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Rheumatology · Rheumatology / Autoinflammatory disease

Cryopyrin-associated periodic syndrome

also: CAPS; familial cold autoinflammatory syndrome; Muckle–Wells syndrome; NOMID/CINCA

Spectrum of NLRP3 inflammasomopathies with urticaria-like rash, fever and sensorineural hearing loss.

Specialty
Rheumatology
Subspecialty
Rheumatology / Autoinflammatory disease
Sex & age distribution
Male: affected; Female: affected equally; Children: yes, infancy
Gene(s) involved
NLRP3
Alternative names & eponyms
CAPS; familial cold autoinflammatory syndrome; Muckle–Wells syndrome; NOMID/CINCA

Etiology & mechanism

Gain-of-function NLRP3 variants cause IL-1 overproduction.

Gene(s) involved (1)

Symptoms & signs (8)

In plain terms, someone with Cryopyrin-associated periodic syndrome may experience the following — click any to explore related syndromes:

Same gene(s)

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Overlapping symptoms

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