白塞病新的国际分类标准提高了诊断敏感度

作者:发布时间:2014/5/22 15:58:33
摘要   目的:白塞病(BD)是一种慢性、复发性、炎症性血管疾病,但无特异性病征。当前应用的国际研究小组(ISG)临床诊断标准的敏感度较低,需要对其进行重新评估。 
方法:由BD国际标准修订小组(27个国家参与)递交了来自2556个临床诊断患有BD的患者、及1163名对照人群(患有类似BD的疾病或最少拥有1项BD主要体征的患者)的数据。这些研究对象被随机分配到研究组和验证组。利用Logistic回归分析、“去除某一国数据”的交叉验证、及临床判断等方法,从研究组数据中开发出新的BD国际标准(ICBD)。在验证组患者中对现有标准及新标准的诊断效力进行验证。

 表2白塞病的国际标准评分系统:得分≥4提示诊断白塞病
 体征/症状     分数 
 眼部损害        2
生殖器溃疡     2 
口腔溃疡         2
皮肤损害         1 
神经系统表现 1 
血管表现         1
针刺试验阳性 1*
* 针刺试验是非必须的,最初的评分系统未包括其在内。但如果进行了针刺试验,且结果为阳性,则加上额外的1分。 

结果:在ICBD中,对眼部损害、口腔溃疡、及生殖器溃疡着三个症状每个赋值2分,而皮肤损害、中枢神经系统累及、及血管表现每个赋值1分,如针刺试验阳性,则赋值1分。如果患者最终得分达到或超过4分,则被诊断患有BD(表2)。在研究组中,ICBD的敏感度和特异度分别为93.9%和92.1%,而ISG标准的敏感度和特异度分别为81.2%和95.9%。在验证组中,ICBD的敏感度很稳定,为94.8%(95%CI:93.4-95.9%),明显高于ISG标准(85.0%);但ICBD的特异度(90.5%,95% CI: 87.9-92.8%)虽然也很高,但较ISG标准(96.0%)低。在那些最少90%的患者及对照人群均接受了针刺试验的国家中,当加上这额外的1分后,ICBD敏感度由95.5%上升到了98.5%,而特异度仅出现轻微下降(从92.1%降到91.6%)。
 结论:与ISG标准相比,利用多国数据开发出的新标准的敏感度得到了很大的提高,而特异度也维持在很高的水平。我们建议,ICBD标准可应用于BD诊断及分类。
 附原文:Abstract  OBJECTIVE: Behçet's disease (BD) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group (ISG) clinical diagnostic criteria led to their reassessment.METHODS: An International Team for the Revision of the International Criteria for BD (from 27 countries) submitted data from 2556 clinically diagnosed BD patients and 1163 controls with BD-mimicking diseases or presenting at least one major BD sign. These were randomly divided into training and validation sets. Logistic regression, 'leave-one-country-out' cross-validation and clinical judgement were employed to develop new International Criteria for BD (ICBD) with the training data. Existing and new criteria were tested for their performance in the validation set. RESULTS: For the ICBD, ocular lesions, oral aphthosis and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement and vascular manifestations 1 point each. The pathergy test, when used, was assigned 1 point. A patient scoring ≥4 points is classified as having BD. In the training set, 93.9% sensitivity and 92.1% specificity were assessed compared with 81.2% sensitivity and 95.9% specificity for the ISG criteria. In the validation set, ICBD demonstrated an unbiased estimate of sensitivity of 94.8% (95% CI: 93.4-95.9%), considerably higher than that of the ISG criteria (85.0%). Specificity (90.5%, 95% CI: 87.9-92.8%) was lower than that of the ISG-criteria (96.0%), yet still reasonably high. For countries with at least 90%-of-cases and controls having a pathergy test, adding 1 point for pathergy test increased the estimate of sensitivity from 95.5% to 98.5%, while barely reducing specificity from 92.1% to 91.6%.CONCLUSION: The new proposed criteria derived from multinational data exhibits much improved sensitivity over the ISG criteria while maintaining reasonable specificity. It is proposed that the ICBD criteria to be adopted both as a guide for diagnosis and classification of BD.
  引自 :Davatchi F, Assaad-Khalil S, Calamia KT, et al. The International Criteria for Behcet's Disease (ICBD): A collaborative study of 27 countries on the sensitivity and specificity of the new criteria. Journal of the European Academy of Dermatology and Venereology, 2014, 28(3): 338-347.