Systemic sclerosis & Raynaud's

Step forward in early recognition of systemic sclerosis: data from the Leiden Combined Care in Systemic Sclerosis (CCISS) cohort

RMD OpenPublished Prospective cohort study

Authors' conclusion

We observed a decrease in disease duration in Leiden CCISS cohort at cohort entry, possibly indicating more timely diagnosis of SSc.

Study context

Population
Dutch systemic sclerosis (SSc) patients fulfilling ACR/EULAR 2013 SSc criteria, Leiden CCISS cohort
Participants
643 patients
Exposure
Cohort-entry periods 2010-2013 vs 2014-2017 vs 2018-2021
  • 2010-2013n = 229reference
  • 2014-2017n = 207
  • 2018-2021n = 207
Outcome
Duration between onset of disease symptoms and cohort entry (early recognition of SSc) over time
Methodological detailssingle-centre cohort, stratified by sex and autoantibodies
  • Single-centre cohort
  • Stratified by sex and autoantibodies

Main finding

As reported in the abstract

Duration between symptom onset and cohort entry
  • Decreased over time in males and females, but always longer in females than in males

Observational data: associations, not effects.

Further findings

Outcome2010-20132018-2021
ILD at presentation in ATA+ patients25%19%

Digital ulcers (DU) at presentation

Proportion decreased, especially in ACA+ SSc

Interstitial lung disease (ILD) at presentation in ATA+ patients

Almost no ACA+ patients presented with ILD

Clinically meaningful ILD and diffuse cutaneous SSc (dcSSc) at presentation

Reduction observed

Overall 8-year survival by sex

Males
59% (95% CI 40% to 73%)
Females
89% (95% CI 82% to 93%)
Trend for improvement over time; always worse in males

Original publication

Step forward in early recognition of systemic sclerosis: data from the Leiden CCISS cohort. RMD Open. Published 7 April 2023. doi:10.1136/rmdopen-2022-002971

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