cryopyrin-associated periodic syndromes

cryopyrin-associated periodic syndromes

A range of autoinflammatory disorders—e.g., familial cold autoinflammatory syndrome (FCAS, aka familial cold-induced urticaria), Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease (NOMID, aka chronic infantile neurologic cutaneous and articular syndrome [CINCA])—which share many clinical features (e.g., fever, rash, joint pain, conjunctivitis, headaches, general malaise).

Management
Monoclonal antibodies against IL 1 beta (e.g., canakinumab), other IL-1 binding proteins (e.g., rilonacept), or IL-1 receptor antagonist (e.g., anakinra).
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References in periodicals archive ?
Polyradiculoneuritis, cryopyrin-associated periodic syndromes, and familial Mediterranean fever.
Canakinumab is marketed by Novartis as Ilaris and is already approved for cryopyrin-associated periodic syndromes, familial Mediterranean fever, juvenile idiopathic arthritis, and other rare autoimmune inflammatory diseases.
Rilonacept has been generally well-tolerated in the study, with adverse events, or AEs, consistent with the FDA-approved label for the treatment of Cryopyrin-Associated Periodic Syndromes, or CAPS, including Familial Cold Autoinflammatory Syndrome and Muckle-Wells Syndrome.
According to the manufacturer's recommendations, the pharmacokinetic characteristics of canakinumab allow its administration once every 8 weeks without loss of efficacy for the treatment of cryopyrin-associated periodic syndromes [8].
Cryopyrin-associated periodic syndromes (CAPS) are a rare, autoinflammatory disease group that generally has autosomal dominant inheritance.
Amar et al., "Efficacy and safety of rilonacept (Interleukin-1 Trap) in patients with cryopyrin-associated periodic syndromes: results from two sequential placebo-controlled studies," Arthritis and Rheumatism, vol.
Ilaris was previously approved for another periodic fever syndrome called Cryopyrin-Associated Periodic Syndromes (CAPS) and for active systemic juvenile idiopathic arthritis.
In early 2013, Sobi announced that the US Food and Drug Administration (FDA) had approved Kineret for the treatment of children and adults with neonatal-onset multisystem inflammatory disease (NOMID), the most severe form of Cryopyrin-Associated Periodic Syndromes (CAPS).
Frediani et al., "Bridging the gap between the clinician and the patient with cryopyrin-associated periodic syndromes," International Journal of Immunopathology and Pharmacology, vol.
Cryopyrin-associated periodic syndromes are a group of rare inherited auto-inflammatory diseases that cause rash, headache, fever, joint pain and other inflammatory symptoms.
Cryopyrin-associated periodic syndromes: background and therapeutics.
Cryopyrin-associated periodic syndromes are a group of autoinflammatory diseases transmitted by autosomal dominant inheritance caused by mutations in the NLRP3 gene (also called CIAS1 or PYPAF) encoding for cryopyrin, a crucial inflammasome protein that directly activates IL-1[beta].