Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease which is characterised by autoimmunity, widespread tissue fibrosis of the skin and internal organs, and vasculopathic alterations. SSc is more common in women but has a more severe expression of disease including internal organ-based complications and higher mortality in men. The extant literature shows that although important pathophysiological sex differences are present in SSc, behavioural differences (e.g. higher smoking rates in men) and occupational exposures may contribute to poorer outcomes in men with SSc. The higher death male death rate in the general population and greater prevalence of lung fibrosis are likely the key factors responsible for excess mortality found in men. Other important factors include (but are not limited to) a greater prevalence of the disease subset, delayed time to diagnosis, and higher disease activity in early disease in men. SSc carries a significant burden of disease-related morbidity; however, no qualitative studies to date have focussed on gender differences in SSc. The purpose of this review is to provide a comprehensive overview of gender differences in SSc including (but not limited to) epidemiology, pathophysiology, clinical expression of disease, mortality, SSc in transgender individuals, and psychosocial aspects of disease.

中文翻译：

---

全身性硬化症的性别相关差异。

系统性硬化症（SSc）是一种复杂的自身免疫结缔组织疾病，其特征是自身免疫，皮肤和内部器官的广泛组织纤维化以及血管病变。SSc在女性中更为常见，但其疾病表达更为严重，包括基于内部器官的并发症和男性较高的死亡率。现有文献表明，尽管在SSc中存在重要的病理生理性别差异，但行为差异（例如男性吸烟率较高）和职业暴露可能会导致SSc男性的结局较差。普通人群中较高的男性死亡率和较高的肺纤维化发生率很可能是导致男性死亡率过高的关键因素。其他重要因素包括（但不限于）该疾病亚型的较高患病率，延迟的诊断时间以及男性早期疾病中较高的疾病活动性。SSc承担了与疾病相关的发病率的重担；但是，迄今为止，尚无定性研究关注SSc中的性别差异。这篇综述的目的是全面概述SSc中的性别差异，包括（但不限于）流行病学，病理生理学，疾病的临床表现，死亡率，跨性别个体中的SSc以及疾病的社会心理方面。