Men at Risk of More Severe and Active Scleroderma at Disease Onset, EUSTAR Study Shows

Alice Melão, MSc avatar

by Alice Melão, MSc |

Share this article:

Share article via email
stem cell transplant

Men are at greater risk of more severe and active systemic sclerosis (SSc) than are women at early stages of the disease, a large review study shows.

The study, “Gender differences in early systemic sclerosis patients: a report from the EULAR scleroderma trials and research group (EUSTAR) database,” was published in the journal Clinical and Experimental Rheumatology.

SSc is a complex disorder than can manifest differently in patients. These differences can be present at disease onset and be sustained throughout disease course, with implications in overall patient outcomes. To improve patient care, a better understanding of factors that could contribute to these differences is important.

Researchers with the European Scleroderma Trials and Research group (EUSTAR) evaluated if gender could be one contributing factor for early differences in SSc presentation.

They reviewed the clinical records of 1,027 patients in the EUSTAR database with an onset of SSc symptoms onset of less than three years. Most were Caucasians, 80% were women, and 40% had diffuse cutaneous systemic sclerosis (dcSSc), a more severe disease form.

In early stages of SSc, men had signs and symptoms of more severe disease than did women, researchers found. Men had higher modified Rodnan skin scores (mRSS), a standard measure to evaluate skin thickness, and about 59% of them had active disease compared to 38% of the women.

Men were also more frequently positive for additional markers of more severe disease, including anti-Scl70 antibodies (53% versus 31% in women).

In this early disease population, about 26% of men had dcSSc, while only 11% of the women had this disease type. Men also more frequently had muscular and pulmonary involvement, with 44% having pulmonary fibrosis (versus 29% in women), impaired lung function (39% versus 26%) as seen in a forced vital capacity of less than 80%, and increased tendon friction rubs (23% versus 13%).

In contrast, women in early stage of SSc were more frequently positive for ACA antibodies than men, and most had limited cutaneous SSc (lcSSC), a milder and more common disease form.

To further explore potential hallmarks of early disease, researchers re-analyzed the clinical record of a subset of 461 patients who had the disease for less than 12 months. Among this very early onset group, 78% were women and about 34% had dcSSc.

Data showed that the disease presentation was similar to that reported above, with men most often showing signs and symptoms of more severe and active disease.

“Men, compared to women, presented data of more severe disease from very early stages of the disease, and these differences are much more evident in patients with early lcSSc,” the researchers wrote.

Researchers believes that these findings can be used in clinical practice to help identify SSc cases, as well as to monitor disease, and improve treatment decisions “in order to minimize SSc impact during the disease course.”

“Men with early SSc, even those with the lcSSc subset, should be carefully monitored, in order to detect visceral involvement even in the absence of other clinical symptoms,” the researchers suggested.