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Rheumatology (Oxford). 2015 Jul;54(7):1262-9. doi: 10.1093/rheumatology/keu450. Epub 2015 Jan 17.

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

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National Scleroderma Reference Centre, Department of Internal Medicine, Lille Nord University, Lille, Orgamétrie biostatistiques, Roubaix, France, UO Reumatologia e Immunologia Clinica, Spedali Civili Brescia, Dipartimento Medicina Clinica e Sperimentale F-Magrassi II Policlinico U.O., Reumatologia, Napoli, Italy, Rheumatology A dpt, Paris 5 University, Cochin Hospital, Paris, France, Rheumatology Unit, Azienda Ospedaliera Universitaria Integrata di Verona, Verona, Centro per la Sclerosi Sistemica - Dipartimento di Medicina Clinica, Università La Sapienza, Policlinico Umberto I, Roma, Italy, Servicio de Reumatología, Hospital 12 de Octubre, Madrid, Spain, Divisione di Reumatologia, Università di Roma La Sapienza, Dipartimento di Clinica e Terapia medica applicata, Policlinico Umberto I, Roma, Ospedale Mauriziano, Centro di Reumatologia, Torino, Italy, Centre for Rheumatology, Royal Free and University College London Medical School, Royal Free Campus, London, UK, Department of Rheumatology, Charité University Hospital, German Rheumatism Research Centre, a Leibniz institute, Berlin, Germany, Dipartimento di Medicina, Ospedale San Gerardo, Monza, Dipartimento e Cattedra di Reumatologia, Università degli Studi di Milano, Istituto Ortopedico Gaetano Pini, Milano, Italy, Department of Internal Medicine and Rheumatology Clinic, Ion Cantacuzino Clinical Hospital, Bucharest, Romania, University Hospital of Copenhagen, Department of Dermatology D-40, HS-Bispebjerg Hospital, Copenhagen, Denmark, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, Clinica Reumatologie, University of Medicine & Pharmacy, Iuliu Hatieganu Cluj, Cluj-Napoca, Romania, Ghent University Hospital, Department of Rheumatology, Gent, Belgium, Rheumatologische Universitätsklinik, Felix Platter Spital, Basel, Switzerland, University of Florence, Department of Medicine, Section of Rheumatology, Florence, Italy and Lehrstuhl für Innere Medizin mit Schwerpunkt Rheumatologie der



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.


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.


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 <30 mmHg 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 50 mmHg 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.


An estimated sPAP >36 mmHg at baseline echocardiography was significantly and independently associated with reduced survival, regardless of the presence of pulmonary hypertension based on right heart catheterization.


pulmonary hypertension; survival; systemic sclerosis; systolic pulmonary arterial pressure; tricuspid regurgitant jet velocity

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