Cargando…

Pulmonary hypertension in connective tissue diseases, new evidence and challenges

Pulmonary arterial hypertension is a lethal complication of different connective tissue diseases such as systemic sclerosis, mixed connective tissue disease and systemic lupus erythematosus. Although the treatment possibilities for patients with pulmonary arterial hypertension have increased in the...

Descripción completa

Detalles Bibliográficos
Autores principales: Vonk, Madelon C., Vandecasteele, Els, van Dijk, Arie P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7988614/
https://www.ncbi.nlm.nih.gov/pubmed/33216992
http://dx.doi.org/10.1111/eci.13453
_version_ 1783668818469453824
author Vonk, Madelon C.
Vandecasteele, Els
van Dijk, Arie P.
author_facet Vonk, Madelon C.
Vandecasteele, Els
van Dijk, Arie P.
author_sort Vonk, Madelon C.
collection PubMed
description Pulmonary arterial hypertension is a lethal complication of different connective tissue diseases such as systemic sclerosis, mixed connective tissue disease and systemic lupus erythematosus. Although the treatment possibilities for patients with pulmonary arterial hypertension have increased in the last two decades and survival of patients with idiopathic pulmonary arterial hypertension has improved, the latter is not the case for patients with pulmonary arterial hypertension associated with connective tissue disease. In this narrative review, we review recent literature and describe the improvement of early diagnostic possibilities, screening modalities and treatment options. We also point out the pitfalls in diagnosis in this patient category and describe the unmet needs and what the focus of future research should be.
format Online
Article
Text
id pubmed-7988614
institution National Center for Biotechnology Information
language English
publishDate 2020
publisher John Wiley and Sons Inc.
record_format MEDLINE/PubMed
spelling pubmed-79886142021-03-29 Pulmonary hypertension in connective tissue diseases, new evidence and challenges Vonk, Madelon C. Vandecasteele, Els van Dijk, Arie P. Eur J Clin Invest Reviews Pulmonary arterial hypertension is a lethal complication of different connective tissue diseases such as systemic sclerosis, mixed connective tissue disease and systemic lupus erythematosus. Although the treatment possibilities for patients with pulmonary arterial hypertension have increased in the last two decades and survival of patients with idiopathic pulmonary arterial hypertension has improved, the latter is not the case for patients with pulmonary arterial hypertension associated with connective tissue disease. In this narrative review, we review recent literature and describe the improvement of early diagnostic possibilities, screening modalities and treatment options. We also point out the pitfalls in diagnosis in this patient category and describe the unmet needs and what the focus of future research should be. John Wiley and Sons Inc. 2020-12-05 2021-04 /pmc/articles/PMC7988614/ /pubmed/33216992 http://dx.doi.org/10.1111/eci.13453 Text en © 22020 The Authors. European Journal of Clinical Investigation published by John Wiley & Sons Ltd on behalf of Stichting European Society for Clinical Investigation Journal Foundation This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Reviews
Vonk, Madelon C.
Vandecasteele, Els
van Dijk, Arie P.
Pulmonary hypertension in connective tissue diseases, new evidence and challenges
title Pulmonary hypertension in connective tissue diseases, new evidence and challenges
title_full Pulmonary hypertension in connective tissue diseases, new evidence and challenges
title_fullStr Pulmonary hypertension in connective tissue diseases, new evidence and challenges
title_full_unstemmed Pulmonary hypertension in connective tissue diseases, new evidence and challenges
title_short Pulmonary hypertension in connective tissue diseases, new evidence and challenges
title_sort pulmonary hypertension in connective tissue diseases, new evidence and challenges
topic Reviews
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7988614/
https://www.ncbi.nlm.nih.gov/pubmed/33216992
http://dx.doi.org/10.1111/eci.13453
work_keys_str_mv AT vonkmadelonc pulmonaryhypertensioninconnectivetissuediseasesnewevidenceandchallenges
AT vandecasteeleels pulmonaryhypertensioninconnectivetissuediseasesnewevidenceandchallenges
AT vandijkariep pulmonaryhypertensioninconnectivetissuediseasesnewevidenceandchallenges