Boyle Maeve, Mulrennan Siobhain, Morey Sue, Vekaria Sona, Popowicz Natalia, Tai Anna
Department of Respiratory Medicine Sir Charles Gairdner Hospital Perth WA Australia.
The Faculty of Health and Medical Sciences University of Western Australia Perth WA Australia.
Respirol Case Rep. 2020 Dec 2;9(1):e00696. doi: 10.1002/rcr2.696. eCollection 2021 Jan.
Allergic bronchopulmonary aspergillosis (ABPA) is common in cystic fibrosis (CF). Treatment is challenging and the relapse rate is high. Standard therapy is oral steroids and antifungals. However, long-term systemic steroid often results in adverse effects and drug interactions between azoles and CFTR modulators are a potential concern. Mepolizumab, an anti-interleukin (IL)-5 monoclonal antibody, can benefit patients with severe eosinophilic asthma and there are reports of mepolizumab use in ABPA but not in ABPA complicating CF. We present the case of an adult with CF who had recurrent ABPA and intolerable treatment side effects with steroid, azole, and omalizumab. Mepolizumab was well tolerated and led to significantly improved clinical stability and symptomatic improvement. To our knowledge, this is the first report of successful mepolizumab treatment for ABPA in CF. Mepolizumab may be an important adjunctive treatment for difficult to control ABPA in CF.
变应性支气管肺曲霉病（ABPA）在囊性纤维化（CF）中很常见。治疗具有挑战性且复发率高。标准治疗是口服类固醇和抗真菌药物。然而，长期全身使用类固醇常常会导致不良反应，并且唑类药物与CFTR调节剂之间的药物相互作用是一个潜在问题。美泊利单抗是一种抗白细胞介素（IL）-5单克隆抗体，可使重度嗜酸性粒细胞性哮喘患者受益，有关于美泊利单抗用于ABPA的报道，但未用于合并CF的ABPA。我们报告了1例成年CF患者，其ABPA反复发作，且对类固醇、唑类药物和奥马珠单抗的治疗副作用无法耐受。美泊利单抗耐受性良好，显著改善了临床稳定性并缓解了症状。据我们所知，这是美泊利单抗成功治疗CF合并ABPA的首例报告。美泊利单抗可能是CF中难以控制的ABPA的一种重要辅助治疗方法。
