CAPS

(Cryopyrin-Associated Periodic Syndrome)

Cryopyrin-Associated Periodic Syndromes (CAPS) are rare chronic inflammatory diseases of which only approx. 1000 cases are known worldwide. The number of unrecorded, not yet documented cases may be significantly higher as these diseases are still unknown even among health care specialists.

CAPS is sub-divided into the groups known as Familial Cold Autoinflammatory Syndrome (FCAS), Muckle Wells Syndrome (MWS) and Neonatal Onset Multisystem Inflammatory Disease (NOMID, or CINCA, respectively). The cause for all three is found in the mutation of the NLRP3 gene. This mutation leads to an increased production of inflammasome-mediated  Interleukin-1ß, which causes the majority of symptoms. CAPS is an autosomal-dominant inherited disease. The underlying mutation in the NLRP3 gene can be passed down for many generations.

The three syndromes FCAS, MWS and NOMID are characterised by bouts of recurrent fever and inflammatory reactions of various organs, however vary with regard to symptom severity.

FCAS (mildest manifestation)

  • Recurrent rash
  • Bouts of fever
  • Fatigue; drowsiness
  • Joint and muscle pain
  • Red eyes, inflammation of the eyes
  • Trigger for the symptoms usually exposure to cold temperatures

MWS (moderate manifestation)

  • Recurrent or daily rash
  • Bouts of fever
  • Fatigue; drowsiness
  • Joint and muscle pain
  • Red eyes, eye inflammation
  • Headaches
  • Gradual progressive loss of hearing
  • Symptoms increase when exposed to cold temperatures, stress; 
  • Frequently continuous symptoms

NOMID/CINCA (severest manifestation)

  • Daily rashes
  • Fever
  • Fatigue, drowsiness
  • Inflammation of the joints and muscle pain
  • Red eyes, inflammation of the eyes, impaired vision
  • Headaches, neurological symptoms
  • Gradual loss of hearing
  • Deposits of protein in the kidneys
  • Continuous symptoms

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