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February 1, 1971

Thrombotic Thrombocytopenic Purpura Complicating Sjögren's Syndrome

Author Affiliations

From the Arthritis and Rheumatism Branch (Dr. Steinberg and Dr. Talal) and the Section on Rheumatic Disease, Laboratory of Experimental Pathology (Dr. Green), National Institute of Arthritis and Metabolic Diseases, Bethesda, Md. Dr. Green is now with Children's Hospital, Pittsburgh.

JAMA. 1971;215(5):757-761. doi:10.1001/jama.1971.03180180033007

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.