STEROID DEPENDENT ASTHMA, PERSISTENT PANSINUSITIS AND RECURRENT NASAL POLYPOSIS NON-RESPONSIVE TO THERAPY: WHAT IS NEXT?

Abstract

Introduction A 15-year-old white male presented with recalcitrant asthma, chronic rhinosinusitis, and nasal polyps. Despite pharmacotherapy and sinus surgery he was unstable and steroid dependent. Case Description PMHx was unremarkable except for wheezing, nasal polyps, complete nasal blockage and CT-proven pansinusitis. A fumigatus antibodies, PPD and QFT Gold tests were negative. Total eosinophils were 3,700/mm3 and IgE was 1,788 IU/mL. The presence of asthma, eosinophilia, mononeuritis multiplex and paranasal sinus disease, suggested a diagnosis of Churg-Strauss Syndrome [Eosinophilic Granulomatosis Polyangiitis (EGPA)] a multi-systemic, clinically challenging condition because of therapy-associated adverse reactions. Benralizumab, was initiated at 30 mg SQ q 4 weeks X 3 doses then 30 mg q 8 weeks with significant clinical improvement and a dramatic drop in eosinophils to 0/mm3 on day 7. He is currently stabilized on benralizumab and has discontinued OCS. Discussion This case report clearly demonstrates that although the patient presented initially with severe asthma, EGPA was correctly diagnosed. The striking clinical improvement with benralizumab suggests its potential greater beneficial role than other anti-IL5 biologics which achieve eosinopenic effects by neutralizing circulating IL-5. Benralizumab targets IL-5Rα expressed by both mature eosinophils and eosinophil-lineage progenitor cells and accomplishes eosinophil depletion via enhanced antibody-dependent cellular toxicity (ADCC). This novel therapeutic agent merits further evaluation of clinical benefit as a promising therapeutic modality for the treatment of EGPA.