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Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis


Coghlan, Gerry; Wolf, Matthias; Distler, Oliver; Denton, Christopher P; Doelberg, Martin; Harutyunova, Satenik; Marra, Alberto M; Benjamin, Nicola; Fischer, Christine; Grünig, Ekkehard (2018). Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis. European Respiratory Journal, 51(3):online.

Abstract

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.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.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.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.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.

Abstract

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.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.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.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.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.

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Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Rheumatology Clinic and Institute of Physical Medicine
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:21 March 2018
Deposited On:27 Mar 2018 18:28
Last Modified:13 Apr 2018 11:48
Publisher:European Respiratory Society
ISSN:0903-1936
OA Status:Closed
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1183/13993003.01197-2017
PubMed ID:29563168

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