Butt Sheraz A, Jeppesen Jørgen L, Fuchs Christine, Mogensen Mette, Engelhart Merete, Torp-Pedersen Christian, Gislason Gunnar H, Jacobsen Søren, Andersson Charlotte
1Department of Internal Medicine and Cardiology, Amager Hvidovre, Glostrup Hospital, Valdemar Hansensvej 1-23, 2600 Glostrup, Denmark.
2Department of Dermatology, Bispebjerg Hospital, Bispebjerg Bakke 23, 2400 Copenhagen, Denmark.
BMC Rheumatol. 2018 Dec 7;2:36. doi: 10.1186/s41927-018-0043-6. eCollection 2018.
To investigate the incidence and the mortality-rates of systemic sclerosis (SSc), its primary causes of death, and the temporal trends in events in Denmark during the last decades.
Using the Danish National Patient Registry, we identified all persons aged ≥18 years with a first-time diagnosis of SSc (ICD-10 code M34, excluding M34.2) between 1995 and 2015.
A total of 2778 incident SSc cases were identified. The mean age at time of SSc diagnosis was 56 (standard deviation 15) years and 76% were women. The overall incidence rate (per 1,000,000 person-years) of diagnosed SSc was 24.4 (95% confidence interval 23.6-25.4), with a slight increase over the study period, age- and sex-adjusted incidence rate ratio 1.02 (95% confidence interval 1.01-1.02) per 1-year increase. The 1-year all-cause mortality rate per 100 person-years decreased from 6.1 (3.1-12.2) in 1995 to 5.3 (2.5-11.1) in 2015, sex- and age-adjusted hazard ratio 0.96 (95% CI 0.94-0.98) per 1-year increase. Over the period, the average age at SSc diagnosis increased and the proportion of women decreased, whereas the burden of comorbidities increased. One fifth of all deaths were attributable to cardiovascular causes, a fourth to pulmonary diseases, and 15% were due to cancer.
Within the last few decades, the incidence of SSc has increased and the 1-year mortality rate has decreased slightly in Denmark. Almost half of all deaths were attributable to cardiopulmonary causes.
调查过去几十年丹麦系统性硬化症（SSc）的发病率、死亡率、主要死因及事件的时间趋势。
利用丹麦国家患者登记系统，我们确定了1995年至2015年间所有首次诊断为SSc（国际疾病分类第十版代码M34，不包括M34.2）且年龄≥18岁的患者。
共确定了2778例SSc新发病例。SSc诊断时的平均年龄为56岁（标准差15），76%为女性。确诊SSc的总体发病率（每100万人年）为24.4（95%置信区间23.6 - 25.4），在研究期间略有上升，年龄和性别调整后的发病率比值比为每增加1年1.02（95%置信区间1.01 - 1.02）。每100人年的1年全因死亡率从1995年的6.1（3.1 - 12.2）降至2015年的5.3（2.5 - 11.1），性别和年龄调整后的风险比为每增加1年0.96（95%置信区间0.94 - 0.98）。在此期间，SSc诊断时的平均年龄增加，女性比例下降，而合并症负担增加。所有死亡中有五分之一归因于心血管原因，四分之一归因于肺部疾病，15%归因于癌症。
在过去几十年中，丹麦SSc的发病率有所上升，1年死亡率略有下降。几乎一半的死亡归因于心肺原因。
