Romanowska-Próchnicka Katarzyna, Walczyk Marcela, Olesińska Marzena
Department of General and Experimental Pathology, 2 Faculty of Medicine, Medical University of Warsaw, Poland; Department of Systemic Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.
Department of Systemic Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.
Reumatologia. 2016;54(6):296-305. doi: 10.5114/reum.2016.64906. Epub 2016 Dec 30.
Systemic sclerosis is a complex disease characterized by autoimmunity, vasculopathy and tissue fibrosis. Although most patients present with some degree of skin sclerosis, which is a distinguishing hallmark, the clinical presentation vary greatly complicating the diagnosis. In this regard, new classification criteria were jointly published in 2013 by American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR). A recent major development in the classification criteria is improved sensitivity, particularly for detecting early disease. The new criteria allow more cases to be classified as having systemic sclerosis (SSc), which leads to earlier treatment. Moreover it is clinically beneficial in preventing the disease progression with its irreversible fibrosis and organ damage. The aim of this review is to give insight into new classification criteria and current trends in the diagnosis of systemic sclerosis.
系统性硬化症是一种复杂的疾病,其特征为自身免疫、血管病变和组织纤维化。虽然大多数患者会出现一定程度的皮肤硬化,这是一个显著特征,但临床表现差异很大,使诊断变得复杂。在这方面,美国风湿病学会(ACR)和欧洲抗风湿病联盟(EULAR)于2013年联合发布了新的分类标准。分类标准最近的一项重大进展是提高了敏感性,尤其是在检测早期疾病方面。新的标准使更多病例能够被归类为患有系统性硬化症(SSc),从而实现更早的治疗。此外,在预防疾病进展及其不可逆的纤维化和器官损害方面具有临床益处。本综述的目的是深入了解系统性硬化症诊断的新分类标准和当前趋势。