Abstracts tagged "DIRA and anti-TNF therapy"

Abstract Number: 798 • 2019 ACR/ARP Annual Meeting
Development of Large Vessel Vasculitis Including Aortitis in a Patient with Deficiency of the IL-1 Receptor Antagonist (DIRA) Points to Converging Roles of IL-1 and TNF in Vascular Pathogenesis Recapitulating Findings from a Murine Model
Gina Montealegre Sanchez¹, Adriana de Jesus ¹, Jenna Wade ², Katherine Townsend ¹, Arianne Soldatos ³, Alessandra Brofferio ⁴, Peter C. Grayson ⁵, Ginger Janow ⁶ and Raphaela Goldbach-Mansky ¹, ¹Translational Autoinflammatory Disease Section/NIAID/NIH, Bethesda, ²Translational Autoinflammatory Diseases Section/NIAID/NIH, Bethesda, ³NIH/NINDS, Bethesda, ⁴NIH/NHLBI, Bethesda, ⁵National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) of the National Institutes of Health, Bethesda, MD, Bethesda, MD, ⁶Joseph M. Sanzari Children's Hospital at Hackensack University Medical Center, NJ
Background/Purpose: Deficiency of interleukin-1-receptor antagonist (DIRA) is a rare autoinflammatory disease caused by autosomal recessive loss of function mutations in IL1RN and characterized by early-onset generalized pustulosis,…

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