Valentini Gabriele, Marcoccia Antonella, Cuomo Giovanna, Iudici Michele, Vettori Serena
Rheumatology Unit, Second University of Napoli, Via S. Pansini 5, 80131 Napoli, Italy.
Curr Rheumatol Rev. 2014;10(1):38-44. doi: 10.2174/1573397110666140404001756.
For many years observational studies and clinical trials on systemic sclerosis (SSc) have been carried out only on patients who met the 1980 American College of Rheumatology-ACR preliminary classification criteria. However, this lead to the exclusion from all those studies of subset patients, particularly those with a limited cutaneous SSc-sine scleroderma subset, because, despite a diagnosis of SSc based on Raynaud's phenomenon (RP) associated with digital pitting scars/ulcers, or by sclerodactyly and telangiectasia and/or typical esophagopathy and/or interstitial fibrosis detected by High Resolution Computed Tomography of the lungs, they did not satisfy the classification criteria. In that setting, LeRoy and Medsger proposed to label as affected by limited SSc (lSSc) or early SSc, cases presenting with RP associated with an SSc-type nailfold capillary pattern and/or SSc-selective autoantibodies. In 2008, Koenig et al. validated these criteria and proposed to name early SSc, or pre-scleroderma, patients with RP and either a scleroderma marker autoantibody or typical capillaroscopy abnormalities or both, who had been clearly shown to have high probabilities but not the certainty to develop definite SSc during a 20-year follow-up. This definition has been recently challenged by the development of the new ACR/EULAR criteria for the classification of SSc. At present, to be labeled as affected by early SSc, or pre-scleroderma, a patient should suffer from RP associated with either scleroderma marker autoantibodies or typical capillaroscopy findings and should not meet the 2013 ACR/EULAR criteria for the classification of SSc nor should he/she meet the criteria for SSc sine scleroderma.
多年来，针对系统性硬化症（SSc）的观察性研究和临床试验仅在符合1980年美国风湿病学会（ACR）初步分类标准的患者中开展。然而，这导致所有这些研究都排除了一部分患者，特别是那些局限性皮肤型SSc-无硬皮病亚型的患者，因为尽管根据与指端凹陷性瘢痕/溃疡相关的雷诺现象（RP），或通过硬皮病样指（趾）、毛细血管扩张和/或典型食管病变和/或肺部高分辨率计算机断层扫描检测到的间质性纤维化诊断为SSc，但他们并不满足分类标准。在这种情况下，勒罗伊和梅兹格提议将出现与SSc型甲襞毛细血管模式和/或SSc选择性自身抗体相关的RP的病例标记为受局限性SSc（lSSc）或早期SSc影响。2008年，凯尼格等人验证了这些标准，并提议将出现RP且伴有硬皮病标记自身抗体或典型毛细血管镜异常或两者皆有的患者命名为早期SSc或硬皮病前期患者，这些患者在20年的随访中已被明确证明有很高的概率但并非一定会发展为明确的SSc。最近，SSc分类的新ACR/EULAR标准的制定对这一定义提出了挑战。目前，要被标记为受早期SSc或硬皮病前期影响，患者应患有与硬皮病标记自身抗体或典型毛细血管镜检查结果相关的RP，且不应符合2013年ACR/EULAR的SSc分类标准，也不应符合无硬皮病型SSc的标准。
