Systemic sclerosis & Raynaud's

Systemic Sclerosis Sine Scleroderma: A Time of Reappraisal

J RheumatolPublished Systematic review with qualitative synthesis

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

Composed by AI from the published abstract and checked against it. How Briefs are made →

Get them every week

New studies on this topic in My Week and in your Monday e-mail.

We e-mail you a sign-in link and a code. No password, no spam.