Clinical features and prognosis of Churg-Strauss syndrome

Abstract

We studied the clinical features and prognosis of 11 patients with Churg-Strauss syndrome (CSS). Subjects included 1 man and 10 women with a mean age of 56.3 years. All subjects had a history of bronchial asthma prior to the onset of CSS. It was noted that the age of onset of bronchial asthma was high, and most of the patients had suffered from frequent and severe asthmatic attacks during the periods preceding the vasculitis. Skin tests and IgE RAST revealed that less than half of the subjects were atopic. Therefore it is considered that asthmatics with late onset and severe symptoms have a high risk for CSS, and poor control of asthmatic symptoms may contribute to the onset of the vasculitis. Eleven of 12 cases including 1 patient with a relapse were treated with PSL alone (9 cases) or PSL plus cyclophosphamide (2 cases). Three patients died of heart failure or central nerve involvement. Vasculitis was ameliorated in the other cases (75%). Seven patients received maintenance therapy with low-dose of PSL (5 cases) or cyclophosphamide (2 case). None suffered from relapse of vasculitis during maintenance therapy. Discontinuation of therapy in five cases resulted in a relapse in only one case 62 months after the cessation of low-dose PSL. Although maintenance therapy appears to be useful in some CSS patients, the significance and indications for maintenance therapy should be further clarified.