A new, just published, large-scale, multicenter study indicates that while systemic scleroderma is much more common in women than men (about 6 to 1 according to this study), men have a significantly worse prognosis due to an increased risk of major lung and heart complications. Part of the reason for this is that men are more likely to present with diffuse systemic scleroderma where women were more likely to present with limited systemic scleroderma. Men also had a much higher risk of digital ulceration. Overall, the study indicated that men were almost twice as likely to die from scleroderma related complications than women during the study period.
The authors also noted that age of onset did not differ significantly between men and women, suggesting that female hormones are not a factor in disease susceptibility.
The study was done using data from the 2013 EULAR scleroderma trials. Two of the main goals of the study were to look at: 1) the influence of gender on initial disease diagnosis, and 2) examining the effects of gender on disease progression and survival.
One important note: this is an observational study of a large population (almost 10,000 patients) and the results apply to the overall population, not to individual patients. It is important to keep in mind that many men have limited scleroderma (I do) and many men who have scleroderma do very well, even with diffuse scleroderma. Scleroderma is a highly variable disease, effecting patients even with the same antibody type very differently over time.
Here is the full reference to this new study:
Elhai M, et al. Ann Rheum Dis 2014;0:1–7. doi:10.1136/annrheumdis-2014-206386