Real-life evidence of low-dose mepolizumab efficacy in EGPA: a case series

Respiratory Research

Giuseppe Quaremba¹  Francescopaolo Granata²  Aikaterini Detoraki²  Antonio Romano³  Ilaria Mormile⁴  Francesca Wanda Rossi⁴  Amato de Paulis⁴  Giuseppe Spadaro⁵  Eugenio Tremante⁶  Remo Poto⁷  Emanuela Morelli⁷ 

[1] Department of Advanced Biomedical Sciences, University of Naples Federico II, Naples, Italy;Department of Internal Medicine, Clinical Immunology, Clinical Pathology and Infectious Diseases, Azienda Ospedaliera Universitaria Federico II, Naples, Italy;Department of Neurosciences, Reproductive and Odontostomatological Sciences, Maxilofacial Surgery Unit, University of Naples Federico II, Naples, Italy;Department of Translational Medical Sciences, University of Naples Ferderico II, Naples, Italy;Department of Translational Medical Sciences, University of Naples Ferderico II, Naples, Italy;Allergy and Clinical Immunology Center for Basic and Clinical Immunology Research (CISI), University of Naples Federico II, Naples, Italy;Division of ENT, Azienda Ospedaliera Dei Colli, Naples, Italy;Post Graduate Program in Clinical Immunology and Allergy, University of Naples Federico II, Naples, Italy;

关键词: Eosinophilic granulomatosis with polyangiitis;    Severe eosinophilic asthma;    Chronic rhinosinusitis;    Nasal polyposis;    Mepolizumab;   

DOI  :  10.1186/s12931-021-01775-z

来源: Springer

【 摘 要 】

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, small vessel, necrotizing vasculitis. The disease is mainly characterized by hypereosinophilia and asthma with frequent sinonasal involvement, although multiple organs can be affected, including the heart, lungs, skin, gastrointestinal tract, kidneys, and nervous system. IL-5 production is pathogenetically central for the development of the disease by promoting proliferation, transvascular migration and functional activation of eosinophils. The degree of blood and tissue eosinophilia appears to be associated with disease pathogenesis and eosinophil depletion represents a promising treatment approach for EGPA. We prospectively evaluated the efficacy and safety of a low dose (100 mg q4w), 12-month course of mepolizumab, an anti-IL-5 monoclonal antibody, in eight patients with severe asthma and active EGPA. Patients were recruited by the tertiary care center of Clinical Immunology and Allergy, University of Naples Federico II. The following outcomes were assessed before (T0), and after 6 (T6) and 12 months (T12) of mepolizumab treatment: Birmingham Vasculitis Activity Score (BVAS), prednisone intake, Sino-Nasal Outcome Test (SNOT-22), Total Endoscopic Polyp Score (TENPS), Asthma Control Test (ACT), Forced Expiratory Volume one second (FEV1)%, blood eosinophilia. BVAS score significantly decreased showing a sharp reduction in disease activity score. Clinical improvements in terms of sinonasal scores and asthma symptoms were observed, in parallel with a drastic drop in eosinophil blood count. Prednisone intake was significantly reduced. In two patients, asthma exacerbations led to discontinuation in mepolizumab therapy after 6 and 12 months despite BVAS reduction. Mepolizumab treatment was well tolerated, and no severe adverse drug effects were registered. In conclusion, our 12-month real-life study suggests that mepolizumab may be beneficial and safe in active EGPA patients by improving disease activity score, sinonasal and asthma outcomes while reducing the burden of prednisone intake.

【 授权许可】

CC BY