Frerix M, Meier F M P, Müller-Ladner U
Lehrstuhl für Innere Medizin mit Schwerpunkt Rheumatologie, Justus-Liebig-Universität Gießen, Gießen, Deutschland,
Z Rheumatol. 2013 Dec;72(10):954-9. doi: 10.1007/s00393-013-1194-x.
Increasing knowledge about the rare disease systemic sclerosis (SSc) and improved diagnostic methods in recent decades has led to the possibility of diagnosing systemic sclerosis in earlier disease stages. In this review, we describe the evolution of diagnostic and classification criteria for SSc, beginning with the preliminary ARA criteria for the classification of SSc in 1980, then presenting the criteria for limited and diffuse cutaneous SSc by LeRoy et al. in 1988 and 2001, and finishing with a discussion of the recently published new ACR-EULAR classification criteria in 2013. In addition, we seize the ongoing discussion about the intermediate subtype of SSc and highlight whether the two special subtypes CREST syndrome as well as SSc sine scleroderma are similar or different from the limited cutaneous SSc subtype. Finally, we address the question when a patient should be referred to the rheumatologist and discuss potential red flags for early diagnosis of systemic sclerosis.
近几十年来,人们对罕见病系统性硬化症(SSc)的了解不断增加,诊断方法也有所改进,这使得在疾病早期阶段诊断系统性硬化症成为可能。在本综述中,我们描述了SSc诊断和分类标准的演变,从1980年美国风湿病学会(ARA)初步制定的SSc分类标准开始,接着介绍了1988年和2001年勒罗伊等人提出的局限性和弥漫性皮肤型SSc的标准,最后讨论2013年最近发布的新的美国风湿病学会(ACR)/欧洲抗风湿病联盟(EULAR)分类标准。此外,我们抓住了关于SSc中间亚型的持续讨论,并强调了两种特殊亚型,即CREST综合征以及无硬皮病的系统性硬化症与局限性皮肤型SSc亚型是相似还是不同。最后,我们探讨了患者何时应转诊给风湿病专家的问题,并讨论了系统性硬化症早期诊断的潜在警示信号。