Cryopyrin-associated periodic syndrome history and symptoms

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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

• Cryopyrin-associated periodic syndrome is a spectrum of three different clinical phenotypes with the following order from mild to severe:
  • 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 of the familial cold autoinflammatory syndrome include cold-induced episodes of fever, joint pain, skin rash, and erythema of the eye.
• Common symptoms of Muckle-Wells syndrome (MWS) include episodes of fever, joint pain, headache, and progressive hearing loss.
• Common symptoms of Neonatal-onset multisystem inflammatory disorder (NOMID) include urticarial skin rash, hearing loss, limb, and joint pain.

History and Symptoms

• Symptoms of the cryopyrin-associated periodic syndrome include episodes of cold-induced fever, skin rash, and joint pain. 

Common Symptoms

• Common symptoms of the familial cold autoinflammatory syndrome (FCAS) include:
  • Cold-induced episodes of fever
  • Joint pain
  • Skin rash
  • Erythema of the eye
• Common symptoms of Muckle-Wells syndrome (MWS) include:
  • Episodes of fever
  • Joint pain
  • Headache
  • Progressive hearing loss
• Common symptoms of Neonatal-onset multisystem inflammatory disorder (NOMID) include:
  • Urticarial skin rash
  • Hearing loss
  • Limb, and joint pain

Less Common Symptoms

• Less common symptoms of the familial cold autoinflammatory syndrome (FCAS) include:
  • Fatigue
  • Headache
• Less common symptoms of Muckle-Wells syndrome (MWS) include:

• Less common symptoms of Neonatal-onset multisystem inflammatory disorder (NOMID) include: