Muntyanu Anastasiya, Aw Katherine, Kaouache Mohammed, Rahme Elham, Osman Mohamed, Baron Murray, Ghazal Stephanie, Netchiporouk Elena
Division of Experimental Medicine, McGill University Health Centre, Montreal, Quebec, Canada.
Division of Dermatology, University of Toronto, Toronto, Ontario, Canada.
Lancet Reg Health Am. 2024 Jun 8;35:100790. doi: 10.1016/j.lana.2024.100790. eCollection 2024 Jul.
Systemic sclerosis (SSc) is a systemic life-threatening autoimmune rheumatic disease. We aimed to assess the incidence, prevalence, mortality and spatiotemporal trends of SSc in Quebec, Canada with stratification by sex and age.
SSc cases were identified from Quebec populational databases from 1989 to 2019. Negative Binomial (NB) Generalized Linear Models were used for age-standardized incidence rates (ASIR) analyses and NB random walk for prevalence and mortality. A Poisson Besag-York-Mollié regression model was used for spatial analysis.
8180 incident SSc cases were identified between 1996 and 2019 with an average age of 57.3 ± 16.3 years. The overall ASIR was 4.14/100,000 person-years (95%, Confidence Interval (CI) 4.05-4.24) with a 4:1 female predominance. ASIR increased steadily over time with an Average Annual Percent Change (AAPC) of 3.94% (95% CI 3.49-4.38). While the highest incidence rates were in those aged 60-79 years old among females and >80 years old among males, the highest AAPC (∼10%) was seen in children. Standarized incidence ratios varied geographically between 0.52 to 1.64. The average prevalence was 28.96/100,000 persons (95% CI 28.72-29.20). The Standardized Mortality Ratio (SMR) decreased from 4.18 (95% CI 3.64-4.76) in 1996 to 2.69 (95% CI 2.42-2.98) in 2019. Females had a greater SMR until 2007 and males thereafter. The highest SMR was in children and young adults [31.2 (95% CI 8.39-79.82) in the 0-19-year age group].
We showed an increasing trend in SSc incidence and prevalence and a decline in SMR over a 25-year period in Quebec. An uneven geographic distribution of SSc incidence was demonstrated.
National Scleroderma Foundation, Canadian Dermatology Foundation/Canadian Institutes of Health Research.
系统性硬化症（SSc）是一种危及生命的全身性自身免疫性风湿性疾病。我们旨在评估加拿大魁北克省系统性硬化症的发病率、患病率、死亡率及时空趋势，并按性别和年龄进行分层。
从魁北克省人口数据库中识别出1989年至2019年期间的系统性硬化症病例。使用负二项式（NB）广义线性模型进行年龄标准化发病率（ASIR）分析，使用NB随机游走模型分析患病率和死亡率。采用泊松贝萨格 - 约克 - 莫利埃回归模型进行空间分析。
1996年至2019年期间共识别出8180例系统性硬化症确诊病例，平均年龄为57.3±16.3岁。总体年龄标准化发病率为4.14/100,000人年（95%置信区间（CI）4.05 - 4.24），女性占比为4:1。随着时间的推移，年龄标准化发病率稳步上升，年均变化百分比（AAPC）为3.94%（95% CI 3.49 - 4.38）。女性中60 - 79岁年龄段发病率最高，男性中80岁以上年龄段发病率最高，而儿童的年均变化百分比最高（约10%）。标准化发病率在地理上的变化范围为0.52至1.64。平均患病率为28.96/100,000人（95% CI 28.72 - 29.20）。标准化死亡率（SMR）从1996年的4.18（95% CI 3.64 - 4.76）降至2019年的2.69（95% CI 2.42 - 2.98）。2007年前女性的标准化死亡率更高，之后男性更高。标准化死亡率最高的是儿童和年轻人（0 - 19岁年龄组为31.2（95% CI 8.39 - 79.82））。
我们发现魁北克省在25年期间系统性硬化症的发病率和患病率呈上升趋势，标准化死亡率呈下降趋势。系统性硬化症发病率存在地理分布不均的情况。
国家硬皮病基金会、加拿大皮肤病学基金会/加拿大卫生研究院。
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