impact factor, citescore



Clinical determinants of elevated systolic pulmonary artery pressure measured by transthoracic Doppler echocardiography in early systemic sclerosis

1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22


  1. Servicio de Reumatología, Hospital Universitario12 de Octubre, Madrid, Spain.
  2. Instituto de Salud Musculoesquelética, Madrid, Spain.
  3. Servicio de Reumatología, Hospital Universitario 12 de Octubre, Madrid, Spain.
  4. Instituto de Salud Musculoesquelética, Madrid, Spain.
  5. Servicio de Reumatología, Hospital Universitario Puerta de Hierro, Madrid, Spain.
  6. Department of Rheumatology, University of Lübeck, Germany.
  7. UOC di Reumatologia, Dipartimento di Internistica Clinica e Sperimentale “F-Magrassi-A-Lanzara”, Seconda Università di Napoli, Italy.
  8. Rheumatology A Department, Cochin Hospital, APHP, Paris Descartes University, France.
  9. B.Shine Rheumatology Unit, Rambam Health Care Campus and Rappaport Faculty of Medicine-Technion, Haifa, Israel.
  10. UO Reumatologia ed Immunologia Clinica, Spedali Civili, Brescia, Italy.
  11. Department of Rheumatology, Unispital Basel, Switzerland.
  12. University of Belgrade School of Medicine, Institute of Rheumatology, Belgrade, Serbia.
  13. Institute of Rheumatology, Russian Academy of Medical Science, Moscow, Russia.
  14. Clinica Reumatologie, University of Medicine & Pharmacy “Iuliu Hatieganu”, Cluj-Napoca, Romania.
  15. Department of Immunology and Rheumatology, Faculty of Medicine, University of Pécs, Hungary.
  16. Division of Rheumatology, University Hospital Zurich, Switzerland.
  17. Department of Internal Medicine, Hopital Saint-Louis, Paris, France.
  18. Department of Rheumatology, Lund University, Sweden.
  19. UO Reumatologia-Università degli Studi di Foggia, Ospedale “Col. D’Avanzo”, Foggia, Italy.
  20. Rheumatology Unit, AOUI, Verona, Italy.
  21. Rheumatology Unit, Department of Medicine Chris Hani Baragwanath, Hospital and University of the Witwatersrand, Johannesburg, South Africa.
  22. Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Florence, Italy.

and EUSTAR co-authors

2017 Vol.35, N°4 ,Suppl.106
PI 0114, PF 0121

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PMID: 28664832 [PubMed]

Received: 24/06/2016
Accepted : 26/10/2016
In Press: 20/06/2017
Published: 12/10/2017


To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Doppler-echocardiography (TTE) in patients with early systemic sclerosis (SSc).
A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research (EUSTAR) database was performed. SSc patients with <3 years from the first non-Raynaud’s phenomenon (RP) symptom at baseline EUSTAR visit, were selected. Elevated sPAP was defined as sPAP>40 mmHg on baseline TTE. First visit SSc related variables, including disease subsets, antibodies and visceral involvement, were examined.
From 1,188 patients, 81% were women. Mean (SD) age at first non-RP symptom was 50 (14) years, 55% had limited cutaneous SSc (lcSSc) and 42% active disease. Elevated sPAP was found in 17% of patients, both lcSSc and diffuse cutaneous SSc (dcSSc). In lcSSc, older age at first non-RP symptom, ACA positivity, joint contractures, restrictive defect and lower DLCO, were independently associated with elevated sPAP. In dcSSc, older age at first non-RP symptom, longer time between RP onset and first non-RP symptom, digital ulcers, cardiac blocks, and proteinuria were associated with elevated sPAP.
The prevalence of elevated sPAP on TTE in early SSc patients is considerable. Association with cardiac, lung and renal involvement suggests that, although some patients might have pulmonary arterial hypertension, others may present pulmonary hypertension secondary to lung or heart involvement. Our findings emphasize the need to consider right heart catheterisation in selected early SSc patients with PH suspicion, to clearly determine the cause of PH.

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