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A French cohort study of 625 systemic sclerosis (SStc) patients has found a significantly increased mortality risk that can be predicted by common clinical variables.
In addition to a multicenter prospective study, investigators performed a metanalysis on SSc standarized mortality ratios (SMR) and hazard ratios of prognosis factors.
A total of 625 patients with SSC included those with limited cutaneous SSc (lcSSc; n=446) and diffuse cutaneous SSc (dcSSc; n=179). During follow-up, there were 104 deaths (16.6%) and 133 patients were lost to follow-up. Mortality was increased with SMR was 5.73 (95% CI 4.68–6.94). Overall survival rates :
- 1 year = 98.0%
- 3 year = 92.5%
- 5 year = 85.9%
- 10 year = 71.7%
Risk factors for reduced survival included: Age at diagnosis > 60 years, diffuse cutaneous SSc, scleroderma renal crisis, dyspnea, 6-min walking distance, forced vital capacity < 70%, diffusing capacity of the lungs for carbon monoxide < 70%, pulmonary hypertension (PH), telangiectasia, valvular disease, malignancy, anemia, and CRP > 8 mg/l.
A review of the literature identified 18 studies (11,719 patients) in the SMR meta-analysis and 36 studies (26,187 patients) in the prognosis factor analysis.
The pooled literature SMR was 3.45 (95%CI 3.03–3.94). Risk factors identified from the metanalysis included age at disease onset, male sex, African origin, diffuse cutaneous SSc, anti-Scl70 antibodies, cardiac and renal involvement, interstitial lung disease, PH, and malignancy.
Anti-centromere antibodies were associated with a better survival.
Higher mortality in SSc patients are seen with advancing skin disease and organ involvement, with further contributions by autoantibodies, 6MWD, and valvular disease.