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familial cold autoinflammatory syndrome

Tuesday 13 October 2009

Definition: Familial Cold Autoinflammatory Syndrome (FCAS) is the mildest of the three cryopyrinopathies.

Symptoms often develop in childhood and tend to worsen with age. Approximately 150 cases of FCAS have been identified in the United States. Patients tend to live in the mid-southern states, south of winter freezing and north of constant exposure to air conditioning in the summertime.

Exposure to cold typically brings on a pruritic, painful rash. Conjunctivitis, fever, joint symptoms, fatigue and headache may be part of clinical syndrome. Hearing loss, a more constant feature of the more severe cryopyrinopathies, is rare. Amyloidosis develops in fewer than 5% of patients. Anakinra is the agent of choice in treating FCAS.

See also

- hereditary autoinflammatory syndromes
- cryopyrinopathies