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

Carreira, Patricia E.; Carmona, Loreto; Joven, Beatriz E.; Loza, Estibaliz; Andreu, José Luis; Riemekasten, Gabriela; Vettori, Serena; Allanore, Yannick; Balbir-Gurman, Alexandra and Airò, Paolo, et al. (2017) In Clinical and Experimental Rheumatology 35. p.114-121
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)... (More)

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.

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published
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keywords
Pulmonary arterial hypertension, Right heart catheterisation, Systemic sclerosis
in
Clinical and Experimental Rheumatology
volume
35
pages
114 - 121
publisher
Pacini
external identifiers
  • scopus:85031665405
ISSN
0392-856X
language
English
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yes
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f8f1aa63-89dc-4892-a43f-40c077da6235
date added to LUP
2017-11-16 12:30:41
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2018-01-07 12:26:03
@article{f8f1aa63-89dc-4892-a43f-40c077da6235,
  abstract     = {<p>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 &lt; 3 years from the first non-Raynaud's phenomenon (RP) symptom at baseline EUSTAR visit, were selected. Elevated sPAP was defined as sPAP &gt; 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.</p>},
  author       = {Carreira, Patricia E. and Carmona, Loreto and Joven, Beatriz E. and Loza, Estibaliz and Andreu, José Luis and Riemekasten, Gabriela and Vettori, Serena and Allanore, Yannick and Balbir-Gurman, Alexandra and Airò, Paolo and Walker, Ulrich A. and Damjanov, Nemanja and Ananieva, Lidia P. and Rednic, Simona and Czirják, László and Distler, Oliver and Farge, Dominique and Hesselstrand, Roger and Corrado, Ada and Caramaschi, Paola and Tikly, Mohammed and Matucci-Cerinic, Marco},
  issn         = {0392-856X},
  keyword      = {Pulmonary arterial hypertension,Right heart catheterisation,Systemic sclerosis},
  language     = {eng},
  pages        = {114--121},
  publisher    = {Pacini},
  series       = {Clinical and Experimental Rheumatology},
  title        = {Clinical determinants of elevated systolic pulmonary artery pressure measured by transthoracic Doppler echocardiography in early systemic sclerosis},
  volume       = {35},
  year         = {2017},
}