PReS-FINAL-2241: Cases of cryopyrin-associated periodic syndromes (CAPS) in one single rheumatologic center of Russia
نویسندگان
چکیده
Introduction Cryopyrin-associated periodic syndromes (CAPS) are the rare hereditary autoinflammatory diseases. CAPS include three similar conditions are distinguished which lie along a phenotypical continuum with increasing levels of severity: familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and CINCA/ NOMID. Distinguishing features include cutaneous, neurological, ophthalmologic and rheumatologic manifestations. CAPS caused by the mutation of the NLRP3 (CIAS1) gene coding for cryopyrin.
منابع مشابه
Cryopyrin-associated periodic syndromes (CAPS): report on two cases (Muckle-Wells syndrome (MWS) and CINCA/NOMID)
Background Cryopyrin-associated periodic syndromes (CAPS) are the rare hereditary autoinflammatory diseases. CAPS include three similar conditions are distinguished which lie along a phenotypical continuum with increasing levels of severity: familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and CINCA/NOMID. Distinguishing features include cutaneous, neurological, ophth...
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Introduction The term CAPS (Cryopyrin-Associated Periodic Syndromes) identifies a spectrum of autoinflammatory diseases caused by heterozygous mutations of the CIAS1/NLRP3. Affected individuals may present three different phenotypes: Familial Cold Autoinflammatory Syndrome (FCAS), Muckle-Wells Syndrome (MWS) and CINCA syndrome, the most severe form of the clinical spectrum. Clinical manifestati...
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Introduction Cryopyrin-associated periodic syndrome (CAPS) is a constellation of diseases with a different and varying clinical spectrum which is rarely seen in childhood. The mildest form of CAPS which is familial autoinflammatory syndrome and the most severe form which is chronic inflammatory neurologic cutaneous arthropathy (CINCA) possess a very different spectrum of clinical findings. Most...
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Introduction Interleukin-1b (IL-1b) is a basic mediator of cryopyrinassociated periodic syndromes (CAPS). In this respect an experience of the use of IL-1 inhibitors has been gained in patients with CAPS. Canakinumab was approved by FDA and EMEA in 2009 and registered in the Russian Federation in 2011 for treatment of CAPS. Canakinumab is shown to have high efficiency and good tolerability in p...
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