C1Q NEPHROPATHY - A DISTINCT PATHOLOGIC ENTITY USUALLY CAUSING NEPHROTIC SYNDROME
C1Q NEPHROPATHY - A DISTINCT PATHOLOGIC ENTITY USUALLY CAUSING NEPHROTIC SYNDROME
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DOI：
10.1016/s0272-6386(85)80150-5
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发表时间：
1985-01-01
影响因子：
13.2
通讯作者：
HIPP, CG
中科院分区：
文献类型：
--
作者：
JENNETTE, JC;HIPP, CG
采用直接免疫荧光显微镜对800例肾活检标本的肾小球C1q定位的存在、分布和强度进行了评估，并通过光镜和电镜进行了研究。确定了15例广泛（平均：3.6 + / 4 +），主要是系膜，C1q定位以及C3和免疫球蛋白，但没有系统性红斑狼疮的证据。病理上，该病变与狼疮性肾炎最为相似。将这15例C1q肾病患者的临床和病理数据与30例狼疮性肾炎和223例其他增生性肾小球肾炎患者的数据进行比较，发现C1q肾病患者在两组之间存在差异。15例C1q肾病患者平均年龄17.8岁，男性8例，女性7例，黑人9例，100%蛋白尿（平均7.5 gold）， 40%血尿，0%低补体血症，0%抗核抗体。电镜下，100%有系膜致密沉积物，20%有毛细血管壁致密沉积物，0%有内皮管网状包裹体。9例接受类固醇治疗的患者没有明确的蛋白尿缓解。我们认为C1q肾病是一种独特的临床病理实体，通常在大龄儿童和年轻人中引起类固醇抵抗性肾病综合征。
The presence, distribution, and intensity of glomerular C1q localization were evaluated by direct immunofluorescence microscopy in 800 renal biopsy specimens which were also studied by light and electron microscopy. Identified were 15 patients with extensive (mean: 3.6 + out of 4 +), predominantly mesangial, C1q localization along with C3 and immunoglobulins, but no evidence for systemic lupus erythematosus. Pathologically, this lesion most closely resembled lupus nephritis. Clinical and pathologic data from these 15 C1q nephropathy patients were compared to data from 30 lupus nephritis and 223 other proliferative glomerulonephritis patients, and the C1q nephropathy patients were found to be dissimilar to both groups. The 15 C1q nephropathy patients had an average age of 17.8 years, 8 males, 7 females, 9 Black, 100% had proteinuria (mean 7.5 gld), 40% hematuria, 0% hypocomplementemia, and 0% antinuclear antibodies. By electron microscopy, 100% had mesangial dense deposits, 20% capillary wall dense deposits, and 0% endothelial tubuloreticular inclusions. Nine patients treated with steroids had no definite resolution of proteinuria. We proposed that C1q nephropathy is a distinct clinicopathologic entity, usually causing steroid-resistant nephrotic syndrome in older children and young adults.