Pathology, Clinical Presentations, and Outcomes of C1q Nephropathy
Pathology, Clinical Presentations, and Outcomes of C1q Nephropathy
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DOI：
10.1681/asn.2007080929
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发表时间：
2008-11-01
影响因子：
13.6
通讯作者：
Jennette, J. Charles
中科院分区：
文献类型：
--
作者：
Vizjak, Alenka;Ferluga, Dugan;Jennette, J. Charles
C1q肾病是一种罕见的肾小球疾病，在免疫荧光显微镜下具有特征性。在本报告中，我们报告了72例C1q肾病患者的临床病理相关性和预后。该研究包括来自28名儿童和54名成人的82例肾脏活检，男性占多数（68%）。免疫荧光显微镜显示C1q在系膜和偶尔的肾小球毛细血管壁呈显性或共显性染色。53例中48例观察到电子致密沉积。光镜下未见病变（n = 27），局灶节段性肾小球硬化（FSGS; n = 11），增生性肾小球肾炎（n = 20）或其他各种病变（n = 14）。无病变组织学的患者临床表现为尿检查正常（7%），无症状血尿和/或蛋白尿（22%），肾病综合征（微小改变样病变；63%），经常复发。所有FSGS患者均表现为肾病综合征。增生性肾小球肾炎患者通常表现为慢性肾病（75%）或无症状尿异常（20%）。在有足够随访数据的患者中，77%的肾病综合征患者出现了微小病变样的完全缓解，33%的FSGS患者进展为终末期肾病，57%的增生性肾小球肾炎患者肾脏疾病保持稳定。总之，本研究确定了C1q肾病的两个主要临床病理亚群：(1)足细胞病伴微小改变样病变或FSGS，典型表现为肾病综合征；(2)典型免疫复合物介导的肾小球疾病，从无肾小球病变到多种形式的肾小球增生，典型表现为慢性肾病。C1q肾病的临床表现、组织学、预后和可能的发病机制是不同的。
C1q nephropathy is an uncommon glomerular disease with characteristic features on immunofluorescence microscopy. In this report, clinicopathologic correlations and outcomes are presented for 72 patients with C1q nephropathy. The study comprised 82 kidney biopsies from 28 children and 54 adults with male preponderance (68%). Immunofluorescence microscopy showed dominant or co-dominant staining for C1q in the mesangium and occasional glomerular capillary walls. Electron-dense deposits were observed in 48 of 53 cases. Light microscopy revealed no lesions (n = 27), focal segmental glomerulosclerosis (FSGS; n = 11), proliferative glomerulonephritis (n = 20), or various other lesions (n = 14). Clinical presentations in the patients who had no lesions histology were normal urine examination (7%), asymptomatic hematuria and/or proteinuria (22%), and nephrotic syndrome (minimal change-like lesion; 63%), which frequently relapsed. All patients with FSGS presented with nephrotic syndrome. Those with proliferative glomerulonephritis usually presented with chronic kidney disease (75%) or asymptomatic urine abnormalities (20%). Of the patients with sufficient follow-up data, complete remission of the nephrotic syndrome occurred in 77% of those with a minimal change-like lesion, progression to end-stage renal disease occurred in 33% of those with FSGS, and renal disease remained stable in 57% of those with proliferative glomerulonephritis. In conclusion, this study identified two predominant clinicopathologic subsets of C1q nephropathy: (1) Podocytopathy with a minimal change-like lesion or FSGS, which typically presents with nephrotic syndrome, and (2) a typical immune complex-mediated glomerular disease that varies from no glomerular lesions to diverse forms of glomerular proliferation, which typically presents as chronic kidney disease. Clinical presentation, histology, outcomes, and presumably pathogenesis of C1q nephropathy are heterogeneous.