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

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

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系统性硬化症的表现和临床结果在美国西南部的西班牙/拉丁裔。
目的:据报道,某些西班牙裔/拉丁裔(西班牙裔)人群的系统性硬化症发病率和严重程度较高;然而,人们对美国西南部的系统性硬化症知之甚少。本研究比较了新墨西哥州西班牙裔与非西班牙裔的系统性硬化症表现。方法:横断面纵向研究纳入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、胸部成像和血清学检查,并观察并发症。疾病特征和长期结果在自我认定为西班牙裔和非西班牙裔受试者之间进行统计学比较。结果:73例(67%)系统性硬化症患者为西班牙裔,36例(33%)非西班牙裔。这些队列在平均年龄、系统性硬化症发病年龄、局限性与弥漫性皮肤系统性硬化症、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、死亡率和合并症恶性肿瘤方面相似(均p > 0.05)。然而,两个队列的标准化死亡率相对于年龄调整死亡率都有所增加:西班牙裔:2.08,可信区间(1.94-2.24);非西班牙裔:1.56,置信区间(1.46-1.68)。此外,两个队列中恶性肿瘤的标准化发病率均有所增加:西班牙裔:1.45,可信区间(1.35-1.56);非西班牙裔:1.24,置信区间(1.16-1.34)。西班牙裔患者癌症诊断的平均年龄明显较年轻(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:美国西南部拉美裔和非拉美裔系统性硬化症患者的系统性硬化症表型、自身抗体、并发症、预后、恶性肿瘤发生率和死亡率大体相似。然而,两组的年龄调整合并症恶性肿瘤和死亡率显著增加。
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