periodic fever syndrome


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periodic fever syndrome

Immunology A heterogeneous group of inherited diseases of uncertain pathogenesis, in which the diagnosis is based on clinical features rather than on specific tests. See Familial Mediterranean fever, Hyper-IgD syndrome.
References in periodicals archive ?
Hyperimmunoglobulinemia D with periodic fever syndrome is an autosomal recessive disorder that is associated with the MVK gene.
Concerted action of wild-type and mutant TNF receptors enhances inflammation in TNF receptor 1-associated periodic fever syndrome. Proc Natl Acad Sci U S A.
The milder phenotype of MKD is a periodic fever syndrome characterized by frequent episodes of fever typically lasting 3-7 days, abdominal pain, lymphadenopathy, inflammatory eye disease, rashes, and arthralgia [1] and typically associated with intracellular MVK activity greater than 1% [2].
Periodic fever syndrome or autoinflammatory diseases are group of disorders characterized by recurrent episodes of systemic inflammation.
Mutations in the gene encoding mevalonate kinase cause hyper-IgD and periodic fever syndrome. Nat Genet 1999; 22: 178-81.
Following the encouraging data from the company's Phase III CLUSTER study, the US FDA granted Ilaris Breakthrough Therapy status and priority reviews for each of the three Periodic Fever Syndrome conditions.
As a result of the superior data, the FDA granted Ilaris Breakthrough Therapy status and priority reviews for each of the three Periodic Fever Syndrome conditions.
Clinical immunology review series: An approach to the patient with a periodic fever syndrome. Clin Exp Immunol 2011; 165: 301-9.
The second familial syndrome, hyperimmunoglobulinemia D with periodic fever syndrome, or hyper IgD (HIDS), has an early age of onset, typically before 12 months and with more than half before 6 months.
periodic fever syndrome (HIDS)###(MVK)###lymphadenopathy###inhibitors
Bertoni et al., "First report of macrophage activation syndrome in hyperimmunoglobulinemia D with periodic fever syndrome," Arthritis and Rheumatism, vol.