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Mortality Risk Factors in Systemic Sclerosis

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.

The author has no conflicts of interest to disclose related to this subject

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