Cryopyrin-Associated Periodic Syndromes (CAPS) are a group of rare genetic disorders characterized by recurrent episodes of inflammation and related symptoms. CAPS is caused by mutations in the NLRP3 gene, which provides instructions for making a protein called cryopyrin. Cryopyrin is involved in the regulation of the immune system, specifically in the process of inflammation.
There are three main conditions within the CAPS spectrum, which vary in terms of severity:
Symptoms of AiArthritis Diseases Regardless of Diagnosis
“Auto” Symptoms
Reference: Early Symptoms of AiArthritis Study, AiArthritis, 2019.
Typically the joint pain will coincide with one or more “Auto” symptoms and start and stop suddenly - for no apparent reason (which is called a "flare"). Some people will experience all of the above symptoms, others only a few. If you have any of the arthritis features, and at least one of the “Auto” features, please consult your physician about a referral to a rheumatologist.
Symptoms Often Associated with CAPS:
Familial Cold Autoinflammatory Syndrome (FCAS):
Muckle-Wells Syndrome (MWS):
Chronic Infantile Neurological, Cutaneous, and Articular Syndrome (CINCA) or Neonatal-Onset Multisystem Inflammatory Disease (NOMID):
What fellow patients want you to know
Do you have a CAPS syndrome? Let us know what YOU would want those others or those who are not yet diagnosed to know!
There’s no single definitive test that can confirm a CAPS diagnosis, so rheumatologists look at a combination of physical exam, blood and urine tests, genetic testing and imaging (MRI), along with a family history of autoimmune or autoinflammatory disease. For CAPS diagnosis, skin biopsies, lumbar punctures, hearing and eye tests are also performed if further testing is needed.
In an effort to ensure this page has the most accurate and up-to-date information, this page is currently awaiting medical review. Some information is subject to change.
Page Last Updated: 9/1/2023
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