دورية أكاديمية
Pathogenesis, clinical features, and phenotypes of pulmonary hypertension associated with interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative ‐ Group 3 Pulmonary Hypertension
| العنوان: | Pathogenesis, clinical features, and phenotypes of pulmonary hypertension associated with interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative ‐ Group 3 Pulmonary Hypertension |
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| المؤلفون: | Lucilla Piccari, Brian Allwood, Katerina Antoniou, Jonathan H. Chung, Paul M. Hassoun, Sylvia M. Nikkho, Rajan Saggar, Oksana A. Shlobin, Patrizio Vitulo, Steven D. Nathan, Stephen John Wort |
| المصدر: | Pulmonary Circulation, Vol 13, Iss 2, Pp n/a-n/a (2023) |
| بيانات النشر: | Wiley, 2023. |
| سنة النشر: | 2023 |
| المجموعة: | LCC:Diseases of the circulatory (Cardiovascular) system LCC:Diseases of the respiratory system |
| مصطلحات موضوعية: | endophenotype, histology, idiopathic pulmonary fibrosis, pathophysiology, pulmonary vascular disease, Diseases of the circulatory (Cardiovascular) system, RC666-701, Diseases of the respiratory system, RC705-779 |
| الوصف: | Abstract Pulmonary hypertension (PH) is a frequent complication of interstitial lung disease (ILD). Although PH has mostly been described in idiopathic pulmonary fibrosis, it can manifest in association with many other forms of ILD. Associated pathogenetic mechanisms are complex and incompletely understood but there is evidence of disruption of molecular and genetic pathways, with panvascular histopathologic changes, multiple pathophysiologic sequelae, and profound clinical ramifications. While there are some recognized clinical phenotypes such as combined pulmonary fibrosis and emphysema and some possible phenotypes such as connective tissue disease associated with ILD and PH, the identification of further phenotypes of PH in ILD has thus far proven elusive. This statement reviews the current evidence on the pathogenesis, recognized patterns, and useful diagnostic tools to detect phenotypes of PH in ILD. Distinct phenotypes warrant recognition if they are characterized through either a distinct presentation, clinical course, or treatment response. Furthermore, we propose a set of recommendations for future studies that might enable the recognition of new phenotypes. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 2045-8940 |
| العلاقة: | https://doaj.org/toc/2045-8940Test |
| DOI: | 10.1002/pul2.12213 |
| الوصول الحر: | https://doaj.org/article/73b2d7d3068442c48437e55307419645Test |
| رقم الانضمام: | edsdoj.73b2d7d3068442c48437e55307419645 |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 20458940 |
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| DOI: | 10.1002/pul2.12213 |