[膜增生性肾小球肾炎中补体系统的变化]

[Changes in the complement system in membranoproliferative glomerulonephritis].

作者信息

Yurova V A, Bobrova L A, Kozlovskaya N L, Korotchaeva Yu V, Serova A G, Kozlov L V, Andina S S, Demyanova K A, Kuchieva A M, Roshchupkina S V

机构信息

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia, Moscow, Russia.

G.N.Gabrichevsky Moscow Research Institute of Epidemiology and Microbiology, Russian Federal Service for Supervision of Consumer Rights Protection and Human Welfare, Moscow, Russia.

出版信息

Ter Arkh. 2017;89(6):69-77. doi: 10.17116/terarkh201789669-77.

DOI:10.17116/terarkh201789669-77

PMID:28745692

Abstract

AIM

To compare the clinical manifestations membranoproliferative glomerulonephritis (MPGN) in its idiopathic variant, lupus nephritis (LN), and C3 glomerulopathy (C3-GP), by comparing them with changes in the complement system.

SUBJECTS AND METHODS

The clinic of nephrology followed up 42 patients with different types of MPGN in 2013 to 2015. The study included 35 patients divided into 3 groups: 1) 8 patients with C3-GP, 2) 13 with idiopathic MPGN; 3) 14 with Class IV LN. The investigators studied the blood and urine levels of components and markers for activation of the classical and alternative pathways (C3 and C4, С3а, C5a, CFH, CFB, and CFD) of the terminal complement complex (TCC).

RESULTS

The detection rate of C3-GP was 19%. The patients with C3-GP were noted to have the lowest blood concentration of S3 and the highest urinary level of С3а, C5a, TCC, CFH, CFB, and CFD. C3 nephritic factor was detected in 2 patients from the C3-GP (dense deposit disease) group.

CONCLUSION

Alternative complement pathway dysregulation caused by genetic or autoimmune factors plays a leading role in the pathogenesis of C3-GP.

摘要

目的

通过比较特发性膜增生性肾小球肾炎（MPGN）、狼疮性肾炎（LN）和C3肾小球病（C3-GP）与补体系统变化，比较它们的临床表现。

研究对象与方法

肾脏病门诊在2013年至2015年对42例不同类型的MPGN患者进行了随访。该研究纳入了35例患者，分为3组：1）8例C3-GP患者；2）13例特发性MPGN患者；3）14例IV型LN患者。研究人员研究了终末补体复合物（TCC）经典途径和替代途径（C3和C4、C3a、C5a、CFH、CFB和CFD）的激活成分和标志物的血液和尿液水平。