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Prevalence of Cryopyrin-Associated Periodic Syndromes (CAPS)

CAPS Patients May Be Suffering Without Diagnosis

CAPS is a group of rare, genetic diseases that include Familial Cold Autoinflammatory Syndrome ( FCAS) and Muckle-Wells Syndrome (MWS).1-3

  • Prevalence of CAPS is estimated at 1 in 1 million2
    • More than 300 patients may suffer from CAPS in the United States
  • CAPS can remain undiagnosed due to2-4:
    • Limited patient and physician awareness of these rare diseases
    • Resemblance of some Symptoms of CAPS to other conditions (eg, allergies, lupus)
  • While symptoms may resemble other illnesses, CAPS symptoms are chronic in nature5

Once diagnosed, effective treatment can help relieve the signs and symptoms of CAPS.

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References:

  • 1. Agostini L, Martinon F, Burns K, McDermott MF, Hawkins PN, Tschopp J. NALP3 forms an IL-1β-processing inflammasome with increased activity in Muckle-Wells autoinflammatory disorder. Immunity. 2004;20:319-325.
  • 2. Toker O, Hashkes PJ. Prevalence of Cryopyrin-Associated Periodic Syndrome (CAPS) Biologics. 2010;4:131-138.
  • 3. Hoffman HM, Mueller JL, Broide DH, Wanderer AA, Kolodner RD. Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle-Wells syndrome. Nat Genet. 2001;29:301-305.
  • 4. Gattorno M, Federici S, Pelagatti MA, et al. Diagnosis and management of autoinflammatory diseases in childhood. J Clin Immunol. 2008;28 (suppl 1):S73-S83.
  • 5. Hoffman HM. Hereditary immunologic disorders caused by pyrin and cryopyrin. Curr Allergy Asthma Rep. 2007;7:323-330.


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