Gender differences in systemic sclerosis: relationship to clinical features, serologic status and outcomes.
Gender differences in systemic sclerosis: relationship to clinical features, serologic status and outcomes.
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DOI：
10.5301/jsrd.5000209
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发表时间：
2016-05
影响因子：
2
通讯作者：
Feghali-Bostwick CA
中科院分区：
文献类型：
--
作者：
Peoples C;Medsger TA Jr;Lucas M;Rosario BL;Feghali-Bostwick CA
在许多结缔组织疾病和几乎所有系统性硬化症（SSc）病例系列中，有很强的女性优势。我们在一个大型单中心队列中比较了SSc患者的性别差异，包括人口统计学特征、疾病亚型、环境暴露、疾病特异性血清自身抗体、器官系统受累（频率和严重程度）和生存率。对皮肤亚群（弥漫性皮肤[dc]和局限性皮肤[lc]）进行调整。我们确定了区分女性和男性SSc患者的关键特征。女性在发病时往往更年轻，第一次就诊时疾病持续时间更长。女性更常患有lcSSc，如果存在重叠综合征，则最常见的是系统性红斑狼疮。相比之下，男性更常发生dcSSc并与肌炎重叠。女性更常有外周血管受累，但男性更严重。男性更经常吸烟，更频繁地接触环境。男性更常发生间质性肺疾病（ILD或肺纤维化），更严重。女性抗着丝粒抗体和男性抗拓扑异构酶I和抗u3rnp抗体的频率显著增加。男性SSc发病后5年生存率为73%，10年生存率为45%。最常见的死亡原因是男性ILD和女性肺动脉高压。性别差异可能是了解SSc的自然历史和发病机制的重要线索。
There is a strong female preponderance reported in many connective tissue diseases and in almost all systemic sclerosis (SSc) case series. We compared gender differences in SSc patients in a large single-center cohort, including demographic features, disease subtype, environmental exposures, disease-specific serum autoantibodies, organ system involvement (frequency and severity) and survival. Adjustment for cutaneous subset (diffuse cutaneous [dc] and limited cutaneous [lc]) was performed. We identified key characteristics which distinguished female from male SSc patients. Females were more frequently younger at disease onset with a longer disease duration at the time of their first visit. Females more often had lcSSc and, if an overlap syndrome was present, it was most often systemic lupus erythematosus. In contrast, males more frequently had dcSSc and overlap with myositis. Females more frequently had peripheral vascular involvement but in males it was more often severe. Males were more often cigarette smokers and more frequently had environmental exposures. Males more frequently had interstitial lung disease (ILD or pulmonary fibrosis) which was more severe. Females had a significantly increased frequency of anti-centromere antibody and males anti-topoisomerase I and anti-U3RNP antibody. Males had significantly reduced survival (73% at 5 years and 45% at 10 years after onset of SSc). The most frequent causes of death were ILD in males and pulmonary hypertension in females. Gender differences may be important clues to understanding the natural history and pathogenesis of SSc.