Males With Scleroderma Found To Be More Affected By Pulmonary Hypertension

Patricia Inácio, PhD avatar

by Patricia Inácio, PhD |

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SSc and Pulmonary Hypertension

SSc and Pulmonary HypertensionIn a study entitled “A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study” published in the November issue of  Annals of the Rheumatic Diseases journal, the authors sought to understand the effects of gender on the systemic sclerosis phenotype and prognosis. 
 Specifically, they found males to be independent predictors of Systemic sclerosis associated life-threatening conditions, such as Pulmonary Hypertension and Heart Dysfunction.

Autoimmune diseases are often associated with sex gender bias. Systemic sclerosis (SSc) is an autoimmune disease of the connective tissue. SSc patients exhibit a thickening of the skin as a result of accumulation of collagen that mainly affects the skin, but can progress to the kidneys, heart, lungs and the gastrointestinal tract. In this case, the disease is termed as diffuse cutaneous scleroderma. A portion of SSc patients frequently develop pulmonary arterial hypertension as well. In fact, SSc prognosis is associated to the disease’s progression to other organs, such as the lungs.

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In this study, the authors analyzed the impact of gender on SSc using the largest European report on SSc patients, the EULAR scleroderma trials and research (EUSTAR) registry. The study included 9,182 patients with SSc, from which 1,321 patients were male. All of the enrolled patients fulfilled the criteria for SSc from the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR).

The authors discovered that in agreement with previous literature, women have a higher incidence for SSc when compared to males. Specifically, they found a sex ratio of six affected women for one man. The incidence however, is independent of age.

However, while more women may have Ssc, the disease is strikingly more severe in men, with a higher incidence of the diffuse cutaneous forms. The authors identified that the male gender is independently associated with pulmonary hypertension (PH), but additionally is also predictive of PH during follow-up studies. While idiopathic pulmonary arterial hypertension occurs more often in women, in the presence of SS, the link between SSc and PH is more prevalent in men. During follow-up studies, the male gender was also found to be an independent predictor of new episodes of heart dysfunction.

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The authors note that their results are the first showing that the male gender is an independent predictor of two life-threatening conditions — PH and heart dysfunction — with clear implications for SSc patients, since these two conditions are the two leading causes of death.

Thus, gender is a consideration when treating SSc patients, and the development of future therapeutics should be stratified by gender in addition to other factors, possibly leading to the development of gender-specific therapeutic treatments.