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| Learn-Autoinflammatory Syndromes | | Cryopyrin-Associated Periodic Syndromes are Autoinflammatory Diseases |
Autoinflammatory diseases are caused by genetic mutations in molecules that are involved in regulating the innate immune response-a "hard wired" defense system that evolved to quickly recognize and act against infectious agents and other danger signals produced by our bodies.
It is important not to confuse autoinflammatory syndromes with autoimmune diseases, such as: Lupus, Rheumatoid Arthritis and others that are caused by the body's adaptive immune system developing antibodies to antigens that then attack healthy body tissues.
For more information about autoinflammatory diseases and the innate immune system, please visit:
NIAMS Autoinflammatory Disease Information
Cryopyrin-Associated Periodic Syndromes (CAPS) are members of a growing family of autoinflammatory diseases, which were originally referred to as Hereditary Periodic Fever Syndromes. To learn about other autoinflammatory diseases besides CAPS, please choose a syndrome on the list to the left. |
| THE THREE KNOWN FORMS OF CAPS ARE: |
- Familial Cold Urticaria Syndrome (FCAS) also known as Familial Cold Urticaria (FCU) or Familial Cold Urticaria Syndrome (FCUS)
- Muckle-Wells Syndrome (MWS)
- Neonatal-Onset Multisystem Inflammatory Disease (NOMID), also known as Chronic Infantile Neurological Cutaneous Articular Syndrome (CINCA)
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| Common Symptoms Present in All CAPS Disorders: |
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Rash |
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Headaches |
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Periodic Fevers |
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General Malaise |
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Joint Pain |
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Conjunctivitis |
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Varying Severity of Symptoms within CAPS |
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Before the genetic mutation for CAPS was discovered, FCAS, MWS and NOMID/CINCA were considered to be different and unrelated periodic fever syndromes. In the 21st century, genetic mutations in the NLRP3 (CIAS1) gene were found to be present in most patients with these syndromes. Now, these three syndromes are believed to represent varying degrees of chronic inflammation caused by the same condition (CAPS) within the NLRP3 genetic mutation region. If you consider all the syndromes of CAPS as the same disorder, with FCAS at the low end of severity, and NOMID/CINCA at the high end, Muckle-Wells falls in the middle region on this disease spectrum. Many patients have overlapping symptoms along the CAPS inflammation spectrum between each classification of FCAS, MWS and NOMID. It is important to look at the classic findings for each of these forms of CAPS to understand how these syndromes are very similar, yet unique.
The most common findings between all the CAPS syndromes are the rash, fevers and inflammatory symptoms present at birth or in early infancy. Other common symptoms include: Flares of increased symptoms accompanying the fevers, rash, joint pain, conjunctivitis, headaches, and general malaise. Almost all patients with FCAS, many MWS and some NOMID patients note that their symptoms can increase after exposure to cold or cooling temperatures.
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Video about CAPS
This is the link to a very informative video "The Mysterious Syndrome Called CAPS" on Heathy Body Healthy Mind episode 1201.
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