Gatenby P A, Smith H, Kirwan P, Lauer C S
J Rheumatol. 1981 May-Jun;8(3):504-8.
Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) are both multisystem diseases and the latter can manifest hemopoietic abnormalities that may mimic TTP. This has led to diagnostic confusion and reports of the 2 diseases occurring in a single patient. We describe a 15-yr-old girl who presented at age 12 with purpura, fever, headaches, changes in conscious state, thrombocytopenia and microangiopathic hemolytic anemia and who was diagnosed despite the absence of renal disease, as having TTP. ANA and LE cells were negative then, and again 1 yr later. Three yr later she presented with the nephrotic syndrome with a positive ANA (1:100), elevated DNA antibody (79 U/ml, normal less than 25) and a circulating anticoagulant. Renal biopsy confirmed the presence of lupus nephritis. The association is discussed.
血栓性血小板减少性紫癜(TTP)和系统性红斑狼疮(SLE)均为多系统疾病,后者可表现出可能类似TTP的血液系统异常。这导致了诊断上的混淆以及关于这两种疾病在同一患者身上发生的报道。我们描述了一名15岁女孩,她在12岁时出现紫癜、发热、头痛、意识状态改变、血小板减少和微血管病性溶血性贫血,尽管当时没有肾脏疾病,但仍被诊断为TTP。当时抗核抗体(ANA)和狼疮细胞均为阴性,1年后复查亦是如此。3年后,她出现肾病综合征,ANA呈阳性(1:100),DNA抗体升高(79 U/ml,正常低于25),并有循环抗凝物质。肾活检证实存在狼疮性肾炎。本文对这种关联进行了讨论。