Plasma complement factor H is associated with disease activity of patients with ANCA-associated vasculitis.
Plasma complement factor H is associated with disease activity of patients with ANCA-associated vasculitis.
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血浆补体因子 H 与 ANCA 相关性血管炎患者的疾病活动性相关
DOI：
10.1186/s13075-015-0656-8
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发表时间：
2015-05-21
影响因子：
4.9
通讯作者：
Chen M
中科院分区：
文献类型：
--
作者：
Chen SF;Wang FM;Li ZY;Yu F;Zhao MH;Chen M
越来越多的证据表明，替代补体途径的激活在抗中性粒细胞胞浆抗体相关性小血管炎(AAV)的发病机制中起着重要作用。本研究旨在探讨补体替代途径的关键调节因子--补体因子H(CFH)与AAV疾病活动性的关系。方法检测82例活动期MPO-AAV患者血浆CFH水平。在82名患者中，27名患者的血浆CFH水平进行了纵向测量。对AAV患者进行血清抗CFH自身抗体筛查。进一步检测活动期AAV患者循环补体C4d、Bb、C3a、C5a和可溶性C5b-9的活性。结果活动期AAV患者血浆CFH水平明显低于缓解期AAV患者和正常对照组。相关分析显示，血浆CFH水平与初始血肌酐、伯明翰血管炎活动评分(BVAS)、肾标本中总新月体和细胞新月体的比例、循环C3a、C5a和Sc5b-9水平呈负相关，与估计肾小球滤过率(EGFR)、血红蛋白水平和循环C3水平呈正相关。此外，多因素生存分析显示，在调整了年龄、性别、血红蛋白水平和尿蛋白(P=0.03，HR 0.85，95%CI 0.73-0.98)或调整了年龄、性别、新月体总数(%)和尿蛋白(P=0.03，HR 0.85，95%CI 0.73-0.98)后，血浆CFH水平与AAV患者死亡或终末期肾病(ESRD)的综合预后独立相关，而在调整年龄、性别、血肌酐和尿蛋白后，CFH水平不是独立预测因素(P=0.57，HR为0.96，95%CI为0.83~1.11。结论血浆CFH水平与MPO-ANCA相关性小血管炎患者的疾病活动性有关，并在一定程度上与患者的综合预后有关。
IntroductionIncreasing evidences have demonstrated that activation of alternative complement pathway plays an important role in the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The current study aimed to investigate the association of complement factor H (CFH), a key regulator of the alternative complement pathway, with the disease activity of AAV.MethodsPlasma CFH levels were measured in 82 patients with myeloperoxidase (MPO)-AAV in active stage. Of the 82 patients, plasma CFH levels of 27 patients were longitudinally measured. Serum anti-CFH autoantibodies were screened in AAV patients. Circulating complement activation profiles including C4d, Bb, C3a, C5a and soluble C5b-9 of AAV patients in active stage were further detected. Associations between plasma CFH levels and clinicopathological parameters as well as the prognosis were analyzed.ResultsPlasma CFH levels were significantly lower in active AAV patients compared with AAV patients in remission and normal controls. Correlation analysis showed that plasma CFH levels inversely correlated with initial serum creatinine, Birmingham Vasculitis Activity Score (BVAS), proportion of total crescents and cellular crescents in renal specimens, and circulating levels of C3a, C5a and Sc5b-9, meanwhile positively correlated with estimated glomerular filtration rate (eGFR), hemoglobin levels and circulating levels of C3. Moreover, multivariate survival analysis revealed that plasma CFH levels were independently associated with composite outcome of death or end stage renal disease (ESRD) in AAV patients, after adjusting for age, gender, hemoglobin level and urinary protein (P = 0.03, HR 0.85, 95 % CI 0.73–0.98) or adjusting for age, gender, total crescents (%) and urinary protein (P = 0.03, HR 0.85, 95 % CI 0.73–0.98), while not as an independent predictor after adjusting for age, gender, serum creatinine and urinary protein (P = 0.57, HR 0.96, 95 % CI 0.83–1.11).ConclusionIn conclusion, plasma CFH levels are associated with disease activity, and, to some extent, associated with composite outcomes of patients with MPO-ANCA-associated vasculitis.
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