Abstract

We report two cases of Churg-Strauss syndrome. The first case was a 25-year-old woman with a one year history of bronchial asthma, who developed fever, skin eruptions, abdominal pain and mononeuritis multiplex. During treatment with prednisolone 40 mg per day, chest X-ray films showed bilateral wide-spread infiltrates. When the dosage of prednisolone was increased to 80 mg per day, these infiltrates disappeared. Skin and lung biopsy specimens demonstrated allergic vasculitis and eosinophilic pneumonia. There was no response to high-dose methylprednisolone pulse therapy for persistent severe abdominal pain and mononeuritis multiplex. Pericardial and pleural effusions with eosinophilia recurred eight months later. The second case was a 31-year-old man with a six year history of bronchial asthma, who developed fever, skin eruptions, myalgia and mononeuritis multiplex. One year later, during treatment with prednisolone 15 mg per day, bronchial asthma with eosinophilia relapsed and chest X-ray films showed bilateral patchy infiltrates. Skin biopsy specimens demonstrated eosinophilic infiltrates and necrotizing vasculitis, while lung biopsy specimens demonstrated eosinophilic infiltrates and small granulomas. With additional administration of cyclophosphamide, he has had no evidence of active disease for six years. In both cases, the neurological symptoms persisted despite treatment with high doses of steroids, and during tapering of prednisolone, vasculitis syndrome relapsed. Therefore, long-term careful surveillance is necessary in this disease.

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