Cytokine Activation During Attacks of the Hyperimmunoglobulinemia D and Periodic Fever Syndrome
نویسندگان
چکیده
The hyperimmunoglobulinemia D and periodic fever (hyperIgD) syndrome is typified by recurrent febrile attacks with abdominal distress, joint involvement (arthralgias/arthritis), headache, skin lesions, and an elevated serum IgD level (>l00 U/mL). This familial disorder has been diagnosed in 59 patients, mainly from Europe. The pathogenesis of this febrile disorder is unknown, but attacks are joined by an acute-phase response. Because this response is considered to be mediated by cytokines, we measured the acute-phase proteins C-reactive protein (CRP) and soluble type4 phospholipase A2 (PLA2) together with circulating concentrations and ex vivo production of the proinflammatory cytokines interleukin-la (IL-la), IL-Ip, IL-6, and tumor necrosis factor a (TNFa) and the inhibitory compounds IL-l receptor antagonist flL-Ira). IL-10. and the soluble TNF receptors p55 (sTNFr p551 and p75 (sTNFr p751 in 22 patients with the hyper-lgD syndrome during attacks and remission. Serum CRP and PLA2 concentrations were elevated during attacks (mean,
منابع مشابه
Cytokine activation during attacks of the hyperimmunoglobulinemia D and periodic fever syndrome.
The hyperimmunoglobulinemia D and periodic fever (hyper-IgD) syndrome is typified by recurrent febrile attacks with abdominal distress, joint involvement (arthralgias/arthritis), headache, skin lesions, and an elevated serum IgD level (> 100 U/mL). This familial disorder has been diagnosed in 59 patients, mainly from Europe. The pathogenesis of this febrile disorder is unknown, but attacks are ...
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