Systemic sclerosis & Raynaud's
Survival and prognostic factors from a multicentre large cohort of unselected Italian systemic sclerosis patients
Authors' conclusion
In the past decade, SSc patients have reached similar mortality of that expected in the Italian general population. Male gender, diffuse cutaneous involvement, comorbidities and PAH with or without ILD represent the main poor prognostic factors.
Study context
- Population
- Unselected SSc patients diagnosed after 1 January 2009 and followed in four Italian rheumatologic centres, assessed up to 31 December 202091.6% females, 20% dcSSc
- Participants
- 912 patients
- Outcome
- Overall survival and predictors of mortality
- Follow-up
- 5 and 10 years
Methodological detailsmulticentre, Kaplan-Meier, Cox regression, standardized mortality ratio vs Italian general population
- Multicentre
- Kaplan-Meier
- Cox regression
- Standardized mortality ratio vs Italian general population
Main finding
As reported in the abstract
Overall survival rate at 5 years
94.4%
Overall survival rate at 10 years
89.4%
Standardized mortality ratio (SMR) vs Italian general population
0.96
95% CI 0.81, 1.13
Observational data: associations, not effects.
Further findings
Main death predictors (Cox regression, 10-year follow-up)
- Male gender, HR
- 2.76
- Diffuse cutaneous involvement, HR
- 3.14
- Older age at diagnosis, HR
- 1.08
- Pulmonary arterial hypertension (PAH), HR
- 3.21
- Interstitial lung disease (ILD)-associated pulmonary hypertension (PH), HR
- 4.11
- Comorbidities, HR
- 3.53
- GC treatment, HR
- 2.02
Survival with PAH and ILD-associated PH
- PAH and ILD associated with PH significantly reduced survival
- P < 0.0001
Original publication
Survival and prognostic factors from a multicentre large cohort of unselected Italian systemic sclerosis patients. Rheumatology (Oxford). Published 8 September 2022. doi:10.
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