系统性硬化症的分类诊断标准更新了

系统性硬化症的分类诊断标准更新了,2013年8月美国风湿学会以及欧洲风湿联盟共同完成更新了系统性硬化症的分类诊断标准。

系统性硬化症的分类诊断标准最晚的一次更新是在1980年。这30多年过去了,系统性硬化症的分类诊断标准又一次更新。系统性硬化症新的分类诊断标准提高了系统性硬化症诊断的敏感性,可以早期发现系统性硬化症以及硬皮病系统性硬化症新的分类诊断标准发表于Arthritis & Rheumatism杂志8月一期。

更新内容

系统性硬化症的分类诊断标准最主要的更新是「指端皮肤的增厚硬化如果向心性发展掌指关节可以诊断为系统性硬化症」。如果是无症状患者,另外还有新的7个标准可以对患者进行评分,以诊断是否患有系统性硬化症。这7个标准分别是如下几个方面:

  • 手指皮肤的增厚
  • 指端皮损
  • 血管扩张
  • 甲周毛细血管异常
  • 间质性肺炎或者肺动脉压增高
  • 雷诺现象
  • 系统性硬化症相关抗体

作者之一的Janet Pope博士称:「系统性硬化症常出现抗核抗体阳性以及雷诺现象。如果累及皮肤,常始于手指。常表现为肿胀或者皮肤紧张,而且系统性硬化症大多累及手指。如果身体某个部位出现增厚发硬,并不是系统性硬化症。」

另外,Janet Pope博士还说:「系统性硬化症疾病早期甲床会出现毛细血管扩张,用肉眼或者放大镜就可以观察到。当然甲床出现毛细血管扩张并不一定是系统性硬化症,还可能是单纯的毛细血管扩张症、胃食管返流症以及吞咽困难。如果疾病早期就进行相应的一些辅助检查,如肺功能和心脏彩超等,很多患者会符合系统性硬化症新的诊断标准」。

系统性硬化症2013年分类诊断标准的敏感性和特异性基于系统性硬化症患者与有类似硬皮病症状的患者进行得到的。

Janet Pope博士说:「现在临床医师所诊断的系统性硬化症中大约有20%左右的并不符合1980年的系统性硬化症分类诊断标准。这对一个非常见病并不是好消息,因为可能有1/5的系统性硬化症患者无法纳入研究。因此,系统性硬化症新的分类和诊断标准就势在必行。」

系统性硬化症新的分类诊断标准的发布,会使更多的患者早期诊断和治疗。

但是也有人对系统性硬化症2013分类诊断标准有异议。著名的系统性硬化症专家Marvin J Fritzler博士说:「我唯一的担心就是系统性硬化症2013分类诊断标准的抗核抗体一项是基于当地实验室的结果。这可能会引起不同的地方、不同的实验室、不同的检验人员做出的结果不一致。」

以下是全文摘要

2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative

Objective

The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc.

Methods

Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by 1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and 2) validating against the combined view of a group of experts on a set of cases with or without SSc.

Results

It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc.

Conclusion

The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease.

以下是全文

http://www.400gb.com/file/31804120

PS:改日我把全文看过后,再把主要内容发布出来。

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