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Cytokine activation during attacks of the hyperimmunoglobulinemia D and
periodic fever syndrome
JP Drenth, M van Deuren, J van der Ven-Jongekrijg, CG Schalkwijk and JW van der Meer
Department of Medicine, University Hospital St. Radboud, Nijmegen, The
Netherlands.
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 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
type-II phospholipase A2 (PLA2) together with circulating concentrations
and ex vivo production of the proinflammatory cytokines interleukin-1 alpha
(IL-1 alpha), IL-1 beta, IL-6, and tumor necrosis factor alpha (TNF alpha)
and the inhibitory compounds IL-1 receptor antagonist (IL-1ra), IL-10, and
the soluble TNF receptors p55 (sTNFr p55) and p75 (sTNFr p75) in 22
patients with the hyper-IgD syndrome during attacks and remission. Serum
CRP and PLA2 concentrations were elevated during attacks (mean, 213 mg/L
and 1,452 ng/mL, respectively) and decreased between attacks. Plasma
concentrations of IL-1 alpha, IL-1 beta, or IL-10 were not increased during
attacks. TNF alpha concentrations were slightly, but significantly, higher
with attacks (104 v 117 pg/mL). Circulating IL-6 values increased with
attacks (19.7 v 147.9 pg/mL) and correlated with CRP and PLA2 values during
the febrile attacks. The values of the antiinflammatory compounds IL-1ra,
sTNFr p55, and sTNFr p75 were significantly higher with attacks than
between attacks, and there was a significant positive correlation between
each. The ex-vivo production of TNF alpha, IL-1 beta, and IL-1ra was
significantly higher with attacks, suggesting that the
monocytes/macrophages were already primed in vivo to produce increased
amounts of these cytokines. These findings point to an activation of the
cytokine network, and this suggests that these inflammatory mediators may
contribute to the symptoms of the hyper-IgD syndrome.
Volume 85,
Issue 12,
pp. 3586-3593,
06/15/1995
Copyright © 1995 by The American Society of Hematology
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