Department of Pathology and Cell Biology and.
Division of Nephrology, Department of Medicine, Columbia University Irving Medical Center, New York, New York.
Clin J Am Soc Nephrol. 2019 Nov 7;14(11):1605-1615. doi: 10.2215/CJN.01570219. Epub 2019 Oct 25.
In 2012, the Systemic Lupus International Collaborating Clinics proposed that lupus nephritis, in the presence of positive ANA or anti-dsDNA antibody, is sufficient to diagnose SLE. However, this "stand-alone" kidney biopsy criterion is problematic because the ISN/RPS classification does not specifically define lupus nephritis. We investigated the combination of pathologic features with optimal sensitivity and specificity for the diagnosis of lupus nephritis.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Three hundred consecutive biopsies with lupus nephritis and 560 contemporaneous biopsies with nonlupus glomerulopathies were compared. Lupus nephritis was diagnosed if there was a clinical diagnosis of SLE and kidney biopsy revealed findings compatible with lupus nephritis. The control group consisted of consecutives biopsies showing diverse glomerulopathies from patients without SLE, including IgA nephropathy, membranous glomerulopathy, pauci-immune glomerulonephritis, membranoproliferative glomerulonephritis (excluding C3 GN), and infection-related glomerulonephritis. Sensitivity and specificity of individual pathologic features and combinations of features were computed.
Five characteristic features of lupus nephritis were identified: "full-house" staining by immunofluorescence, intense C1q staining, extraglomerular deposits, combined subendothelial and subepithelial deposits, and endothelial tubuloreticular inclusions, each with sensitivity ranging from 0.68 to 0.80 and specificity from 0.8 to 0.96. The presence of at least two, three, or four of the five criteria had a sensitivity of 0.92, 0.8, and 0.66 for the diagnosis of lupus nephritis, and a specificity of 0.89, 0.95, and 0.98.
In conclusion, combinations of pathologic features can distinguish lupus nephritis from nonlupus glomerulopathies with high specificity and varying sensitivity. Even with stringent criteria, however, rare examples of nonlupus glomerulopathies may exhibit characteristic features of lupus nephritis.
2012 年，系统性红斑狼疮国际合作临床组织提出，在存在抗核抗体或抗双链 DNA 抗体阳性的情况下，狼疮肾炎足以诊断为系统性红斑狼疮。然而，这种“孤立”的肾活检标准存在问题，因为 ISN/RPS 分类并未专门定义狼疮肾炎。我们研究了病理特征与狼疮肾炎诊断的最佳敏感性和特异性的结合。
设计、设置、参与者和测量：比较了 300 例狼疮肾炎连续活检和 560 例同期非狼疮性肾小球病活检。如果存在系统性红斑狼疮的临床诊断且肾活检显示符合狼疮肾炎的发现，则诊断为狼疮肾炎。对照组由来自无系统性红斑狼疮的患者的连续活检组成，显示出多种肾小球病，包括 IgA 肾病、膜性肾小球病、少免疫性肾小球肾炎、膜增生性肾小球肾炎（不包括 C3 GN）和感染相关的肾小球肾炎。计算了单个病理特征和特征组合的敏感性和特异性。
确定了狼疮肾炎的五个特征性特征：免疫荧光的“全屋”染色、强烈的 C1q 染色、肾小球外沉积物、内皮下和上皮下沉积物的联合以及内皮小管网状内含物，每种特征的敏感性范围为 0.68 至 0.80，特异性范围为 0.8 至 0.96。至少存在两种、三种或四种五种标准的情况下，诊断狼疮肾炎的敏感性分别为 0.92、0.8 和 0.66，特异性分别为 0.89、0.95 和 0.98。
总之，病理特征的组合可以以高特异性和不同的敏感性来区分狼疮肾炎和非狼疮性肾小球病。然而，即使采用严格的标准，少数非狼疮性肾小球病也可能表现出狼疮肾炎的特征性特征。
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