Coghlan, G;
Wolf, M;
Distler, O;
Denton, CP;
Doelberg, M;
Harutyunova, S;
Marra, AM;
... Grünig, E; + view all
(2018)
Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis.
European Respiratory Journal
, 51
(3)
10.1183/13993003.01197-2017.
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Abstract
Objective: The objective of this study was to evaluate the incidence of pulmonary hypertension (PH) and determining factors in patients with systemic sclerosis (SSc) and a DLCO < 60% predicted.Methods:In this bicentric, prospective cohort study, patients with SSc were assessed at baseline and after 3 years clinically including right heart catheterization (RHC). Analysis of determining factors for development of PH was performed using univariate and multivariate analysis.Results:Ninety-six patients with mean pulmonary artery pressure (mPAP) <25 mmHg at baseline were followed 2.95±0.7 (median 3) years. Seventy-one had a second RHC; 18 of the 71 patients (25.3%) developed PH, 5 (7%) a SSc-associated pulmonary arterial hypertension. For patients with mPAP between 21 and 24 mmHg at baseline, the likelihood of presenting with PH as opposed to normal pressures on follow-up was significantly higher (p=0.026). Pulmonary vascular resistance, tricuspid regurgitation velocity, diffusion capacity and size of inferior vena cava at baseline were independent predictors for development of PH during follow-up.Conclusion:In a selected cohort of SSc patients with a DLCO < 60%, pulmonary pressures appear to rise progressively during follow up. In this population using prospective RHC during follow-up it was possible to identify manifest PH in almost 25% of 44 patients. Therefore, regular clinical assessment including RHC might be useful in SSc-patients.Most important findings:In a selected cohort of SSc patients pulmonary pressures appear to rise progressively, leading to a development of manifest PH in 25% within 3 years.
| Type: | Article |
|---|---|
| Title: | Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis |
| Location: | England |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1183/13993003.01197-2017 |
| Publisher version: | http://doi.org/10.1183/13993003.01197-2017 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Systemic sclerosis, pulmonary hypertension, risk stratification |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10045793 |
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