Benralizumab substitution monotherapy in symptomatic relapsing asthma-dominant eosinophilic granulomatosis with polyangiitis

Abstract

There is increasing interest in biologicals in steroid-resistant or steroid-dependent eosinophilic granulomatosis with polyangiitis (EGPA). We describe successful benralizumab monotherapy in asthma-dominant EGPA. Sixteen years ago, a 38-year-old male was diagnosed with multisystemic illness with asthma, arthralgias, Bell’s palsy and nephrotic syndrome, with 20.3% eosinophilia (absolute count 1160 K/µL), ESR 50 mm/hour, albumin 2.3 g/dL, serum creatinine 1.1 mg/dL, slightly above baseline and negative immunology work. Kidney biopsy demonstrated diffuse podocyte foot process effacement with minimal change disease, without vasculitis and EGPA was diagnosed. He achieved early remission with corticosteroids and intravenous cyclophosphamide and was maintained on prednisone and mycophenolate mofetil. Nevertheless, he repeatedly experienced severe asthma exacerbations, falls in FEV-1% and recurrent relapsing eosinophilia. He was successfully started on benralizumab and was weaned off mycophenolate mofetil and prednisone. Eosinophilia was corrected and FEV-1% normalized. We support calls for larger trials of the biologicals in EGPA.