTP)。我们描述了一例患有SLE相关CAPS的年轻女性尸检病例,该病例难以与继发于SLE的TTP相鉴别。