title: Clinical and serological features of systemic sclerosis in a Chinese cohort.
abstract: Our goal was to study the prevalence of systemic sclerosis (SSc) subtypes, autoantibody profile, and pulmonary fibrosis in a large group of Han Chinese. Chinese SSc patients (n = 419) were recruited from a multicenter study including hospitals and outpatient clinics in China. All patients met the American College of Rheumatology classification criteria for SSc. Anti-topoisomerase (ATA), anti-centromere (ACA), anti-RNA polymerase III (anti-RNAP3), and anti-U1-ribonucleoprotein (anti-U1RNP) were detected utilizing commercially available kits. The clinical and autoantibody information in Chinese patients was compared to that in the US Caucasian patients (n = 834), recruited from the Genetics versus Environment in Scleroderma Outcome Study and Scleroderma Family Registry. Chi-square test was utilized for the abovementioned comparisons. Chinese patients showed 40.3 % limited (lcSSc) and 59.7 % diffuse (dcSSc) forms of SSc. ATA was found in 59.9 %, ACA in 13.4 %, anti-RNAP3 in 1.3 %, and anti-U1RNP in 18 % of Chinese SSc patients. Compared to US patients (65.1 % lcSSc, 34.9 % dcSSc, ATA in 18.7 %, ACA in 32.4 %, anti-RNAP3 in 17.4 %, and anti-U1RNP in 2.8 %), Chinese SSc patients are significantly higher in dcSSc and the frequencies of ATA and anti-U1RNP, but lower in ACA and anti-RNAP3. In addition, pulmonary fibrosis was observed in 78 % Chinese SSc patients and was strongly associated with the presence of ATA. The present study represents the first report of SSc features in a large group of Chinese patients. Clinical subtypes and the frequencies of SSc-related autoantibodies in Chinese SSc patients are significantly different from those in SSc patients of the US Caucasian descent.
authors.affiliation: Ministry of Education Key Laboratory of Contemporary Anthropology and State Key Laboratory of Genetic Engineering, School of Life Sciences, Fudan University, Shanghai, China.
authors.forename: Jiucun
authors.initials: J
authors.lastname: Wang
authors.forename: Shervin
authors.initials: S
authors.lastname: Assassi
authors.forename: Gang
authors.initials: G
authors.lastname: Guo
authors.forename: Wenzhen
authors.initials: W
authors.lastname: Tu
authors.forename: Wenyu
authors.initials: W
authors.lastname: Wu
authors.forename: Li
authors.initials: L
authors.lastname: Yang
authors.forename: Rong
authors.initials: R
authors.lastname: Xiao
authors.forename: Yongqiang
authors.initials: Y
authors.lastname: Zhao
authors.forename: Haiyan
authors.initials: H
authors.lastname: Chu
authors.forename: Jie
authors.initials: J
authors.lastname: Liu
authors.forename: Yanyun
authors.initials: Y
authors.lastname: Ma
authors.forename: Sarah J
authors.initials: SJ
authors.lastname: Kim
authors.forename: Li
authors.initials: L
authors.lastname: Jin
authors.forename: Maureen D
authors.initials: MD
authors.lastname: Mayes
authors.forename: Hejian
authors.initials: H
authors.lastname: Zou
authors.forename: Xiaodong
authors.initials: X
authors.lastname: Zhou
mesh_terms: D000974:Antibodies, Antinuclear / Q000097:blood; D044466:Asian People; D001323:Autoantibodies / Q000097:blood; D002681:China; D015331:Cohort Studies; D026942:DNA Topoisomerases / Q000276:immunology; D005006:Ethnicity; D005260:Female; D006801:Humans; D008297:Male; D011658:Pulmonary Fibrosis / Q000097:blood / Q000150:complications / Q000208:ethnology; D012595:Scleroderma, Systemic / Q000097:blood* / Q000150:complications / Q000208:ethnology*; D014481:United States; D044465:White People
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; C538950:anticentromere antibody; D026942:DNA Topoisomerases