Abstract
A patient with polymyositis (PM) and the one with Sjögren's syndrome (SjS) who both developed thrombotic thrombocytopenic purpura (TTP) were reported. Case 1, a 41-yearold female who had been suffered from PM for five years and interstitial pneumonitis for three years, had been treated with low doses of prednisolone (PSL) and methotrexate. She was hospitalized because of the exacerbation of the pneumonitis. On admission, blood analysis showed prominent thrombocytopenia. Soon after she developed microangiopathic anemia, renal damage and repeated transient ischemic attack of the brain, and diagnosis of TTP was established. The initial treatment with the increased dose of PSL was not effective, but she responded well to the subsequent treatment with the infusion of fresh plasma and antiplatelet agents. Case 2 was a 35-years-old female with SjS who had been treated with a low dose of methylprednisolone for arthritis, fever and digital ulcers. Mild thrombocytopenia was initially recognized three months prior to the admission and gradually progressed. On admission, she was accompanied by renal damage, microangiopathic anemia and hypesthesia on the left leg and diagnosed as TTP. Corticosteroid therapy was not again successful in this case and the combination of fresh plasma and anti-platelet agents were found to be effective.