Cryopyrin-associated periodic syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Overview

The cryopyrin-associated periodic syndrome may be classified according to clinical phenotype into three subtypes: familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID)

Classification

  • The cryopyrin-associated periodic syndrome may be classified according to clinical phenotype into three subtypes:[1]
    • Familial cold autoinflammatory syndrome (FCAS)
    • Muckle-Wells syndrome (MWS)
    • Neonatal-onset multisystem inflammatory disease (NOMID)

References

  1. Aksentijevich, Ivona; Nowak, Miroslawa; Mallah, Mustapha; Chae, Jae Jin; Watford, Wendy T.; Hofmann, Sigrun R.; Stein, Leonard; Russo, Ricardo; Goldsmith, Donald; Dent, Peter; Rosenberg, Helene F.; Austin, Frances; Remmers, Elaine F.; Balow, James E.; Rosenzweig, Sergio; Komarow, Hirsh; Shoham, Nitza G.; Wood, Geryl; Jones, Janet; Mangra, Nadira; Carrero, Hector; Adams, Barbara S.; Moore, Terry L.; Schikler, Kenneth; Hoffman, Hal; Lovell, Daniel J.; Lipnick, Robert; Barron, Karyl; O'Shea, John J.; Kastner, Daniel L.; Goldbach-Mansky, Raphaela (2002). "De novoCIAS1 mutations, cytokine activation, and evidence for genetic heterogeneity in patients with neonatal-onset multisystem inflammatory disease (NOMID): A new member of the expanding family of pyrin-associated autoinflammatory diseases". Arthritis & Rheumatism. 46 (12): 3340–3348. doi:10.1002/art.10688. ISSN 0004-3591.

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