Background/Purpose: Although significant female gender predominance is seen in majority of large systemic sclerosis (SSc) cohorts, multiple studies have suggested that male gender generally associates with more severe disease and worse outcome. We explore disease characteristics, morbidity and mortality in male and female patients with SSc from a large single centre cohort.

Methods: We analysed a cohort of incident cases of SSc that developed over a period of 5 years and were followed up over a decade. Basic demographic, clinical and serological characteristics were recorded and comparison of survival and cumulative incidence of clinically-significant organ complications was made between male and female patients.

Results: A total of 398 SSc patients were included. Of those 54 (14%) were male. Age at onset was very similar in both male (mean±SD 50±13 years) and female (mean±SD 48±13 years) patients. A higher proportion of men had diffuse cutaneous (dc) subset of SSc compared to women – 48% (n=26) v 35% (n=120), although the difference showed only a trend towards statistical significance (p=0.069). Duration of Raynaud’s phenomenon (RP) with relation to onset of first non-RP symptom of SSc was significantly shorter in male patients with mean (range) of 40 (-12 to 576) months compared to 85 (-29 to 744) months in females (p=0.004). Comparison between frequencies of autoantibodies demonstrated significant differences only in anti-centromere antibody (ACA) positivity, which was twice as common among women – 28% (n=96) compared to 15% (n=8) in men (p=0.046).

There was a significantly higher proportion of male patients with some degree of pulmonary fibrosis (PF) confirmed on HRCT compared to females – 72% (n=39) v 49% (n=168), p=0.002. Analysis of cumulative incidence of clinically significant PF also demonstrated significantly higher frequencies among men. At 5 and 10 years from disease onset 32% and 43% of men had developed significant PF compared to 21% and 26% of women (p=0.017). There were no differences in incidence of pulmonary hypertension (PH), cardiac involvement or scleroderma renal crisis (SRC) between genders. At 5 years 6% of men and 4% of women had developed PH, 2% of men and 3% of women – cardiac SSc and 4% of men and 6% of women – SRC while at 10 years PH was found in 16% of men and 13% of women, SRC in 4% of men and 7% of women and there were no new cases of cardiac SSc in either group.

There was also no difference in survival between genders with 91% of both male and female patients being alive at 5 years. Mortality rate among men increased slightly between 5 and 10 years compared to women, although the difference did not reach statistical significance and at 10 years survival was 79% in women and 70% in men. There was also no difference in survival from development of significant organ complications between the genders.  

Conclusion: Although male patients have significantly higher incidence of PF, reflected by the lower frequency of ACA and show a trend towards greater frequency of diffuse SSc subset compared to females, there was no significant increase in overall mortality.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/gender-associated-differences-in-disease-characteristics-and-outcome-in-systemic-sclerosis/