Study paints unprecedented picture of morphea in Quebec

For the first time, a large-scale population-based study has provided a detailed picture of the epidemiology of localized scleroderma in Quebec. This rare autoimmune disease, also known as morphea, causes fibrosis of the skin and can sometimes affect underlying connective tissue, leading to esthetic and functional complications. Between 1996 and 2019, more than 6,000 cases of morphea were identified from administrative databases in the Quebec healthcare system. Age- and sex-standardized incidence was estimated at 3.25 per 100,000 person-years (95% CI: 3.17-3.33).“This shows that it's a rare disease, but not all that rare,” observed Dr. Elena Netchiporouk, institute scientist and dermatologist at the McGill University Health Centre in Montreal and author of the study.More common in womenWomen accounted for almost 75% of cases, and were mainly affected between the ages of 60 and 79. In men, the disease was more common after the age of 80. The mean age at diagnosis was 53.The study was published in the Lancet Regional Health – Americas.Incidence, measured using a negative binomial random walk model, changed over time. The number of new cases rose steadily until 2004, followed by a plateau, then a decline from 2013 onwards, especially after 2017. This increase could be partly linked to better recognition of the disease, while the more pronounced decline after 2017 may have been influenced by changes in medical billing modalities.Prevalence increased steadily, reaching 24.5 cases per 100,000 people (95% CI: 24.3-24.8) over the study period. This trend is comparable to that observed for other cutaneous autoimmune diseases. Some recent cases appear to be linked to medical treatments such as chemotherapy, radiotherapy or immunotherapy. Hot spots in QuebecSignificant geographical disparities in the disease were also revealed with a spatial analysis using the Besag-York-Mollié Poisson model. Incidence hotspots were observed in southern Quebec, particularly in the Outaouais and Montérégie regions, where standardized incidence ratios were up to four times higher than the provincial average. This suggests the possible influence of environmental factors.Various factors can cause DNA damage to cells, and when we have cell damage, our body goes on defense and will stimulate a cytokine called type 1 interferon," said Dr. Netchiporouk, who specializes in understanding the interaction between the environment and autoimmune skin diseases. “It is this cytokine that we see over-expressed in the skin of patients with active morphea. Several environmental factors can induce interferon."Research is continuing, notably through the Canadian Morphea Registry, which already has over 200 participants. People affected by morphea are invited to participate in order to advance knowledge and care of this disease.This article has been translated from its original French and first appeared on our sister site Profession Santé.

Study paints unprecedented picture of morphea in Quebec

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