Three patients with Sjögren'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 Sjögren's syndrome.
Steinberg AD, Green WT, Talal N. Thrombotic Thrombocytopenic Purpura Complicating Sjögren's Syndrome. JAMA. 1971;215(5):757-761. doi:10.1001/jama.1971.03180180033007