CAPS
CAPS
CAPS (pronounced /kæps/), an acronym for Cryopyrin-Associated Periodic Syndromes, is a group of rare, inherited autoinflammatory diseases.
Etymology
The term "CAPS" is derived from the name of the protein "cryopyrin" which is associated with these syndromes, and the term "periodic syndromes" which refers to the episodic nature of the symptoms.
Definition
CAPS encompasses three diseases: Familial Cold Autoinflammatory Syndrome (FCAS), Muckle-Wells Syndrome (MWS), and Neonatal-Onset Multisystem Inflammatory Disease (NOMID). These diseases are characterized by episodes of inflammation and fever, skin rash, joint pain, and often eye problems. They are caused by mutations in the NLRP3 gene, which leads to overproduction of a protein called interleukin-1beta, causing inflammation.
Familial Cold Autoinflammatory Syndrome (FCAS)
FCAS (pronounced /ef-see-ey-es/) is the mildest form of CAPS. It is characterized by episodes of fever, skin rash, and joint pain triggered by exposure to cold temperatures.
Muckle-Wells Syndrome (MWS)
MWS (pronounced /mʌkəl-wɛlz/) is a more severe form of CAPS. In addition to the symptoms seen in FCAS, patients with MWS may also have hearing loss and kidney damage.
Neonatal-Onset Multisystem Inflammatory Disease (NOMID)
NOMID (pronounced /noʊ-mɪd/) is the most severe form of CAPS. It is characterized by skin rash, joint pain, and inflammation of the membranes surrounding the brain and spinal cord (meningitis) starting in infancy.
Related Terms
- Autoinflammatory diseases: A group of diseases characterized by episodes of inflammation without a known cause.
- NLRP3: A gene that provides instructions for making a protein involved in the immune system.
- Interleukin-1beta: A protein that promotes inflammation and is involved in immune responses.
External links
- Medical encyclopedia article on CAPS
- Wikipedia's article - CAPS
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