Systemic sclerosis & Raynaud's
Systemic Sclerosis Sine Scleroderma: A Time of Reappraisal
Authors' conclusion
Published data on ssSSc vary widely on prevalence, clinical expression, and prognosis, partly due to underdiagnosis and misclassification.
Study context
- Population
- SSc cohorts with data on SSc sine scleroderma (systemic sclerosis sine scleroderma (ssSSc)), studies published 1976–2023
- Participants
- 25,455 participants35 studies
- Outcome
- Prevalence and severity of ssSSc
Methodological detailssystematic literature review
- Systematic literature review
Pooled result
As reported in the abstract
Prevalence of ssSSc
Mean prevalence of ssSSc (range 0-23%); different definitions used
almost 10%
Cross-sectional prevalence in the largest study
13%
Further pooled results
Reclassification of ssSSc into lcSSc or dcSSc (5 studies, follow-up up to 9 years)
- Reclassification range
- 0-28%
Organ involvement in ssSSc
- ILD (range 9.3-59.1%)
- 46%
- PAH (range 5.9-24.6%)
- 15%
- Scleroderma renal crisis (range 1.6-24.6%)
- 5%
- Cardiac diastolic dysfunction (range 1.8-40.7)
- 26.5%
Survival
- Survival comparable to lcSSc and better than dcSSc
Original publication
Systemic Sclerosis Sine Scleroderma: A Time of Reappraisal. J Rheumatol. Published 1 July 2024. doi:10. 3899/ jrheum. 2023-1113
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