Systemic sclerosis & Raynaud's

Increased incidence of sudden cardiac death in systemic sclerosis: data from a nationwide cohort study

Semin Arthritis RheumPublished Prospective cohort study

Authors' conclusion

SCD occurred in 1.9% of a large SSc cohort, with an incidence rate of 300/100,000PY, representing a significant increased frequency compared to the general population.

Study context

Population
Individuals with systemic sclerosis (SSc) meeting ACR/EULAR criteria from the Australian Scleroderma Cohort Study
Participants
1,708 individuals10,650 person-years
Exposure
Presumed sudden cardiac death (SCD) vs overall cohort
  • SCDn = 32
Outcome
Incidence of sudden cardiac death (SCD)
Methodological detailsnationwide, multivariable Cox proportional hazard modelling
  • Nationwide
  • Multivariable Cox proportional hazard modelling

Main finding

As reported in the abstract

SCD incidence

SCD events

32

1.9%

Incidence rate

300/100,000 person-years

Observational data: associations, not effects.

Further findings

History of CAD

P < 0.001

Reduced LVEF

P = 0.004

Elevated NT-proBNP

P = 0.042

SSc heart involvement

P = 0.074

Factors associated with SCD (multivariable Cox model)

HR (95% CI)P value
Older age1.15 (1.10–1.20)P < 0.001
CAD2.28 (1.04–5.00)P = 0.040
Reduced LVEF2.89 (1.06–7.90)P = 0.038
Smoking history2.80 (1.21–6.49)P = 0.016

Original publication

Increased incidence of sudden cardiac death in systemic sclerosis: data from a nationwide cohort study. Semin Arthritis Rheum. Published 8 March 2026. doi:10.1016/j.semarthrit.2026.152964

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