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.
Abstract
This is the first prospective study indicating that borderline elevation of mPAP is associated with higher incidence of PH in high risk SSc-patients using systematic recatheterisation
Footnotes
This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.
Conflict of interest: Dr. Coghlan reports grants from Actelion Ltd, during the conduct of the study; personal fees from Bayer, personal fees from Actelion, personal fees from GSK, outside the submitted work.
Conflict of interest: Matthias Wolf has nothing to disclose.
Conflict of interest: Dr. Distler reports grants and personal fees from Actelion, grants and personal fees from Bayer, personal fees from BiogenIdec, grants and personal fees from Boehringer Ingelheim, personal fees from ChemomAb, personal fees from espeRare foundation, personal fees from Genentech/Roche, personal fees from GSK, personal fees from Inventiva, personal fees from Lilly, personal fees from medac, personal fees from MedImmune, personal fees from Pharmacyclics, grants and personal fees from Pfizer, grants and personal fees from Sanofi, personal fees from Mitsubishi Tanabe Pharma, personal fees from Sinoxa, grants and personal fees from Novartis, personal fees from UCB, outside the submitted work; In addition, Dr. Distler has a patent mir-29 for the treatment of systemic sclerosis licensed.
Conflict of interest: Dr. Denton reports personal fees from Actelion, grants and personal fees from GlaxoSmithKline, personal fees from Bayer, personal fees from Sanofi-Aventis, grants and personal fees from Inventiva, personal fees from Boehringer Ingelheim, personal fees from Roche, personal fees from Bristol Myers Squibb, personal fees from Merck-Serono, outside the submitted work.
Conflict of interest: Martin Doelberg
Conflict of interest: Mrs. Harutyunova, MD, reports personal fees from Bayer MSD, personal fees from Actelion, personal fees from GSK, outside the submitted work.
Conflict of interest: Dr. Marra reports personal fees from Bayer, outside the submitted work.
Conflict of interest: Mrs. Benjamin reports personal fees from Bayer, personal fees from Actelion, outside the submitted work.
Conflict of interest: Dr. Fischer has nothing to disclose.
Conflict of interest: Ekkehard Grünig has received fees for lectures and/or consultations from Actelion, Bayer/MSD, GSK, United Therapeutics and Pfizer.
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