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. 2018 Dec 7:2:36.
doi: 10.1186/s41927-018-0043-6. eCollection 2018.

Trends in incidence, mortality, and causes of death associated with systemic sclerosis in Denmark between 1995 and 2015: a nationwide cohort study

Sheraz A Butt  1 , Jørgen L Jeppesen  1 , Christine Fuchs  2 , Mette Mogensen  2 , Merete Engelhart  3 , Christian Torp-Pedersen  4 , Gunnar H Gislason  5 , Søren Jacobsen  6 , Charlotte Andersson  5
Affiliations

Trends in incidence, mortality, and causes of death associated with systemic sclerosis in Denmark between 1995 and 2015: a nationwide cohort study

Sheraz A Butt et al. BMC Rheumatol. .

Abstract

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

Keywords: Cause of death; Epidemiology; Incidence; Mortality; Scleroderma; Systemic sclerosis.

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Conflict of interest statement

The study was approved by the Danish Data Protection Agency (ref. 2007-58-0015, int. ref. GEH-2014-018). As a retrospective registry-based study, Danish law does not require ethical approval [45]. The chart review for the validation process was approved by the Danish Patient Safety Authority and the permission was granted based on the assumption that only physicians at a given department reviewed its own patients’ charts.Not applicable.The authors declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Age and sex stratified incidence rates. Incidence rates per million for men (blue) and women (red) with 95% confidence intervals
Fig. 2
Fig. 2
Temporal trends in overall incidence and 1-year mortality rates between 1995 and 2015. Incidence rate (black line) and mortality rate (red line) with 95% confidence intervals of estimate
Fig. 3
Fig. 3
Temporal trends in sex-stratified incidence rates between 1995 and 2015. Incidence rate per million (blue and red lines) with 95% confidence intervals of estimate
Fig. 4
Fig. 4
Temporal trends in sex-stratified mortality rates between 1995 and 2015. 1-year mortality rates for men and women
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