Ethnic Variations in Systemic Sclerosis Disease Manifestations, Internal Organ Involvement, and Mortality
Ethnic Variations in Systemic Sclerosis Disease Manifestations, Internal Organ Involvement, and Mortality
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DOI：
10.3899/jrheum.180042
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发表时间：
2019-09-01
影响因子：
3.9
通讯作者：
Johnson, Sindhu R.
中科院分区：
文献类型：
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作者：
Al-Sheikh, Haifa;Ahmad, Zareen;Johnson, Sindhu R.
Objective.一项多种族系统性硬化症（SSc）队列研究，旨在评估疾病表现、内脏受累和生存率的种族差异。纳入了1970年至2017年期间符合美国流变学学院/欧洲反流变学联盟SSc分类标准的成年人。自我报告的种族被归类为欧洲血统的白色，非洲-加勒比，西班牙裔，阿拉伯，东亚，南亚，第一民族，或波斯。主要结局是从诊断到全因死亡的时间。采用Kaplan-Meier生存曲线确定生存概率和中位生存时间。共评价了1005例受试者，其中大多数为欧洲裔白色人（n = 745，74%）、非洲裔加勒比人（n = 58，6%）、南亚人（n = 70，7%）和东亚人（n = 80，8%）。与欧洲血统的白色受试者相比，东亚人较少发生钙质沉着症（29% vs 9%，p = 0.002）和食管动力障碍（88% vs 69%，p = 0.002）;非洲裔加勒比人更常发生间质性肺病（31% vs 53%，p = 0.007）;原住民受试者更常患有弥漫性皮肤病（35% vs 56%，p = 0.02）和糖尿病（5% vs 33%，p = 0.03）。我们发现不同种族的短期生存率没有差异。西班牙裔受试者的长期生存率（81.3%，95% CI 63-100）优于欧洲裔白色受试者（55%，95% CI 51-60）。东亚人的中位生存时间最长（433年），阿拉伯人的中位生存时间最短（15年）。加勒比黑人和欧洲裔白色受试者的中位生存时间无显著差异（22.2 vs 22.6年）。观察到一些SSc疾病表现的种族差异。然而，这不会导致短期生存的显著差异，但可能影响长期生存。
Objective. A multiethnic systemic sclerosis (SSc) cohort study to evaluate ethnic variations in disease manifestations, internal organ involvement, and survival.Methods. Adults who fulfilled the American College of Rheumatology/European League Against Rheumatism classification criteria for SSc between 1970 and 2017 were included. Self-reported ethnicity was categorized as European-descent white, Afro-Caribbean, Hispanic, Arab, East Asian, South Asian, First Nations, or Persian. The primary outcome was the time from diagnosis to death from all causes. Survival probabilities and median survival times were determined using Kaplan-Meier survival curves.Results. There were 1005 subjects evaluated, the majority of whom were European-descent white (n = 745, 74%), Afro-Caribbean (n = 58, 6%), South Asian (n = 70,7%), and East Asian (n = 80, 8%). Compared to European-descent white subjects, East Asians less frequently had calcinosis (29% vs 9%, p = 0.002) and esophageal dysmotility (88% vs 69%, p = 0.002); Afro-Caribbeans more frequently had interstitial lung disease (31% vs 53%, p = 0.007); and First Nations subjects more frequently had diffuse cutaneous disease (35% vs 56%, p = 0.02) and diabetes (5% vs 33%, p = 0.03). We found no difference in the short-term survival across ethnicities. Hispanic subjects have better longterm survival (81.3%, 95% CI 63-100) compared to European-descent white subjects (55%, 95% CI 51-60). East Asians appear to have the longest median survival time (433 yrs) and Arabs the shortest median survival time (15 yrs). There was no significant difference in median survival times between Afro-Caribbean and European-descent white subjects (22.2 vs 22.6 yrs).Conclusion. Ethnic variations in some SSc disease manifestations are observed. however, this does not result in significant differences in short-term survival but may affect longterm survival.