PDF(Pubmed)
Abstract：
Conventional immunosuppressants are ineffective for the management of EGPA-related asthma. Tezepelumab is a human monoclonal antibody that inhibits thymic stromal lymphopoietin (TLSP) that has proven efficacy in several phase 3 studies for the treatment of asthma. We treated with off-label tezepelumab the first two patients with severe refractory EPGA-related asthma. These preliminary findings suggest that targeting upstream signaling of the T2 inflammatory pathway can improve symptoms, reduce BVAS and increase Asthma Control Test scores, even in patients with refractory asthma who have failed several previous lines of treatment. Nevertheless, by analogy with dupilumab-induced IL-4/13 blockade, the persistence of sputum eosinophilia (reported in both patients) raises questions as to whether TSLP inhibition could lead to a rebound of eosinophilia and potentially to eosinophil-related symptoms in patients with EGPA.
摘要：
常规免疫抑制剂对EGPA相关哮喘的治疗无效。Tezepelumab是一种抑制胸腺基质淋巴细胞生成素（TLSP）的人单克隆抗体，已在几项3期研究中证明了治疗哮喘的有效性。我们对前两名患有严重难治性EPGA相关哮喘的患者进行了超标签tezepelumab治疗。这些初步发现表明，靶向T2炎症途径的上游信号可以改善症状，减少BVAS并增加哮喘控制测试评分，即使是在之前几次治疗失败的难治性哮喘患者中。然而，类似于dupilumab诱导的IL-4/13阻断，痰嗜酸性粒细胞增多的持续存在(2例患者均有报道)引发了以下问题:在EGPA患者中,TSLP抑制是否会导致嗜酸性粒细胞增多反弹,并可能导致嗜酸性粒细胞相关症状.
