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

EUSTAR co-authors

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Objective. To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Dopplerechocardiography (TTE) in patients with early systemic sclerosis (SSc). Methods. 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. Results. 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 (dc- SSc). 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. Conclusion. 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 emphasise the need to consider right heart catheterisation in selected early SSc patients with PH suspicion, to clearly determine the cause of PH.

Original languageEnglish
Pages (from-to)S114-S121
JournalClinical and Experimental Rheumatology
Volume35
Publication statusPublished - Jan 1 2017

Fingerprint

Doppler Echocardiography
Systemic Scleroderma
Pulmonary Artery
Echocardiography
Pressure
Pulmonary Hypertension
Skin
Diffuse Scleroderma
Lung
Contracture
Cardiac Catheterization
Proteinuria
Ulcer
Cross-Sectional Studies
Joints
Databases
Kidney
Antibodies
Research

Keywords

  • Pulmonary arterial hypertension
  • Right heart catheterisation
  • Systemic sclerosis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology

Cite this

@article{8af2fc628ce5450687673fcb1700e29d,
title = "Clinical determinants of elevated systolic pulmonary artery pressure measured by transthoracic Doppler echocardiography in early systemic sclerosis",
abstract = "Objective. To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Dopplerechocardiography (TTE) in patients with early systemic sclerosis (SSc). Methods. 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. Results. 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 (dc- SSc). 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. Conclusion. 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 emphasise the need to consider right heart catheterisation in selected early SSc patients with PH suspicion, to clearly determine the cause of PH.",
keywords = "Pulmonary arterial hypertension, Right heart catheterisation, Systemic sclerosis",
author = "{EUSTAR co-authors} and Carreira, {Patricia E.} and Loreto Carmona and Joven, {Beatriz E.} and Estibaliz Loza and Andreu, {Jos{\'e} Luis} and Gabriela Riemekasten and Serena Vettori and Yannick Allanore and Alexandra Balbir-Gurman and Paolo Air{\`o} and Walker, {Ulrich A.} and Nemanja Damjanov and Ananieva, {Lidia P.} and Simona Rednic and L. Czirj{\'a}k and Oliver Distler and Dominique Farge and Roger Hesselstrand and Ada Corrado and Paola Caramaschi and Mohammed Tikly and Marco Matucci-Cerinic",
year = "2017",
month = "1",
day = "1",
language = "English",
volume = "35",
pages = "S114--S121",
journal = "Clinical and Experimental Rheumatology",
issn = "0392-856X",
publisher = "Clinical and Experimental Rheumatology S.A.S.",

}

TY - JOUR

T1 - Clinical determinants of elevated systolic pulmonary artery pressure measured by transthoracic Doppler echocardiography in early systemic sclerosis

AU - EUSTAR co-authors

AU - Carreira, Patricia E.

AU - Carmona, Loreto

AU - Joven, Beatriz E.

AU - Loza, Estibaliz

AU - Andreu, José Luis

AU - Riemekasten, Gabriela

AU - Vettori, Serena

AU - Allanore, Yannick

AU - Balbir-Gurman, Alexandra

AU - Airò, Paolo

AU - Walker, Ulrich A.

AU - Damjanov, Nemanja

AU - Ananieva, Lidia P.

AU - Rednic, Simona

AU - Czirják, L.

AU - Distler, Oliver

AU - Farge, Dominique

AU - Hesselstrand, Roger

AU - Corrado, Ada

AU - Caramaschi, Paola

AU - Tikly, Mohammed

AU - Matucci-Cerinic, Marco

PY - 2017/1/1

Y1 - 2017/1/1

N2 - Objective. To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Dopplerechocardiography (TTE) in patients with early systemic sclerosis (SSc). Methods. 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. Results. 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 (dc- SSc). 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. Conclusion. 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 emphasise the need to consider right heart catheterisation in selected early SSc patients with PH suspicion, to clearly determine the cause of PH.

AB - Objective. To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Dopplerechocardiography (TTE) in patients with early systemic sclerosis (SSc). Methods. 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. Results. 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 (dc- SSc). 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. Conclusion. 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 emphasise the need to consider right heart catheterisation in selected early SSc patients with PH suspicion, to clearly determine the cause of PH.

KW - Pulmonary arterial hypertension

KW - Right heart catheterisation

KW - Systemic sclerosis

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M3 - Article

VL - 35

SP - S114-S121

JO - Clinical and Experimental Rheumatology

JF - Clinical and Experimental Rheumatology

SN - 0392-856X

ER -