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. 2019 Nov 5;10:2537. doi: 10.3389/fimmu.2019.02537

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Copyright © 2019 Gröbner, Kapferer-Seebacher, Amberger, Redolfi, Dalonneau, Björck, Milnes, Bally, Rossi, Thielens, Stoiber, Gaboriaud and Zschocke.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Significantly increased complement 1 activation in fibroblast supernatants from individuals with pEDS. C4a was measured in serum and supernatant as a marker for complement 1 activation. (A) After addition of C4 to the supernatant of human-derived skin fibroblast a significant increase of C4 cleavage was detected in pEDS (n = 9) but not in controls (n = 4) (line indicates median, p = 0.003). P-values were calculated by Mann-Whitney test (α = 0.05). (B) Serum concentrations of C4a were increased in some pEDS patients, but there was no significant overall difference between patients (n = 10) and controls (n = 11). α = 0.05; ^* ≤ 0.05; ^** ≤ 0.01; ^*** ≤ 0.001.