Department of Internal Medicine, National Scleroderma Center, Hôpital Claude Huriez, Université de Lille, France.
Clin Rev Allergy Immunol. 2011 Apr;40(2):78-83. doi: 10.1007/s12016-010-8198-y.
As the diagnosis of systemic sclerosis (SSc) is generally suggested by the presence of Raynaud's phenomenon followed by typical skin thickening associated with the presence of additional extracutaneous features, capillaroscopic abnormalities, and characteristic autoantibodies, the first classification criteria, published by the American Rheumatism Association in 1980, were based only on clinical and chest X-ray items. As a consequence, 10% to 20% of the patients did not meet these criteria. In 1988, an international consensus was reached resulting in the proposal of a new and more practical classification based on the judgment and clinical practice of an expert panel. This classification introduced the SSc nail fold capillaroscopy abnormalities (dilation and/or avascular areas) and specific antinuclear antibodies. Two subsets of SSc emerged from discussions: diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc). The calcifications, Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia (CREST) syndrome can be considered an lcSSc. In 2001, LeRoy and Medsger, realizing the shortcomings of the 1988 subsets in being too exclusive and taking advantage of increased experience with nail fold capillaroscopy and autoantibody determination, proposed criteria for an additional early or limited subset of SSc (lSSc), to supplement the previously recognized lcSSc and dcSSc forms. Patients with lSSc must have Raynaud's phenomenon and SSc-specific nail fold capillary changes and/or SSc-specific autoantibodies. Some lSSc patients who have no cutaneous involvement but common SSc nail fold capillaroscopy abnormalities, specific antinuclear antibodies, and visceral involvement are sometimes called SSc sine scleroderma. Whether or not lSSc and SSc sine scleroderma are the same or two different subsets is currently not known.
由于系统性硬化症（SSc）的诊断通常是基于雷诺现象的存在，随后是典型的皮肤增厚，伴有额外的皮肤外特征、毛细血管异常和特征性自身抗体，因此，1980 年美国风湿病学会发布的第一个分类标准仅基于临床和胸部 X 线项目。因此，10%到 20%的患者不符合这些标准。1988 年，达成了一项国际共识，提出了一种新的、更实用的分类方法，基于专家组的判断和临床实践。这种分类方法引入了 SSc 甲襞毛细血管异常（扩张和/或无血管区）和特定的核抗体。从讨论中出现了两种 SSc 子集：弥漫性皮肤 SSc（dcSSc）和局限性皮肤 SSc（lcSSc）。钙化、雷诺现象、食管低动力、硬皮病和毛细血管扩张（CREST）综合征可被视为局限性 SSc。2001 年，LeRoy 和 Medsger 意识到 1988 年分类标准过于排他，并且利用甲襞毛细血管镜检查和自身抗体检测的经验增加，提出了 SSc 的另一个早期或局限性子集（lSSc）的标准，以补充先前公认的局限性 SSc 和 dcSSc 形式。lSSc 患者必须有雷诺现象和 SSc 特异性甲襞毛细血管变化和/或 SSc 特异性自身抗体。一些没有皮肤受累但有常见的 SSc 甲襞毛细血管异常、特定核抗体和内脏受累的 lSSc 患者有时被称为无硬皮病的 SSc。lSSc 和无硬皮病的 SSc 是否相同或属于两个不同的子集目前尚不清楚。
