Incidence, prevalence, and mortality of localized scleroderma in Quebec, Canada: a population-based study
Notice bibliographique
Résumé
Background: Localized scleroderma is an understudied autoimmune disease characterized by fibrosis of the skin and/or subcutaneous tissue. To date, only 6 articles reported on the incidence and/or prevalence estimates globally, with significant design limitations and risk of bias. None of the studies originated from Canada or investigated mortality/geospatial epidemiology. We aimed to study the incidence, prevalence, mortality and spatiotemporal trends of localized scleroderma in Quebec, Canada, stratified by sex and age. Methods: Quebec populational health administrative databases were used to identify localized scleroderma cases from 1989 to 2019. Crude incidence rate, age-standardized incidence rate, prevalence and mortality analyses were conducted using negative binomial random walk models. Spatial analyses were conducted using a Poisson Besag-York-Mollié regression model. Findings: There were 6063 incident localized scleroderma cases identified over the total period of the study (mean age 53.0, standard deviation [SD] 20.2 years at diagnosis). The overall age and sex-standardized incidence rate was 3.25/100,000 person-years [95% Confidence Interval (CI) 3.17-3.33]. Among 6063 incident cases, 4510 (74.4%) were female and 1553 (25.6%) were male, yielding a female-to-male ratio of approximately 3:1. In females, we noted an initial increase in age-standardized incidence rate followed by a plateau and a decrease after 2013 (average annual percent change -2.0 [95% CI -3.7 to -0.2]%). In males, a steady decrease in age-standardized incidence rate was observed (average annual percent change -3.3 [95% CI -5.0 to -1.8]%). The highest incidence rate was observed in the 60-79 year-old age group for females and the 80+ group for males. Age-standardized incidence rate varied geographically with hotspots identified in the south of Quebec. The average prevalence was 24.5/100,000 [95% CI 24.3-24.8]. The overall standardized mortality ratio was comparable for females (1.04 [95% CI 0.95-1.14]) and males (1.14 [95% CI 0.98-1.33]) and decreased steadily over time for both sexes (from 1.31 [95% CI 1.06-1.58] in 1996 to 0.81 [95% CI 0.66-0.98] in 2019). Standardized mortality ratio analysis revealed excess death only in females aged 40-59 years. Interpretation: From 1989 onward, we report an initial increase in the age and sex-standardized incidence rate of localized scleroderma in Quebec followed by a recent decrease after 2013, as well as a generally increasing prevalence from 1996 to 2019. Standardized mortality ratio analysis confirmed the clinical observation that localized scleroderma is a morbid rather than life-threatening disease. We demonstrate an uneven geographic distribution of localized scleroderma incidence in Quebec. Funding: This project was funded by Canadian Dermatology Foundation, National Scleroderma Foundation and Canadian Institutes of Health Research. Dr. Netchiporouk received FRQS Junior 1 Clinician Scientist Salary Award.
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Comment cette classification a été obtenuedéplier
Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,000 |
| Bibliométrie | 0,000 | 0,001 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule tête enseignante, pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».