Thrombotic Thrombocytopenic Purpura Complicating Sjögren's Syndrome

Three patients with Sjogren's syndrome developed fever, central nervous system disease, thrombocytopenia, and bleeding. All died within two weeks. Hyaline thrombi were present in arterioles and capillaries post mortem. To our knowledge, this is the first report of an association between thrombotic thrombocytopenic purpura and Sjogren's syndrome.