Ng Sue-Ann, Low Andrea Hsiu Ling
Department of Rheumatology and Immunology, Singapore General Hospital, Singapore.
Duke-National University of Singapore, Singapore.
J Scleroderma Relat Disord. 2022 Jun;7(2):98-109. doi: 10.1177/23971983221074749. Epub 2022 Feb 3.
Systemic sclerosis is a multisystemic autoimmune disease characterized by vasculopathy and fibrosis. Racial factors exert a significant influence on the epidemiology, clinical manifestations, antibody profile, mortality and genetic factors in systemic sclerosis. In this review, we examined Asian systemic sclerosis cohorts reported in Asia and multi-racial cohort studies to evaluate the disease characteristics and outcomes of systemic sclerosis in Asians. Asian patients have distinct genetic susceptibility to systemic sclerosis, younger age of systemic sclerosis onset, higher frequency of diffuse skin involvement, different autoantibody profiles such as higher frequency of anti-Scl70 and anti-U1-RNP antibodies, and more severe clinical phenotype. There was a suggestion of poorer survival among Asians that may be contributed by more severe disease, socioeconomic factors and differences in healthcare systems. Recognizing the influence of racial differences in systemic sclerosis disease course is important as it has implications for appropriate treatment, monitoring and prognostication.
系统性硬化症是一种以血管病变和纤维化为特征的多系统自身免疫性疾病。种族因素对系统性硬化症的流行病学、临床表现、抗体谱、死亡率和遗传因素有重大影响。在本综述中，我们研究了亚洲报道的亚洲系统性硬化症队列以及多种族队列研究，以评估亚洲人系统性硬化症的疾病特征和结局。亚洲患者对系统性硬化症有独特的遗传易感性，系统性硬化症发病年龄较轻，弥漫性皮肤受累频率较高，有不同的自身抗体谱，如抗Scl70和抗U1-RNP抗体频率较高，且临床表型更严重。有迹象表明亚洲人的生存率较低，这可能是由更严重的疾病、社会经济因素和医疗保健系统差异导致的。认识到种族差异对系统性硬化症病程的影响很重要，因为这对适当治疗监测和预后有影响。
