[A case of MPO-ANCA positive progressive systemic sclerosis with status epileptics].

A 37-year-old female, who had been suffering from progressive systemic sclerosis (PSS) with long-term renal failure, was diagnosed as MPO-ANCA positive PSS in active stage. She had systemic scleroderma, normotensive renal failure and pulmonary fibrosis. Respiratory management and steroid therapy under the control of CVVH were done for her treatment. She developed hemolytic anemia and thrombocytopenia during the treatment. She was diagnosed as microangiopathic hemolytic anemia, and was treated with plasma exchange treatment. About eight weeks after her admission, she developed status epileptics. Cerebral computed tomography and lumbar puncture did not reveal any abnormal sign. The case reported herein is a MPO-ANCA positive PSS with normotensive renal failure. Generally, PSS rarely reveals neurological sign. This case suggests possibility of association between MPO-ANCA and systemic vasculitis in PSS.