PReS-FINAL-2234: Successful canakunimab treatment in uveitis secondary to cryopyrin-associated periodic syndrome
نویسندگان
چکیده
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 important organ involvement in CAPS is the associated uveitis. Uveitis in CAPS is unresponsive to most of the classic treatment modalities.
منابع مشابه
PReS-FINAL-2241: Cases of cryopyrin-associated periodic syndromes (CAPS) in one single rheumatologic center of Russia
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, op...
متن کاملPReS-FINAL-2199: A novel mutation in the CIAS1/NLRP3 gene associated with an unusual phenotype of CAPS
Introduction Cryopyrin-associated periodic syndrome (CAPS) is an autoinflammatory syndrome caused by heterozygous mutations of CIAS1/NLRP3 gene. Affected patients may present three different phenotypes: familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome and CINCA syndrome. Common symptoms include sporadic or cold-induced non pruritic urticarial rash and fever. Severe cases su...
متن کاملObvious optic disc swelling in a patient with cryopyrin-associated periodic syndrome
Cryopyrin-associated periodic syndrome (CAPS) is a group of rare hereditary autoinflammatory diseases caused by mutations of the NLRP3 gene, and leads to excessive production of the proinflammatory cytokine, interleukin-lβ. A 35-year-old male presented with recurrent symptoms of urticarial-like rash, periodic fever, arthralgia, headache, and eye redness. His best-corrected visual acuity was 1.0...
متن کاملPReS-FINAL-2200: Phenotype of V198M and Q703K NLRP3 variants
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...
متن کاملSuccessful resolution of stromal keratitis and uveitis using canakinumab in a patient with chronic infantile neurologic, cutaneous, and articular syndrome: a case study.
BACKGROUND Cryopyrin-associated periodic syndrome (CAPS) is a group of rare autoinflammatory diseases, and of these, chronic infantile neurologic, cutaneous, and articular/neonatal-onset multisystem inflammatory disease (CINCA/NOMID) syndrome has the most severe phenotype. Canakinumab, a monoclonal antibody that targets interleukin-1β, has been shown to be an effective treatment for resolving s...
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عنوان ژورنال:
دوره 11 شماره
صفحات -
تاریخ انتشار 2013