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Value of systolic pulmonary arterial pressure as a prognostic factor of death in the systemic sclerosis EUSTAR population

(2015) RHEUMATOLOGY. 54(7). p.1262-1269
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Abstract
Objective. The aim of this study was to assess the prognostic value of systolic pulmonary artery pressure (sPAP) estimated by echocardiography in the multinational European League Against Rheumatism Scleroderma Trial and Research (EUSTAR) cohort. Methods. Data for patients with echocardiography documented between 1 January 2005 and 31 December 2011 were extracted from the EUSTAR database. Stepwise forward multivariable statistical Cox pulmonary hypertension analysis was used to examine the independent effect on survival of selected variables. Results. Based on our selection criteria, 1476 patients were included in the analysis; 87% of patients were female, with a mean age of 56.3 years (S.D. 13.5) and 31% had diffuse SSc. The mean duration of follow-up was 2.0 years (S.D. 1.2, median 1.9). Taking index sPAP of <30mmHg as reference, the hazard ratio (HR) for death was 1.67 (95% CI 0.92, 2.96) if the index sPAP was between 30 and 36 mmHg, 2.37 (95% CI 1.14, 4.93) for sPAP between 36 and 40 mmHg, 3.72 (95% CI 1.61, 8.60) for sPAP between 40 and 50mmHg and 9.75 (95% CI 4.98, 19.09) if sPAP was >50 mmHg. In a multivariable Cox model, sPAP and the diffusing capacity for carbon monoxide (DLCO) were independently associated with the risk of death [HR 1.833 (95% CI 1.035, 3.247) and HR 0.973 (95% CI 0.955, 0.991), respectively]. sPAP was an independent risk factor for death with a HR of 3.02 (95% CI 1.91, 4.78) for sPAP >= 36 mmHg. Conclusion. An estimated sPAP >36mmHg at baseline echocardiography was significantly and independently associated with reduced survival, regardless of the presence of pulmonary hypertension based on right heart catheterization.
Keywords
tricuspid regurgitant jet velocity, systemic sclerosis, systolic pulmonary arterial pressure, pulmonary hypertension, survival, EULAR SCLERODERMA TRIALS, HYPERTENSION, SURVIVAL, DIAGNOSIS, MULTICENTER, DATABASE

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Chicago
Hachulla, Eric, Pierre Clerson, Paolo Airò, Giovanna Cuomo, Yannick Allanore, Paola Caramaschi, Edoardo Rosato, et al. 2015. “Value of Systolic Pulmonary Arterial Pressure as a Prognostic Factor of Death in the Systemic Sclerosis EUSTAR Population.” Rheumatology 54 (7): 1262–1269.
APA
Hachulla, E., Clerson, P., Airò, P., Cuomo, G., Allanore, Y., Caramaschi, P., Rosato, E., et al. (2015). Value of systolic pulmonary arterial pressure as a prognostic factor of death in the systemic sclerosis EUSTAR population. RHEUMATOLOGY, 54(7), 1262–1269.
Vancouver
1.
Hachulla E, Clerson P, Airò P, Cuomo G, Allanore Y, Caramaschi P, et al. Value of systolic pulmonary arterial pressure as a prognostic factor of death in the systemic sclerosis EUSTAR population. RHEUMATOLOGY. 2015;54(7):1262–9.
MLA
Hachulla, Eric et al. “Value of Systolic Pulmonary Arterial Pressure as a Prognostic Factor of Death in the Systemic Sclerosis EUSTAR Population.” RHEUMATOLOGY 54.7 (2015): 1262–1269. Print.
@article{7059924,
  abstract     = {Objective. The aim of this study was to assess the prognostic value of systolic pulmonary artery pressure (sPAP) estimated by echocardiography in the multinational European League Against Rheumatism Scleroderma Trial and Research (EUSTAR) cohort. 
Methods. Data for patients with echocardiography documented between 1 January 2005 and 31 December 2011 were extracted from the EUSTAR database. Stepwise forward multivariable statistical Cox pulmonary hypertension analysis was used to examine the independent effect on survival of selected variables. 
Results. Based on our selection criteria, 1476 patients were included in the analysis; 87% of patients were female, with a mean age of 56.3 years (S.D. 13.5) and 31% had diffuse SSc. The mean duration of follow-up was 2.0 years (S.D. 1.2, median 1.9). Taking index sPAP of <30mmHg as reference, the hazard ratio (HR) for death was 1.67 (95% CI 0.92, 2.96) if the index sPAP was between 30 and 36 mmHg, 2.37 (95% CI 1.14, 4.93) for sPAP between 36 and 40 mmHg, 3.72 (95% CI 1.61, 8.60) for sPAP between 40 and 50mmHg and 9.75 (95% CI 4.98, 19.09) if sPAP was >50 mmHg. In a multivariable Cox model, sPAP and the diffusing capacity for carbon monoxide (DLCO) were independently associated with the risk of death [HR 1.833 (95% CI 1.035, 3.247) and HR 0.973 (95% CI 0.955, 0.991), respectively]. sPAP was an independent risk factor for death with a HR of 3.02 (95% CI 1.91, 4.78) for sPAP >= 36 mmHg. 
Conclusion. An estimated sPAP >36mmHg at baseline echocardiography was significantly and independently associated with reduced survival, regardless of the presence of pulmonary hypertension based on right heart catheterization.},
  author       = {Hachulla, Eric and Clerson, Pierre and Airò, Paolo and Cuomo, Giovanna and Allanore, Yannick and Caramaschi, Paola and Rosato, Edoardo and Carreira, Patricia E and Riccieri, Valeria and Sarraco, Marta and Denton, Christopher P and Riemekasten, Gabriela and Pozzi, Maria Rosa and Zeni, Silvana and Mihai, Carmen Marina and Ullman, Susanne and Distler, Oliver and Rednic, Simona and Smith, Vanessa and Walker, Ulrich A and Matucci-Cerinic, Marco and Müller-Ladner, Ulf and Launay, David},
  issn         = {1462-0324},
  journal      = {RHEUMATOLOGY},
  keywords     = {tricuspid regurgitant jet velocity,systemic sclerosis,systolic pulmonary arterial pressure,pulmonary hypertension,survival,EULAR SCLERODERMA TRIALS,HYPERTENSION,SURVIVAL,DIAGNOSIS,MULTICENTER,DATABASE},
  language     = {eng},
  number       = {7},
  pages        = {1262--1269},
  title        = {Value of systolic pulmonary arterial pressure as a prognostic factor of death in the systemic sclerosis EUSTAR population},
  url          = {http://dx.doi.org/10.1093/rheumatology/keu450},
  volume       = {54},
  year         = {2015},
}

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