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Familial cold urticaria (also properly known as familial cold autoinflammatory syndrome, FCAS) is an autosomal dominant condition characterized by rash, conjunctivitis, fever/chills and arthralgias [4] elicited by exposure to cold – sometimes temperatures below 22 °C (72 °F). [3] [5]
It encompasses a spectrum of three clinically overlapping autoinflammatory syndromes including familial cold autoinflammatory syndrome (FCAS, formerly termed familial cold-induced urticaria), the Muckle–Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID, also called chronic infantile neurologic cutaneous and ...
Autoinflammatory syndromes This page was last edited on 5 February 2014, at 19:48 (UTC) . Text is available under the Creative Commons Attribution-ShareAlike 4.0 License ; additional terms may apply.
Muckle–Wells syndrome; Familial cold autoinflammatory syndrome, types 1, 2, 3, and 4; Neonatal onset multisystem inflammatory disease; NLRP1 deficiency; PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, acne) ADAM17 deficiency; Blau syndrome; Majeed syndrome (Chronic recurrent multifocal osteomyelitis and congenital ...
FCAS may refer to: Familial cold autoinflammatory syndrome, a genetic disease; Fellow of the Casualty Actuarial Society, a U.S. professional society; The Foundation to Combat Antisemitism, a philanthropic effort by Robert Kraft; Frequency Control Ancillary Services for electric power grids
Periodic fever syndromes are a set of disorders characterized by recurrent episodes of systemic and organ-specific inflammation.Unlike autoimmune disorders such as systemic lupus erythematosus, in which the disease is caused by abnormalities of the adaptive immune system, people with autoinflammatory diseases do not produce autoantibodies or antigen-specific T or B cells.
As a result, MWS is considered a type of periodic fever syndrome. MWS is caused by a defect in the CIAS1 gene which creates the protein cryopyrin . MWS is closely related to two other syndromes, familial cold urticaria and neonatal onset multisystem inflammatory disease —in fact, all three are related to mutations in the same gene and ...
This includes familial cold autoinflammatory syndrome (FCAS), Muckle–Wells syndrome (MWS), chronic infantile neurological cutaneous and articular syndrome, neonatal onset multisystem inflammatory disease (NOMID), and keratoendotheliitis fugax hereditaria. [5] [20] Defects in this gene have also been linked to familial Mediterranean fever. [21]