Article
Vasculitis as a Major Morbidity Factor in Patients With Partial RAG Deficiency.
Frontiers in immunology - 1 Jan 2020
Geier Christoph B, Farmer Jocelyn R, Foldvari Zsofia, Ujhazi Boglarka, Steininger Jolanda, Sleasman John W, Parikh Suhag, Dilley Meredith A, Pai Sung-Yun, Henderson Lauren, Hazen Melissa, Neven Benedicte, Moshous Despina, Sharapova Svetlana O, Mihailova Snezhina, Yankova Petya, Naumova Elisaveta, Özen Seza, Byram Kevin, Fernandez James, Wolf Hermann M, Eibl Martha M, Notarangelo Luigi D, Calabrese Leonard H, Walter Jolan E
Abstract excerpt
Vasculitis can be a life-threatening complication associated with high mortality and morbidity among patients with primary immunodeficiencies (PIDs), including variants of severe and combined immunodeficiencies ((S)CID). Our understanding of vasculitis in partial defects in recombination activating gene (RAG) deficiency, a prototype of (S)CIDs, is limited with no published systematic evaluation of diagnostic and...
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