C1q nephropathy: A variant of focal segmental glomerulosclerosis
C1q nephropathy: A variant of focal segmental glomerulosclerosis
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DOI：
10.1046/j.1523-1755.2003.00218.x
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发表时间：
2003-10-01
影响因子：
19.6
通讯作者：
D'Agati, VD
中科院分区：
文献类型：
--
作者：
Markowitz, GS;Schwimmer, JA;D'Agati, VD
背景。 C1q 肾病是一种人们知之甚少且有争议的实体，具有独特的免疫病理学特征。为了更好地定义临床病理谱，我们报告了最大的单中心系列。方法。从 1994 年至 2002 年收到的 8909 份自体肾活检中，鉴定出 19 份患有 C1q 肾病的活检（0.21%）。定义标准包括 (1) C1q 显性或共显性免疫荧光染色，(2) 系膜电子致密沉积物，以及 (3) 没有系统性红斑狼疮 (SLE) 的临床或血清学证据。结果。这 19 名患者主要是非裔美国人 (73.7%)、女性 (73.7%)、年轻人和儿童（年龄范围 3 至 42 岁；平均 24.2 岁）。表现包括肾病范围蛋白尿（78.9%）、肾病综合征（50%）、肾功能不全（27.8%）和血尿（22.2%）。没有患者患有低补体血症或潜在自身免疫性疾病或传染病的证据。肾活检显示 17 例患者患有局灶节段性肾小球硬化症 (FSGS)（其中 6 例为塌陷性肾小球硬化症，2 例为细胞性肾小球硬化症），2 例患者患有微小病变肾病 (MCD)。所有活检均显示免疫球蛋白 G (IgG) 的共沉积物，以及更多变异的 IgM (84.2%)、IgA (31.6%) 和 C3 (52.6%)。足突消失范围从 20% 到 100%（平均 51%）。 16 名可进行随访的患者中有 12 名接受了免疫抑制治疗。一名患者蛋白尿完全缓解，六名患者部分缓解。 4 名 FSGS 模式患者患有进行性肾功能不全，其中两名患者达到终末期肾病 ( ESRD)。从活检到 ESRD 的中位时间为 81 个月。多变量分析显示，活检时和随访时肾功能不全的最佳相关性是肾小管萎缩和间质纤维化的程度（分别为 P = 0.0495 和 0.0341）。结论。 C1q 肾病属于 MCD/FSGS 的临床病理范围。尽管需要进一步研究来确定 C1q 沉积的病理机制，但我们假设它可能是肾小球蛋白尿背景下系膜运输增加的非特异性标志物。
Background. C1q nephropathy is a poorly understood and controversial entity with distinctive immunopathologic features. In order to better define the clinical-pathologic spectrum, we report the largest single-center series.Methods. Nineteen biopsies with C1q nephropathy were identified from among 8909 native kidney biopsies received from 1994 to 2002 (0.21%). Defining criteria included ( 1) dominant or co-dominant immunofluorescence staining for C1q, ( 2) mesangial electron dense deposits, and ( 3) no clinical or serologic evidence of systemic lupus erythematosus (SLE).Results. The 19 patients were predominantly African American (73.7%), female ( 73.7%), young adults and children ( range, 3 to 42 years; mean, 24.2 years). Presentation included nephrotic range proteinuria (78.9%), nephrotic syndrome (50%), renal insufficiency (27.8%), and hematuria (22.2%). No patient had hypocomplementemia or evidence of underlying autoimmune or infectious disease. Renal biopsy revealed focal segmental glomerulosclerosis (FSGS) in 17 ( including six collapsing and two cellular) and minimal-change disease (MCD) in two. All biopsies displayed co-deposits of immunoglobulin G (IgG), with more variable IgM (84.2%), IgA (31.6%), and C3 (52.6%). Foot process effacement varied from 20% to 100% (mean, 51%). Twelve of 16 patients with available follow-up received immunosuppressive therapy. One patient had complete remission of proteinuria and six had partial remission. Four patients with FSGS pattern had progressive renal insufficiency, including two who reached end-stage renal disease ( ESRD). Median time from biopsy to ESRD was 81 months. On multivariate analysis, the best correlate of renal insufficiency at biopsy and at follow-up was the degree of tubular atrophy and interstitial fibrosis ( P = 0.0495 and 0.0341, respectively).Conclusion. C1q nephropathy falls within the clinical-pathologic spectrum of MCD/FSGS. Although further studies are needed to determine the pathomechanism of C1q deposition, we hypothesize that it may be a non-specific marker of increased mesangial trafficking in the setting of glomerular proteinuria.