Cases of systemic scleroderma, nicknamed the “mummifying disease” because of the hardening of the skin and internal tissues it causes, are on the rise in Quebec, particularly among children and young adults. Environmental factors could be to blame, researchers say.
This is what a study published in July in the journal The Lancet Regional Health – America. From 1996 to 2019, the number of new cases of this potentially fatal autoimmune disease increased by approximately 3.3% per year among Quebecers of all ages. As for those aged 0 to 19, we are talking about an increase of approximately 9.5% per year for the same period.
“Systemic sclerosis used to be extremely rare in children. Now, it’s a little less so,” says Elena Netchiporouk, the study’s lead author and an assistant professor in McGill University’s Faculty of Medicine and Health Sciences. Provincially, the disease is estimated to affect about 29 people in 100,000 of all ages, she notes.
Several factors could explain the increase in new cases of this disease among young people and the rest of the population, she suggests, specifying that she does not have a “100% clear” answer to this question for the moment. The greater ease of diagnosing systemic scleroderma may be one, she says, in an interview with Duty.
Then, in general, this disease mainly affects older people, adds Elena Netchiporouk. “Some of the increase in new cases could be due to the fact that the population is aging.”
Environmental factors could also partly explain the increase in disease in children and other age groups, she continues. “I think we need to explore more on the side of air pollution and industrial pollution.”
She also points out that new cases of the disease are more concentrated in eastern and northern Quebec than in other regions. “It could potentially have some connection with industries that are located in those places and that can emit certain particles into the air,” says the professor.
Mortality down
While the number of new cases of systemic scleroderma is increasing in Quebec, the mortality rate has been falling for around twenty years, says Elena Netchiporouk.
“In 1996, a person with this disease was 4.2 times more likely to die than a Quebecer of the same age and sex who did not have systemic scleroderma,” she explains. In 2019, this rate was 2.6.
“I think it’s the arrival of better care, better treatments, better screening and early diagnosis that explain this improvement in mortality,” the professor emphasizes. Systemic sclerosis, however, remains a difficult disease to treat, adds Elena Netchiporouk.
According to her, it is therefore essential to conduct more studies to properly target what causes the disease. “We have already started to do this, but we must allow researchers to really understand the impact that the environment in which we live has on our health.”
The professor also says that it is essential to provide adequate care to populations in the regions most affected by the increase in new cases of systemic scleroderma, in particular by sending specialists there.
She also stresses the need to educate patients more about the actions they can take to reduce the severity of the disease. “If they smoke, we really need to support them in quitting smoking.”