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Pulmonary hypertension is an uncommon complication of systemic sclerosis (SSc) and historically thought to slowly evolve in those with limited scleroderma over many years.
Researchers in Toronto have assessed the frequency of pulmonary arterial hypertension (PAH) noting that it occurs in 7% of SSc patients. They also report that men are more likely than women to develop PAH early, have more severe disease and worse survival rates in the first five years after diagnosis. (Citation source http://buff.ly/1T1G1hY)
While the ratio is often estimated at 3–4 women for every man with SSc, studies suggest that men affected with the disease have reduced survival and increased mortality. However, male sex does not independently impact SSc-PAH survival.
Men also had more severe SSc manifestations. Renal crisis appeared in 19 percent of men versus 8 percent of women. Interstitial lung disease was present in 67 percent of men and 48 percent of women, and diffuse cutaneous disease in 40 percent of men compared to 22 percent of women. Adjusting the analyses for the presence of interstitial lung disease did not affect the outcome of the results. On other SSc features, men and women were the same (Raynaud’s, digital ulcers, esophageal disorders, autoantibodies, WHO functional class, brain natriuretic peptide levels, comorbidities, or medications).