Pulmonary hypertension phenotypes in patients with systemic sclerosis.
Journal
European respiratory review : an official journal of the European Respiratory Society
ISSN: 1600-0617
Titre abrégé: Eur Respir Rev
Pays: England
ID NLM: 9111391
Informations de publication
Date de publication:
30 Sep 2021
30 Sep 2021
Historique:
received:
28
02
2021
accepted:
04
05
2021
entrez:
19
8
2021
pubmed:
20
8
2021
medline:
25
11
2021
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 34407977
pii: 30/161/210053
doi: 10.1183/16000617.0053-2021
pmc: PMC9517999
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : British Heart Foundation
ID : FS/18/13/33281
Pays : United Kingdom
Informations de copyright
Copyright ©The authors 2021.
Déclaration de conflit d'intérêts
Conflict of interest: A. Haque has nothing to disclose. Conflict of interest: D.G. Kiely reports personal fees and non-financial support from Bayer, GSK, Janssen and MSD, outside the submitted work. Conflict of interest: G. Kovacs reports personal fees and non-financial support from Bayer, GSK, Janssen, MSD, Boehringer Ingelheim, Novartis, Chiesi and Vitalaire, outside the submitted work. Conflict of interest: A.A.R. Thompson reports personal fees and non-financial support from Janssen, outside the submitted work. Conflict of interest: R. Condliffe reports personal fees and non-financial support from Bayer, GSK, Janssen and MSD, outside the submitted work.
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