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Clinical Trial
. 2015 Mar;45(3):915-21.
doi: 10.1002/eji.201444948. Epub 2015 Jan 19.

Dysregulation of adaptive immune responses in complement C3-deficient patients

Pirkka T Pekkarinen  1 , Nelli Heikkilä, Kai Kisand, Pärt Peterson, Marina Botto, Mohamed R Daha, Christian Drouet, Lourdes Isaac, Merja Helminen, Tari Haahtela, Seppo Meri, Hanna Jarva, T Petteri Arstila
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Clinical Trial

"VSports手机版" Dysregulation of adaptive immune responses in complement C3-deficient patients

Pirkka T Pekkarinen et al. Eur J Immunol. 2015 Mar.
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V体育平台登录 - Abstract

In addition to its effector functions, complement is an important regulator of adaptive immune responses. Murine studies suggest that complement modulates helper T-cell differentiation, and Th1 responses in particular are impaired in the absence of functional complement VSports手机版. Here, we have studied humoral responses to toxoid vaccines in eight patients with C3 deficiency, representing more than 25% of all the known patients worldwide. Serum cytokine levels were also studied. The patients developed normal Ig responses to tetanus and diphtheria toxoids, but IgE levels were low. The pattern of antigen-specific IgG subclasses was abnormal, with increased Th1-related IgG3 responses, low IgG2, and almost completely undetectable IgG4. The patients also had increased amounts of Th1-related cytokines IL-12p70 and IL-21, and these showed a positive correlation with IgG3 levels. Our results confirm that complement modulates Th differentiation, but reveal a more nuanced outcome than previously reported. Since IgG4 has been linked to tolerogenic responses, the data also suggest that in the absence of functional complement at least some aspects of systemic tolerance are impaired. .

Keywords: Adaptive immunity; Complement; Immune deficiency; Immunoglobulin G subclass; Immunoglobulin G4 V体育安卓版. .

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