Haque, A., Kiely, D.G. orcid.org/0000-0003-0184-6502, Kovacs, G. et al. (2 more authors) (2021) Pulmonary hypertension phenotypes in patients with systemic sclerosis. European Respiratory Review, 30 (161). 210053. ISSN 0905-9180
Abstract
Pulmonary hypertension (PH) commonly affects patients with systemic sclerosis (SSc) and is associated with significant morbidity and increased mortality. PH is a heterogenous condition and several different forms can be associated with SSc, including pulmonary arterial hypertension (PAH) resulting from a pulmonary arterial vasculopathy, PH due to left heart disease and PH due to interstitial lung disease. The incidence of pulmonary veno-occlusive disease is also increased. Accurate and early diagnosis to allow optimal treatment is, therefore, essential. Recent changes to diagnostic haemodynamic criteria at the 6th World Symposium on Pulmonary Hypertension have resulted in therapeutic uncertainty regarding patients with borderline pulmonary haemodynamics. Furthermore, the optimal pulmonary vascular resistance threshold for diagnosing PAH and the role of exercise in identifying early disease require further elucidation. In this article we review the epidemiology, diagnosis, outcomes and treatment of the spectrum of pulmonary vascular phenotypes associated with SSc.
Metadata
Item Type: | Article |
---|---|
Authors/Creators: |
|
Copyright, Publisher and Additional Information: | © The authors 2021. This version is distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. (http://creativecommons.org/licenses/by-nc/4.0/) For commercial reproduction rights and permissions contact permissions@ersnet.org |
Dates: |
|
Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Sheffield Teaching Hospitals |
Funding Information: | Funder Grant number BRITISH HEART FOUNDATION FS/18/13/33281 |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 11 Mar 2022 09:55 |
Last Modified: | 11 Mar 2022 09:55 |
Status: | Published |
Publisher: | European Respiratory Society (ERS) |
Refereed: | Yes |
Identification Number: | 10.1183/16000617.0053-2021 |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:184619 |