Targeted IL-4Rα blockade ameliorates refractory allergic eosinophilic inflammation in a patient with dysregulated TGF-β signaling due to ERBIN deficiency

H. Richard Droghini,J. Pablo Abonia,Margaret H. Collins,Joshua D. Milner,Jonathan J. Lyons,Alexandra F. Freeman,Vincent A. Mukkada,Kimberly A. Risma,Marc E. Rothenberg,Justin T. Schwartz

The Journal of Allergy and Clinical Immunology: In Practice（2022）

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摘要

Clinical Implications An ERBB2-interacting protein (ERBIN) deficiency causes dysregulated transforming growth factor-beta signaling driving aberrant interleukin-4 (IL- 4)eassociated type 2 immunity. AntieIL-4/IL-13 receptor immunotherapy effectively treated an ERBB2-interacting protein edeficient patient with refractory severe atopy including eosinophilic gastrointestinal disease and may benefit other patients with dysregulated transforming growth factorbeta signalingeassociated atopy.

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