periodic fever syndrome

(redirected from Autoinflammatory syndrome)

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 ?
(Regeneron) and is approved by the FDA under the brand name ARCALYST for the treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), which includes Familial Cold Autoinflammatory Syndrome and Muckle-Wells Syndrome.
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.
Three familial cold syndromes exist: familial delayed CU, familial cold autoinflammatory syndrome (FCAS), and familial atypical CU (FACU).[1] Here, we report three Chinese pedigrees of FCU with an autosomal dominant inheritance pattern [Figure 1]a.{Figure 1}
CPAS has three clinical subtypes according to the severity of the disease: Familial cold autoinflammatory syndrome (FCAS), Muckle--Wells syndrome (MWS), and chronic infantile neurologic cutaneous articular syndrome/neonatal-onset multisystemic inflammatory disease (CINCA/NO-MID).
Boyle et al., "Prevention of cold-associated acute inflammation in familial cold autoinflammatory syndrome by interleukin-1 receptor antagonist," The Lancet, vol.
(1) The disease is thought to be an adult onset autoinflammatory syndrome with unknown etiology and unknown pathogenesis.
CANDLE, which stands for chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature, is a proteasome-associated autoinflammatory syndrome characterized by dysregulation of type 1 interferon signaling, Dr.
In the US, Ilaris is approved for two subtypes of CAPS: Muckle-Wells Syndrome (MWS) and Familial Cold Autoinflammatory Syndrome (FCAS).
Pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PAPASH): a new autoinflammatory syndrome associated with a novel mutation of the PSTPIP1 gene.
"The presence of systemic symptoms could mean the urticarial rash is not ordinary urticaria," he said, suggesting that vasculitis, Schitzler's syndrome, adult-onset Still's disease, an autoinflammatory syndrome, or urticarial dermatitis could be at play.
* Autoinflammatory syndrome induced by adjuvants, a rare syndrome associated with vaccine administration, has been linked to postvaccination adverse events, exposure to silicone implants, Gulf War syndrome (related to multiple vaccinations), and macrophagic myofasciitis.
The least severe is familial cold autoinflammatory syndrome (FCAS) (OMIM 120100).