An inherited condition characterized by recurrent episodes of fever; onset is typically in early childhood. Fevers usually persist for about three weeks, though they can be longer or shorter, and may not occur again for several weeks or several years. Other symptoms may include abdominal pain; myalgia; arthralgia; skin rash and systemic inflammation; amyloidosis may also occur in some cases. There is often no trigger, but episodes are sometimes preceded by infections, injury, or stress. Germline mutations in the TNFRSFIA gene have been identified.
Type:
Clinical condition associated with amyloidosis
ICD-10 Classification
Diseases of the Musculoskeletal System and Connective Tissue
Serum amyloid A-1 protein (SAA) [Cleaved into: Amyloid protein A (Amyloid fibril protein AA); Serum amyloid protein A(2-104); Serum amyloid protein A(3-104); Serum amyloid protein A(2-103); Serum amyloid protein A(2-102); Serum amyloid protein A(4-101)]