Genth E, Krieg T
Rheumaklinik und Rheumaforschungsinstitut Aachen, Burtscheider Markt 24, 52066, Aachen, Germany.
Z Rheumatol. 2006 Jul;65(4):268-74. doi: 10.1007/s00393-006-0065-0.
Systemic sclerosis (SSc) is a polymorphic and heterogenic systemic disorder with inflammation, fibrosis and vascular damage. Early diagnosis and classification may be difficult if disease expression is oligosymptomatic (undifferentiated), presenting with only Raynaud's phenomenon or limited scleroderma. Scleroderma specific antinuclear autoantibodies, which are present early and persistently in about 90% of the patients with SSc, play an important taxonomic role. Scleroderma specific findings in nailfold capillary microscopy are sensitive and predictive for evolving SSc. An algorithm will be presented for the diagnosis and classification of SSc using clinical, capillaroscopic and serologic criteria, which are also useful for mixed or special forms of SSc. The 6th Outcome Measures in Rheumatology Clinical Trials (OMERACT) conference proposed different outcome measurements for clinical studies, however, for daily clinical practice there is as yet no consensus on status indices for disease activity, disease related damage or suitable prognostic criteria.
系统性硬化症（SSc）是一种具有多态性和异质性的全身性疾病，伴有炎症、纤维化和血管损伤。如果疾病表现为症状较少（未分化型），仅出现雷诺现象或局限性硬皮病，则早期诊断和分类可能会很困难。硬皮病特异性抗核自身抗体在约90%的SSc患者中早期出现且持续存在，发挥着重要的分类学作用。甲襞毛细血管显微镜检查中的硬皮病特异性表现对SSc的进展具有敏感性和预测性。将介绍一种使用临床、毛细血管镜和血清学标准对SSc进行诊断和分类的算法，这些标准对SSc的混合或特殊形式也很有用。第六届风湿病临床试验结果测量（OMERACT）会议提出了临床研究的不同结果测量方法，然而，对于日常临床实践，关于疾病活动状态指数、疾病相关损伤或合适的预后标准尚未达成共识。
