Research Laboratory, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
Hungarian Angioedema Center, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
Allergy. 2018 Feb;73(2):516-520. doi: 10.1111/all.13327. Epub 2017 Oct 30.
We studied the kinetics of C1-inhibitor (C1-INH) and other complement parameters in a self-limited edematous attack (EA) in a patient with hereditary angioedema due to C1-INH deficiency to better understand the pathomechanism of the evolution, course, and complete resolution of EAs. C1-INH concentration and functional activity (C1-INH ), C1(q,r,s), C3, C4, C3a, C4a, C5a, and SC5b-9 levels were measured in blood samples obtained during the 96-hour observation period. The highest C1-INH , C4, and C1(q,r,s) levels were measured at baseline, and their continuous decrease was observed during the entire observation period. C4 depletion started at prodromal phase, and C4 was lowest after the maximum severity peak. Compared to baseline, C4a level was four times higher 7 hours before the onset of the attack. C1-INH did not increase after resolution of the attack suggesting that factors other than C1-INH may be important in this process. C4a may be a useful biomarker for the prediction of EAs.
我们研究了遗传性血管性水肿患者自限性水肿发作（EA）期间 C1 抑制剂（C1-INH）和其他补体参数的动力学，以更好地了解 EA 进展、病程和完全缓解的病理机制。在 96 小时观察期间采集血样，测量 C1-INH 浓度和功能活性（C1-INH）、C1（q，r，s）、C3、C4、C3a、C4a、C5a 和 SC5b-9 水平。在基线时测量到最高的 C1-INH、C4 和 C1（q，r，s）水平，在整个观察期间观察到其持续下降。C4 耗竭始于前驱期，C4 在最大严重程度峰值后最低。与基线相比，在发作前 7 小时 C4a 水平升高了四倍。C1-INH 在攻击缓解后并未增加，这表明在这个过程中除了 C1-INH 之外，其他因素可能很重要。C4a 可能是预测 EA 的有用生物标志物。
