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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.<\/jats:p>","DOI":"10.1186\/s12931-024-02888-x","type":"journal-article","created":{"date-parts":[[2024,7,11]],"date-time":"2024-07-11T15:02:36Z","timestamp":1720710156000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Tezepelumab for refractory eosinophilic granulomatosis with polyangiitis-related asthma"],"prefix":"10.1186","volume":"25","author":[{"given":"Nina","family":"Vincent-Galti\u00e9","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Quentin","family":"Marquant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emilie","family":"Catherinot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"Ackermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antoine","family":"Magnan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colas","family":"Tcherakian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthieu","family":"Groh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,7,11]]},"reference":[{"key":"2888_CR1","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.autrev.2016.09.018","volume":"16","author":"V Cottin","year":"2017","unstructured":"Cottin V, Bel E, Bottero P, Dalhoff K, Humbert M, Lazor R, et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun rev. 2017;16:1\u20139.","journal-title":"Autoimmun rev"},{"key":"2888_CR2","doi-asserted-by":"publisher","first-page":"2175","DOI":"10.1002\/art.40205","volume":"69","author":"X Pu\u00e9chal","year":"2017","unstructured":"Pu\u00e9chal X, Pagnoux C, Baron G, Qu\u00e9meneur T, N\u00e9el A, Agard C, et al. Adding azathioprine to remission-induction glucocorticoids for eosinophilic granulomatosis with polyangiitis (Churg\u2010Strauss), microscopic polyangiitis, or polyarteritis nodosa without poor prognosis factors: a randomized, controlled trial. Arthritis Rheumatol. 2017;69:2175\u201386.","journal-title":"Arthritis Rheumatol"},{"key":"2888_CR3","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1016\/j.jaut.2013.03.003","volume":"43","author":"M Samson","year":"2013","unstructured":"Samson M, Pu\u00e9chal X, Devilliers H, Ribi C, Cohen P, Stern M, et al. 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EC: consulting fees from AstraZeneca and Sanofi.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"272"}}