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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).
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 ...
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.
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.
Rather than predisposing for infections, most of the autoinflammatory disorders lead to excessive inflammation. Many manifest themselves as periodic fever syndromes. They may involve various organs directly, as well as predisposing for long-term damage (e.g. by leading to amyloid deposition). [7] Familial Mediterranean fever
Autoinflammatory syndromes are a group of inherited disorders characterized by bouts of inflammatory skin lesions and periodic fevers. [29] [30] Blau syndrome Blau syndrome; Chronic infantile neurologic cutaneous and articular syndrome; Familial cold urticaria (familial cold autoinflammatory syndrome) Familial Mediterranean fever; Hyper-IgD ...
Rilonacept, sold under the brand name Arcalyst, is a medication used to treat cryopyrin-associated periodic syndromes, including familial cold autoinflammatory syndrome, and Muckle–Wells syndrome; deficiency of interleukin-1 receptor antagonist; and recurrent pericarditis. [1] [2] [3] Rilonacept is an interleukin 1 inhibitor. [4]
Familial cold autoinflammatory syndrome; Familial Mediterranean fever; Familial partial lipodystrophy; Familial Adenomatous Polyposis; Fanconi syndrome; Favre–Racouchot syndrome; Febrile infection-related epilepsy syndrome; Febrile neutrophilic dermatosis; Fechtner syndrome; Feingold syndrome; Feline hyperesthesia syndrome; Felty's syndrome ...