TNF inhibition in vasculitis management in adenosine deaminase 2 deficiency (DADA2).

doi:10.1016/j.jaci.2021.10.030

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Article

TNF inhibition in vasculitis management in adenosine deaminase 2 deficiency (DADA2).

The Journal of allergy and clinical immunology - 1 May 2022

Deuitch Natalie T, Yang Dan, Lee Pui Y, Yu Xiaomin, Moura Natalia Sampaio, Schnappauf Oskar, Ombrello Amanda K, Stone Deborah, Kuehn Hye Sun, Rosenzweig Sergio D, Hoffmann Patrycja, Cudrici Cornelia, Levy Deborah M, Kessler Elizabeth, Soep Jennifer B, Hay Arielle D, Dalrymple Austin, Zhang Yu, Sun Li, Zhang Qiuye, Tang Xuemei, Wu Yuan, Rao Koneti, Li Haibo, Luo Hong, Zhang Yao, Burnham Jon M, Boehm Manfred, Barron Karyl, Kastner Daniel L, Aksentijevich Ivona, Zhou Qing

Abstract excerpt

BACKGROUND: Deficiency of adenosine deaminase 2 (DADA2) is a recessively inherited autoinflammatory disorder caused by a loss of functional ADA2 protein. TNF inhibition (TNFi) has proven to be highly effective in treating inflammatory manifestations. OBJECTIVE: We sought to explore the pathophysiology and the underlying mechanisms of TNF-inhibitor response in these patients. METHODS: We performed Sanger...

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PMID: 34780847
DOI: 10.1016/j.jaci.2021.10.030

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