Complement profile in microscopic polyangiitis and granulomatosis with polyangiitis: analysis using sera from a nationwide prospective cohort study
Complement profile in microscopic polyangiitis and granulomatosis with polyangiitis: analysis using sera from a nationwide prospective cohort study
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DOI：
10.1080/03009742.2019.1695927
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发表时间：
2020-04
影响因子：
2.1
通讯作者：
S. Fukui;K. Ichinose;K. Sada;J. Miyamoto;M. Harigai;K. Amano;T. Atsumi;Y. Takasaki;H. Dobashi;Y. Arimura;H. Hasegawa;Y. Yuzawa;K. Yamagata;N. Tsuboi;S. Maruyama;S. Matsuo;H. Makino;T. Maeda;A. Kawakami
中科院分区：
文献类型：
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作者：
S. Fukui;K. Ichinose;K. Sada;J. Miyamoto;M. Harigai;K. Amano;T. Atsumi;Y. Takasaki;H. Dobashi;Y. Arimura;H. Hasegawa;Y. Yuzawa;K. Yamagata;N. Tsuboi;S. Maruyama;S. Matsuo;H. Makino;T. Maeda;A. Kawakami
Objective The complement cascade, especially the alternative pathway of complement, has been shown in basic research to be associated with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). We aimed to elucidate relationships between serum complement components and clinical characteristics in AAV. Method In a nationwide prospective cohort study (RemIT-JAV-RPGN), we measured the serum levels of C1q, C2, C3, C3b/iC3b, C4, C4b, C5, C5a, C9, factor B, factor D, factor H, factor I, mannose-binding lectin, and properdin in 52 patients with microscopic polyangiitis (MPA) and 39 patients with granulomatosis with polyangiitis (GPA). Results The properdin level of MPA and GPA was significantly lower than that of healthy donors. The properdin level was negatively correlated with the Birmingham Vasculitis Activity Score (BVAS) (ρ = −0.2148, p = 0.0409). The factor D level at 6 months was significantly positively correlated with the Vasculitis Damage Index (VDI) at 6, 12, and 24 months (ρ = 0.4207, 0.4132, and 0.3115, respectively). Patients with a higher ratio of C5a to C5 had higher neutrophil percentage and serum immunoglobulin G levels, and significantly lower creatinine levels. Cluster analysis divided the MPA and GPA patients into three subgroups. A principal component (PC) analysis aggregated 15 types of complements into alternative pathway-related PC 1 and complement classical pathway and common pathway-related PC 2. Conclusions The serum levels of properdin and factor D were correlated with the BVAS and the VDI in MPA and GPA, respectively. Our analyses suggested the pathological heterogeneity of MPA and GPA from the aspect of complement components.
