Watad Abdulla, Bragazzi Nicola Luigi, Sharif Kassem, Shovman Ora, Gilburd Boris, Amital Howard, Shoenfeld Yehuda
Department of Medicine 'B', Sheba Medical Center, Tel Hashomer, Israel.
Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, Israel.
Isr Med Assoc J. 2017 Jul;19(7):424-428.
Anti-glomerular basement membrane (GBM) antibody disease, or Goodpasture's disease, is the clinical manifestation of the production of anti-GBM antibodies, which causes rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. Anti-GBM antibody detection is mandatory for the diagnosis of Goodpasture's disease either from the serum or kidney biopsy. Renal biopsy is necessary for disease confirmation; however, in cases in which renal biopsy is not possible or is delayed, serum detection of anti-GBM antibody is the only way for diagnosis.
To assess the predictive value of positive anti-GBM antibodies in a clinical setting.
Data from anti-GBM antibody tests performed at one medical center between 2006 and 2016 were systematically and retrospectively retrieved. We recruited 1914 patients for the study. Continuous variables were computed as mean ± standard deviation, while categorical variables were recorded as percentages where appropriate. Sensitivity and specificity of anti-GBM titers were calculated. Kaplan-Meyer analysis was performed, stratifying survival according to the anti-GBM antibody titers.
Of the 1914 anti-GBM test results detected, 42 were positive, 23 were borderline, 142 were excluded, and 1707 results were negative. Male-to-female ratio was 1:1.2. Sensitivity of anti-GBM test was 41.2% while specificity was 85.4%. Concerning the Kaplan-Meyer analysis, overall survival was 1163.36 ± 180.32 days (median 1058 days).
Our study highlights the lack of sensitivity of serological testing of anti-GBM titers. Comparing survival curves, the survival correlated with anti-GBM titer only in a borderline way. Because highly sensitive bioassays are not routinely used in clinics, renal biopsy is still pivotal for Goodpasture's disease diagnosis.
抗肾小球基底膜(GBM)抗体疾病,即肺出血肾炎综合征,是抗GBM抗体产生的临床表现,可导致伴有或不伴有肺出血的快速进行性肾小球肾炎。抗GBM抗体检测对于肺出血肾炎综合征的诊断至关重要,无论是通过血清检测还是肾活检。肾活检对于疾病确诊是必要的;然而,在无法进行或延迟进行肾活检的情况下,血清抗GBM抗体检测是唯一的诊断方法。
评估临床环境中抗GBM抗体阳性的预测价值。
系统回顾性检索了2006年至2016年在一家医疗中心进行的抗GBM抗体检测数据。我们招募了1914名患者进行研究。连续变量计算为均值±标准差,分类变量在适当情况下记录为百分比。计算抗GBM滴度的敏感性和特异性。进行Kaplan-Meier分析,根据抗GBM抗体滴度对生存进行分层。
在检测的1914份抗GBM检测结果中,42份为阳性,23份为临界值,142份被排除,1707份结果为阴性。男女比例为1:1.2。抗GBM检测的敏感性为41.2%,特异性为85.4%。关于Kaplan-Meier分析,总生存期为1163.36±180.32天(中位数1058天)。
我们的研究突出了抗GBM滴度血清学检测缺乏敏感性。比较生存曲线,生存与抗GBM滴度仅呈临界相关。由于临床未常规使用高灵敏度生物测定法,肾活检对于肺出血肾炎综合征的诊断仍然至关重要。