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美国西南部西班牙裔/拉丁裔人群中的系统性硬化症表现及临床结局

Systemic sclerosis manifestations and clinical outcomes in Hispanics/Latinos of the American Southwest.

作者信息

Nunez Sharon E, Ariza-Hutchinson Angie, Fields Roderick A, Vondenberg Jaime A, Patel Rosemina A, Emil N Suzanne, Muruganandam Maheswari, Gibb James I, Poole Janet L, Sibbitt Wilmer L

机构信息

Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA.

Department of Medicine, Rheumatology/Immunology, Cleveland Clinic, Cleveland, OH, USA.

出版信息

J Scleroderma Relat Disord. 2022 Jun;7(2):135-143. doi: 10.1177/23971983221086214. Epub 2022 Apr 10.

Abstract

OBJECTIVE

Certain Hispanic/Latino (Hispanic) populations have been reported to have higher rates and severity of systemic sclerosis; however, little is known of systemic sclerosis in the American Southwest. This study compared manifestations of systemic sclerosis in Hispanics with non-Hispanics of New Mexico.

METHODS

This cross-sectional longitudinal study included 109 systemic sclerosis patients followed over a mean of 12.6 ± 8.9 years. Subjects were repetitively evaluated including physical examination, echocardiography, chest imaging, and serologic testing and observed for complications. Disease characteristics and long-term outcomes were statistically compared between self-identified Hispanic and non-Hispanic subjects.

RESULTS

A total of 73 (67%) systemic sclerosis subjects were Hispanic and 36 (33%) were non-Hispanic. The cohorts were similar in mean age, age of systemic sclerosis onset, limited versus diffuse cutaneous systemic sclerosis, telangiectases, gastroesophageal reflux disease, Raynaud's phenomenon, autoantibody profile, interstitial lung disease, pulmonary hypertension, scleroderma renal crisis, mortality, and comorbid malignancy (all  > 0.05). However, the standardized mortality ratio was increased in both cohorts relative to age-adjusted mortality: Hispanic: 2.08, confidence interval (1.94-2.24); non-Hispanic: 1.56, confidence interval (1.46-1.68). Furthermore, the standardized incidence ratio for malignancy was increased in both cohorts: Hispanic: 1.45, confidence interval (1.35-1.56); non-Hispanic: 1.24, confidence interval (1.16-1.34). The mean age of cancer diagnosis occurred at a significantly younger age in Hispanics (Hispanics: 53.1 ± 9.7 years; non-Hispanics 63.7 ± 7.9 years; 95% confidence interval: -19 ⩽ 10.6 ⩽ 2.2;  = 0.016).

CONCLUSION

Systemic sclerosis phenotype, autoantibodies, complications, outcomes, malignancy rates, and mortality are generally similar between Hispanics and non-Hispanics with systemic sclerosis in the American Southwest. However, age-adjusted comorbid malignancy and mortality rates are significantly increased in both groups.

摘要

目的

据报道,某些西班牙裔/拉丁裔(西班牙裔)人群系统性硬化症的发病率和严重程度较高;然而,对于美国西南部的系统性硬化症情况知之甚少。本研究比较了新墨西哥州西班牙裔和非西班牙裔系统性硬化症的表现。

方法

这项横断面纵向研究纳入了109例系统性硬化症患者,平均随访12.6±8.9年。对受试者进行反复评估,包括体格检查、超声心动图、胸部影像学检查和血清学检测,并观察并发症情况。对自我认定的西班牙裔和非西班牙裔受试者的疾病特征和长期结局进行统计学比较。

结果

共有73例(67%)系统性硬化症受试者为西班牙裔,36例(33%)为非西班牙裔。两组在平均年龄、系统性硬化症发病年龄、局限性与弥漫性皮肤型系统性硬化症、毛细血管扩张、胃食管反流病、雷诺现象、自身抗体谱、间质性肺疾病、肺动脉高压、硬皮病肾危象、死亡率和合并恶性肿瘤方面均相似(均>0.05)。然而,相对于年龄调整后的死亡率,两组的标准化死亡率均有所升高:西班牙裔:2.08,置信区间(1.94 - 2.24);非西班牙裔:1.56,置信区间(1.46 - 1.68)。此外,两组的恶性肿瘤标准化发病率均有所升高:西班牙裔:1.45,置信区间(1.35 - 1.56);非西班牙裔:1.24,置信区间(1.16 - 1.34)。西班牙裔癌症诊断的平均年龄显著更年轻(西班牙裔:53.1±9.7岁;非西班牙裔63.7±7.9岁;95%置信区间:-19⩽10.6⩽2.2;P = 0.016)。

结论

在美国西南部,患有系统性硬化症的西班牙裔和非西班牙裔在系统性硬化症表型、自身抗体、并发症、结局、恶性肿瘤发生率和死亡率方面总体相似。然而,两组的年龄调整后合并恶性肿瘤和死亡率均显著升高。

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