Gender-related differences in systemic sclerosis

Abstract

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.

Keywords: Epidemiology; Gender: Men, Women; Mortality; Organ-involvement; Pathogenesis; Scleroderma; Systemic sclerosis; Treatment.