Developer site: The content shown here is test data.

MyEClinic
MyEClinic
Health Dictionary AI Health Chat Find an Online Doctor
A serene Zen garden with raked sand and smooth stones, symbolising clarity and calm

Health Dictionary

Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

Back to skin conditions

Cryopyrin-associated periodic syndrome history and symptoms

Introduction

Overview

Overview

The cryopyrin-associated periodic syndrome is a spectrum of three different clinical phenotypes with the mildest form being familial cold autoinflammatory syndrome (FCAS), formerly called familial cold urticaria and neonatal-onset multisystem inflammatory disorder (NOMID), also known as chronic infantile neurologic cutaneous and articular (CINCA) as the most severe form. Muckle-Wells syndrome (MWS) is the intermediate form of the disease in terms of severity. Symptoms of the cryopyrin-associated periodic syndrome include episodes of cold-induced fever, skin rash, and joint pain. 

History and Symptoms

History and Symptoms

  • Symptoms of the cryopyrin-associated periodic syndrome include episodes of cold-induced fever, skin rash, and joint pain.[1]
  • The cryopyrin-associated periodic syndrome is a spectrum of three different clinical phenotypes with the following milde to severe order:
    • Familial cold autoinflammatory syndrome (FCAS), formerly called familial cold urticaria
    • Muckle-Wells syndrome (MWS)
    • Neonatal-onset multisystem inflammatory disorder (NOMID), also known as chronic infantile neurologic cutaneous and articular (CINCA)

Common Symptoms

Less Common Symptoms

References

References

  1. ↑ Federici, Silvia; Sormani, Maria Pia; Ozen, Seza; Lachmann, Helen J; Amaryan, Gayane; Woo, Patricia; Koné-Paut, Isabelle; Dewarrat, Natacha; Cantarini, Luca; Insalaco, Antonella; Uziel, Yosef; Rigante, Donato; Quartier, Pierre; Demirkaya, Erkan; Herlin, Troels; Meini, Antonella; Fabio, Giovanna; Kallinich, Tilmann; Martino, Silvana; Butbul, Aviel Yonatan; Olivieri, Alma; Kuemmerle-Deschner, Jasmin; Neven, Benedicte; Simon, Anna; Ozdogan, Huri; Touitou, Isabelle; Frenkel, Joost; Hofer, Michael; Martini, Alberto; Ruperto, Nicolino; Gattorno, Marco (2015). “Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers”. Annals of the Rheumatic Diseases. 74 (5): 799–805. doi:10.1136/annrheumdis-2014-206580. ISSN 0003-4967.
  2. ↑ Hawkins, Philip N.; Lachmann, Helen J.; Aganna, Ebun; McDermott, Michael F. (2004). “Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra”. Arthritis & Rheumatism. 50 (2): 607–612. doi:10.1002/art.20033. ISSN 0004-3591.
  3. ↑ Kuemmerle-Deschner, Jasmin B.; Koitschev, Assen; Ummenhofer, Katharina; Hansmann, Sandra; Plontke, Stefan K.; Koitschev, Christiane; Koetter, Ina; Angermair, Eva; Benseler, Susanne M. (2013). “Hearing loss in Muckle-Wells syndrome”. Arthritis & Rheumatism. 65 (3): 824–831. doi:10.1002/art.37810. ISSN 0004-3591.
  4. ↑ 4.0 4.1 Goldbach-Mansky, Raphaela; Dailey, Natalie J.; Canna, Scott W.; Gelabert, Ana; Jones, Janet; Rubin, Benjamin I.; Kim, H. Jeffrey; Brewer, Carmen; Zalewski, Christopher; Wiggs, Edythe; Hill, Suvimol; Turner, Maria L.; Karp, Barbara I.; Aksentijevich, Ivona; Pucino, Frank; Penzak, Scott R.; Haverkamp, Margje H.; Stein, Leonard; Adams, Barbara S.; Moore, Terry L.; Fuhlbrigge, Robert C.; Shaham, Bracha; Jarvis, James N.; O’Neil, Kathleen; Vehe, Richard K.; Beitz, Laurie O.; Gardner, Gregory; Hannan, William P.; Warren, Robert W.; Horn, William; Cole, Joe L.; Paul, Scott M.; Hawkins, Philip N.; Pham, Tuyet Hang; Snyder, Christopher; Wesley, Robert A.; Hoffmann, Steven C.; Holland, Steven M.; Butman, John A.; Kastner, Daniel L. (2006). “Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1β Inhibition”. New England Journal of Medicine. 355 (6): 581–592. doi:10.1056/NEJMoa055137. ISSN 0028-4793.
  5. ↑ Kastner, D. L. (2005). “Hereditary Periodic Fever Syndromes”. Hematology. 2005 (1): 74–81. doi:10.1182/asheducation-2005.1.74. ISSN 1520-4391.
  6. ↑ Hoffman, Hal M.; Simon, Anna (2009). “Recurrent febrile syndromes—what a rheumatologist needs to know”. Nature Reviews Rheumatology. 5 (5): 249–256. doi:10.1038/nrrheum.2009.40. ISSN 1759-4790.

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