Corticosteroid/cyclophosphamide pulse treatment in South African patients with pemphigus

Abstract

Treatment of pemphigus remains a challenge. Corticosteroid/cyclo-phosphamide pulse treatment has been used to reduce the morbidity associated with long-term treatment with high-dose corticosteroids. We describe our experience with pulse treatment in South African patients with pemphigus. To assess, in patients who achieved remission of pemphigus, the number of pulses, time and total amounts of steroid and cyclophosphamide required to achieve remission of pemphigus, any side-effects and the long-term outcome. Patient charts were reviewed retrospectively. In those who had remission of disease (resolution of cutaneous lesions and no new mucosal lesions), details of medication were analysed. The relationships between medication, disease severity, type of disease and patient demographics were investigated. Of the 70 patients, 35 achieved remission as defined in the study criteria; 43% of them with < 6 treatment pulses. Neither the type nor severity of pemphigus correlated with the number of treatment pulses or time to remission. However, 73% of patients who presented with severe disease did receive additional oral corticosteroids between treatment pulses. Lymphopenia occurred in 80% of patients, who needed more treatment pulses (P = 0.002) and thus larger total amounts of oral (P = 0.006) and intravenous (P = 0.02) cyclophosphamide to eventually achieve remission. Of the 35 patients who achieved remission, 94% remained in remission, although seven patients required retreatment to achieve this. Pulse treatment in our setting was associated with minimal morbidity. In the nine patients who relapsed, the regimen was not strictly followed, emphasizing the importance of compliance. Our use of low-dose oral corticosteroids between treatment pulses, early in the management of poorly controlled patients, supports the current modified recommendation of Pasricha.