Division of Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil.
Universidade Federal de Alfenas, Alfenas, Brazil.
Clin Rheumatol. 2020 Jan;39(1):9-17. doi: 10.1007/s10067-019-04575-5. Epub 2019 May 7.
African-Brazilians comprise a group of blacks and "pardos." As racial differences can be associated with distinct presentations, we evaluated the clinical and serological associations of African-Brazilians with systemic sclerosis (SSc).
Sera from 260 adult SSc patients (203 whites and 57 African-Brazilians) were evaluated. Patients with overlap syndromes were excluded. Clinical and demographic data were obtained from an electronic register database. Laboratory analysis included the following: anti-CENP-A/CENP-B, Scl70, RNA polymerase III, Ku, fibrillarin, Th/To, PM-Scl75, and PM-Scl100 by line immunoassay and anti-nuclear antibodies (ANA) by indirect immunofluorescence (IIF) on HEp-2 cells.
African-Brazilian SSc patients presented shorter disease duration (12.8 ± 6.5 vs. 15.9 ± 8.1 years, p = 0.009), higher frequency of nucleolar ANA pattern (28% vs. 13%, p = 0.008), and lower frequencies of centromeric ANA pattern (14% vs. 29%, p = 0.026) and CENP-B (18% vs. 34%, p = 0.017), as well as an association with severe interstitial lung disease (58% vs. 43%; p = 0.044). Further comparison of ethnic groups according to subsets revealed that diffuse SSc African-Brazilian patients presented higher frequency of pulmonary hypertension (p = 0.017), heart involvement (p = 0.037), nucleolar ANA pattern (p = 0.036), anti-fibrillarin antibodies (p = 0.037), and higher mortality (48% vs. 19%; p = 0.009). A different pattern was observed for the limited subset with solely a lower frequency of esophageal involvement (p = 0.050) and centromeric ANA pattern (p = 0.049). Survival analysis showed that African-Brazilians had a higher mortality, when adjusted for age, gender, and clinical subset (RR 2.06, CI 95% 1.10-3.83, p = 0.023).
African-Brazilians have distinct characteristics according to clinical subset and an overall more severe SSc than whites, similar to the blacks from other countries.Key Points • African-Brazilian SSc patients were associated with severe interstitial lung disease and nucleolar ANA pattern when compared to white SSc patients. • When disease subsets were considered, African-Brazilian patients with diffuse SSc presented association with pulmonary hypertension, heart involvement, nucleolar ANA pattern, and anti-fibrillarin antibodies. • White SSc patients were associated with centromeric ANA pattern. • Survival analysis at 5, 10, 15, and 20 years, adjusted for age, gender, and disease subset, was significantly worse in African-Brazilian SSc patients.
非裔巴西人由黑人和“pardo”（混血儿）组成。由于种族差异可能与不同的表现相关，我们评估了非裔巴西人系统性硬化症（SSc）的临床和血清学关联。
评估了 260 名成年 SSc 患者（203 名白人，57 名非裔巴西人）的血清。排除重叠综合征患者。从电子登记数据库中获取临床和人口统计学数据。实验室分析包括：抗着丝粒蛋白 A/CENP-B、Scl70、RNA 聚合酶 III、Ku、核仁纤维蛋白、Th/To、PM-Scl75 和 PM-Scl100 的线性免疫分析，以及核抗体（ANA）的间接免疫荧光（IIF）在 Hep-2 细胞上。
与白人 SSc 患者相比，非裔巴西人 SSc 患者的疾病病程更短（12.8±6.5 与 15.9±8.1 年，p=0.009），核仁 ANA 模式的频率更高（28%与 13%，p=0.008），着丝粒 ANA 模式（14%与 29%，p=0.026）和 CENP-B（18%与 34%，p=0.017）的频率较低，与严重的间质性肺病（58%与 43%；p=0.044）相关。根据亚组进一步比较种族群体，发现弥漫性 SSc 非裔巴西患者的肺动脉高压（p=0.017）、心脏受累（p=0.037）、核仁 ANA 模式（p=0.036）、抗核仁纤维蛋白抗体（p=0.037）和更高的死亡率（48%与 19%；p=0.009）的发生率更高。在局限性亚组中观察到了不同的模式，仅表现为食管受累（p=0.050）和着丝粒 ANA 模式（p=0.049）的频率较低。生存分析显示，在调整年龄、性别和临床亚组后，非裔巴西人死亡率更高（RR 2.06，95%CI 1.10-3.83，p=0.023）。
与白人 SSc 患者相比，非裔巴西人具有不同的临床特征，总体上比白人患者表现出更严重的 SSc，与来自其他国家的黑人相似。关键点：• 与白人 SSc 患者相比，非裔巴西人 SSc 患者与严重的间质性肺病和核仁 ANA 模式相关。• 当考虑疾病亚组时，弥漫性 SSc 的非裔巴西患者与肺动脉高压、心脏受累、核仁 ANA 模式和抗核仁纤维蛋白抗体相关。• 白人 SSc 患者与着丝粒 ANA 模式相关。• 调整年龄、性别和疾病亚组后，非裔巴西人 SSc 患者在 5、10、15 和 20 年的生存分析明显较差。
