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 diffusing capacity of the lung for carbon monoxide (DLCO) <60% predicted.
In this bicentric, prospective cohort study, patients with SSc were clinically assessed at baseline and after 3 years, including right heart catheterisation (RHC). Analysis of determining factors for the development of PH was performed using univariate and multivariate analyses.
96 patients with a mean pulmonary arterial pressure (mPAP) <25 mmHg at baseline were followed for 2.95±0.7 years (median 3 years). Of these, 71 had a second RHC; 18 of these 71 patients (25.3%) developed PH, and five (7%) developed SSc-associated pulmonary arterial hypertension. For patients with an mPAP of 21–24 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 the size of the inferior vena cava at baseline were independent predictors for the development of PH during follow-up.
In a selected cohort of SSc patients with a DLCO <60%, pulmonary pressures appeared to rise progressively during follow-up. In this population, it was possible to identify manifest PH in almost 25% of patients using prospective RHC during follow-up. Therefore, regular clinical assessment including RHC might be useful in patients with SSc.
Abstract
Borderline elevation of mPAP is associated with higher incidence of pulmonary hypertension in high-risk systemic sclerosis patients http://ow.ly/stXU30iyzrU
Footnotes
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Conflict of interest: G. Coghlan received an unrestricted grant to support investigator-led study from Actelion Ltd, during the conduct of the study; and has received lecture fees and travel support from Bayer, lecture and consultancy fees from Actelion, and lecture fees from GSK, outside the submitted work.
Conflict of interest: M. Distler has received grants and personal fees from Actelion, Bayer, Boehringer Ingelheim, Pfizer, Sanofi and Novartis, and personal fees from BiogenIdec, ChemomAb, espeRare foundation, Genentech/Roche, GSK, Inventiva, Lilly, medac, MedImmune, Pharmacyclics, Mitsubishi Tanabe Pharma, Sinoxa and UCB, outside the submitted work, to investigate potential treatments of scleroderma and its complications. In addition, M. Distler has a patent mir-29 for the treatment of systemic sclerosis licensed.
Conflict of interest: C.P. Denton has received personal fees from Actelion, Bayer, Sanofi-Aventis, Boehringer Ingelheim, Roche, Bristol Myers Squibb and Merck-Serono, and grants and personal fees from GlaxoSmithKline and Inventiva, outside the submitted work.
Conflict of interest: M. Doelberg is an employee of Actelion Pharmaceuticals Ltd.
Conflict of interest: S. Harutyunova has received personal fees from Bayer, MSD, Actelion and GSK, outside the submitted work.
Conflict of interest: A.M. Marra has received personal fees from Bayer, outside the submitted work.
Conflict of interest: N. Benjamin has received lecture fees and travel support from Bayer, and lecture fees from Actelion, outside the submitted work.
Conflict of interest: E. Grünig has received fees for lectures and/or consultations from Actelion, Bayer/MSD, GSK, United Therapeutics and Pfizer.
Support statement: The study was funded by a grant from Actelion.
- Received June 17, 2017.
- Accepted February 15, 2018.
- Copyright ©ERS 2018