Eosinophilic granulomatosis with polyangiitis developed after dupilumab administration in patients with eosinophilic chronic rhinosinusitis and asthma: a case report

BMC Pulmonary Medicine

Case Report

Akihiko Tanaka¹  Ryo Yanai²  Yuki Maruyama³  Isao Suzaki³  Kojiro Hirano³  Hitome Kobayashi³  Sawa Kamimura³ 

[1]Department of Medicine, Division of Respiratory Medicine and Allergology, School of Medicine, Showa University, Tokyo, Japan

[2]Department of Medicine, Division of Rheumatology, School of Medicine, Showa University, Tokyo, Japan

[3]Department of Otorhinolaryngology, Head and Neck Surgery, School of Medicine, Showa University, 1-5-8 Hatanodai Shinagawa-ku, 142-8666, Tokyo, Japan

关键词: Asthma;    Dupilumab;    Eosinophilic granulomatosis with polyangiitis;    Eosinophilic chronic rhinosinusitis;   

DOI  :  10.1186/s12890-023-02415-6

 received in 2023-02-14, accepted in 2023-04-04,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is a form of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis characterized by eosinophil-rich granulomatous inflammation and small-to-medium vessel vasculitis associated with asthma, rhinosinusitis, and eosinophilia. EGPA is often difficult to distinguish from severe asthma and eosinophilic chronic rhinosinusitis (ECRS) in cases when there are no findings that suggest vasculitis. Dupilumab, an anti-IL-4Rα monoclonal antibody, is expected to be effective in eosinophilic airway inflammatory diseases, such as refractory asthma and chronic rhinosinusitis (CRS). Although transient eosinophilia and eosinophilic pneumoniae have been reported in patients with refractory asthma and CRS associated with dupilumab, few studies have examined the development of EGPA.Case presentationWe report a case of a 61-year-old woman treated with dupilumab for refractory ECRS and eosinophilic otitis media (EOM) complicated by severe asthma. Although she had a previous history of eosinophilic pneumoniae and myeloperoxidase (MPO) ANCA positivity, there were no apparent findings of vasculitis before the initiation of dupilumab. After the second administration of dupilumab, several adverse events developed, including worsening of ECRS, EOM and asthma, and neuropathy. A blood test showed an eosoinophilia and re-elevation of MPO-ANCA levels after the administration of dupilumab. Therefore, dupilumab was discontinued owing to the development of EGPA, and prednisolone and azathioprine administration was initiated for a remission induction therapy.ConclusionTo the best of our knowledge, this is the first case report that suggests that dupilumab may directly trigger the manifestation of vasculitis in patients who were previously MPO-ANCA-positive. Although the precise mechanism of how dupilumab could trigger the development of EGPA requires further elucidation, measuring MPO-ANCA in patients with multiple eosinophilic disorders before the initiation of dupilumab might be helpful when considering the possibility of a latent EGPA. When administering dupilumab to patients with a previous history of MPO-ANCA positivity, clinicians must carefully monitor and collaborate with other specialists in the pertinent fields of study for appropriate usage.

【 授权许可】

CC BY   
© The Author(s) 2023

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