Churg-Strauss Syndrom: Necessity Points in the Diagnosis and Treatment
The Second Department of Internal Medicine, Nippon Medical School
A 32-year-old man with a history of adult-onset asthma and a high level of P-ANCA and eosinophilia was referred to our hospital because of renal insufficiency. Renal and skin biopsy specimens confirmed a diagnosis of Churg-Strauss Syndrome (CSS). Treatment with steroid therapy alone including methylprednisolone pulse therapy was unsatisfactory, so additional treatment with cyclophosphamide was started. Three days later, he presented with convulsions and consciousness disturbance. MRI of the brain demonstrated bilateral white-matter abnormality that was characteristic of reversible posterior encephalophathy syndrome (RPES). Since the activity of CSS was still high, immunosuppressive therapy was continued combined with antihypertensive therapy. His consciousness was soon recovered and MRI findings improved later. RPES is an uncommon complication of CSS. In this case RPES might have been associated with CNS vasculitis.
ϊγεγο 2005; 1(1), 21-25
Churg-Strauss Syndrome, RPES, cyclophosphamide, CNS vasculitis
Kae Ueda, Department of Internal Medicine (II), Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan