Eosinophilic polyangiitisgranulomatosa (EGPA) is a vasculitis syndrome that affects small and medium-sized blood vessels throughout the body. The mainstay of treatment is steroids, but there are no standardized doses or durations for steroids in combination with mepolizumab.
We investigated the efficacy, steroid administration, and course of steroid reduction in five patients who were diagnosed with EGPA and treated with mepolizumab for 1 year. Five patients who were diagnosed with EGPA using the American College of Rheumatology criteria and treated with mepolizumab for 1 year were included in our study from 2018–2020.
Eosinophil levels as well as the Birmingham Vasculitis Activity Score (BVAS), remission rate, annual relapse rate, and steroid dosage were observed at 1 year after mepolizumab treatment. Fifty-two weeks after mepolizumab treatment, eosinophils, BVAS, and the steroid dosage showed a trend toward improvement in four out of five patients. In the one patient who received no dose reduction, there was no exacerbation of bronchial asthma that had previously occurred during the follow-up period. Although neurological symptoms often remain even in remission, all patients with neurological symptoms at diagnosis improved. There were no cases of relapse in this study, including two anti-neutrophil cytoplasmic antibody-positive cases.
Management that includes early administration of mepolizumab may improve patients’ quality of life by alleviating lingering neurological symptoms that are caused by EGPA.