Background:The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in SSc patients, and contradictory results have often been observed.Objectives:The aim of the study was to assess differences in disease manifestations in a cohort of SSc patients according to gender.Methods:We performed a retrospective observational study using data extract from the EULAR scleroderma trials and research (EUSTAR) cohort 096.We looked at sex influence on disease characteristics at baseline and then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival.Results:173 patients with SSc were available for the baseline analyses. Males were older (52,96 vs 45,88, p=0.009), were more likely to smoke (73% vs 7%, p<0,001), had more frequent diffuse skin involvement (73,1% vs 56,5%,p<0.01), higher modified Rodnan skin score (34,61% vs 17%, p=0.01) and activity score(84,62% vs 46,26%,p<0.001) and were more often associed with positive acute phase reactants (65,38% vs 38,77%, p=0.01). Severe interstitial lung disease was more common in males (59,09% vs 27,53%, p=0.003), also the presence of tendon friction rubs was more frequent in this sex group (23,07% vs 8,84%, p=0.032).In the longitudinal analysis after a mean follow-up of 3,5(±0,65) years, male sex was associated with a higher risk of scleroderma renal crisis (OR:9.45 (1.49 to 59.69); p=0.004), digital contractures (OR:8,2 (3,1 to 21,9); p<0,001), arrhythmias (OR:3,37 (1.36 to 8,34); p=0.006), pulmonary fibrosis (OR: 3.56, (1.51 to 8.41); p=0.003), pulmonary hypertension (OR: 3.01 (1.19 to 7,59); p=0.016), severe vascular involvement (OR:2,86, (1,22 to 6,73); p=0.013) and low ventricular ejection fraction (OR: 2,84, (1.2 to 6,73); p=0.014). Males had significantly reduced survival time after diagnosis (p=0,004). The most frequent causes of death were scleroderma renal crisis in males and pulmonary hypertension in females.Conclusion:Although more common in women, SSc appears as strikingly more severe in men. Our results demonstrate a higher risk of severe organ involvement and poor prognosis in men. These results raise the point of including sex in the management and the decision-making process.Disclosure of Interests:None declared
