Thrombotic Thrombocytopenic Purpura and Systemic Lupus Erythematosus

Abstract

In 1924 Eli Moschcowitz⁸⁹described an acute, febrile, rapidly fatal illness characterized by hyaline thrombi in the terminal arterioles—a syndrome later termed thrombotic thrombocytopenic purpura (TTP).¹²¹Subsequently several investigators noted that TTP was occasionally associated with systemic lupus erythematosus (SLE)^12,52,119; however, most authors have denied the existence of any clinical or pathologic relationship between these entities.^{5,8,41,45,54,79,121,136} Changes compatible with SLE were found at autopsy in two of three patients who died recently of TTP in this hospital. The two cases with features of both conditions are herein reported in detail. In view of the absence in the literature of any study establishing the frequency of this association, published reports of TTP were analyzed for evidence of SLE. Included in the analysis were only those published cases of TTP in which the characteristic pathologic features described by Moschcowitz⁸⁹were found at necropsy. In 23% of the