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A3.2 Five year survival rate and predictors of death and disease worsening in a single-center cohort of patients with systemic sclerosis
  1. A Soare1,
  2. C Mihai1,
  3. R Dobrotă1,
  4. AM Gherghe1,
  5. C Vancea1,
  6. M Gorga1,
  7. R Ioniţescu1,
  8. R Jurcuţ2,
  9. Ş Magda 3,
  10. T Constantinescu4,
  11. R Sfrenţ-Cornăţeanu1,5,
  12. I Ancuţa1,
  13. M Sasu1,
  14. M Milicescu1,
  15. L Macovei1,
  16. C Ciofu1,
  17. A Martin1,
  18. A Shorab1,
  19. M Bojincă1,
  20. V Stoica1
  1. 1EUSTAR 100 Center, Dr. Ion Cantacuzino Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest
  2. 2Prof. Dr. C. C. Iliescu Institute of Cardiovascular Disease, Carol Davila University of Medicine and Pharmacy, Bucharest
  3. 3Cardiology Clinic of the University Emergency Hospital Bucharest
  4. 4Prof. Dr. Marius Nasta National Institute of Pulmonology, Carol Davila University of Medicine and Pharmacy, Bucharest
  5. 5Physiology and Immunology Department, Carol Davila University of Medicine and Pharmacy, Bucharest

Abstract

Background Systemic sclerosis (SSc) is associated with a significant reduction in survival in comparison to the general population. This is the first report on 5-year survival and its predictors in a Romanian cohort of SSc patients.

Objective We aimed to assess the 5-year survival rate in a single-center cohort of SSc patients and to identify predictors of death and disease worsening.

Methods All patients of the EUSTAR Center 100 enrolled before 2009 and who had at least 2 visits at a minimum interval of 5 years, or who died after at least 3 months of follow-up, were included. All patients were assessed according to current EUSTAR recommendations. A comparison was made between the surviving and the deceased patients regarding all MEDS baseline parameters. Using age-adjusted univariate logistic regression we identified predictors for death and for several outcomes considered as disease worsening: 20% reduction in forced vital capacity (FVC); 20% reduction in diffusing capacity of the lung for carbon monoxide (DLCO); development of pulmonary arterial hypertension (PAH) as assessed by power Doppler heart ultrasound; and digital ulcers (DUs) recurrent at prospective visits.

Results Out of 68 patients enrolled before 01.01.2009, 40 met the inclusion criteria (82,5% females, 55% limited cutaneous subset, mean ± SD follow-up period 5.7 ± 2.1 years; mean ± SD age at first visit 49 ± 11.8 years; mean ± SD disease duration at first visit 4.4 ± 6.0 years). Throughout the 5-year follow-up period there were 7 deaths (including 3 SSc-related deaths), resulting in an overall 5-year survival rate of 82,5%. In survivors, lung function tests deteriorated significantly in 11% (FVC) and 52% (DLCO), while 20% developed PAH. Recurrent or newly appearing DUs occurred in 21% of all 42 patients. Significant predictors for death were the diffuse cutaneous subset, and the presence of DUs at presentation, with odd ratios [95% confidence interval] (OR[CI95%]) of 14,2 [1.3-151] and 38 [2.9-501] respectively. Conduction blocks on the baseline ECG predicted PAH with an OR [95% CI] of 12.8 [1.5-108]. Significant predictors for DUs were active DUs and calcinosis at presentation: OR [CI95%] 5.2 [1.1-26] and 6 [1.1-33] respectively. None of the parameters tested as potential predictors of the respiratory function decline achieved statistical significance.

Conclusions The survival rate in our cohort was 82.5%, which is similar to other cohorts from developed countries. We identified diffuse cutaneous subset, active digital ulcers, and heart conduction blocks as predictors for a poor disease outcome.

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