The role of pulmonary arterial hypertension-targeted therapy in systemic sclerosis [version 1; peer review: 2 approved]
Pulmonary arterial hypertension, categorized as group 1 pulmonary hypertension by the World Health Organization classification system, represents a major complication of systemic sclerosis resulting from pulmonary vascular involvement of the disease. The high mortality seen in systemic sclerosis-ass...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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F1000 Research Ltd
2019-12-01
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| Series: | F1000Research |
| Online Access: | https://f1000research.com/articles/8-2124/v1 |
| _version_ | 1818623182142177280 |
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| author | Michael H Lee Todd M Bull |
| author_facet | Michael H Lee Todd M Bull |
| author_sort | Michael H Lee |
| collection | DOAJ |
| description | Pulmonary arterial hypertension, categorized as group 1 pulmonary hypertension by the World Health Organization classification system, represents a major complication of systemic sclerosis resulting from pulmonary vascular involvement of the disease. The high mortality seen in systemic sclerosis-associated pulmonary arterial hypertension is likely due to the impairment of right ventricular systolic function and the coexistence of other non-group-1 pulmonary hypertension phenotypes that may negatively impact clinical response to pulmonary arterial hypertension-targeted therapy. This review highlights two areas of recent advances regarding the management of systemic sclerosis patients with pulmonary hypertension: the tolerability of pulmonary arterial hypertension-targeted therapy in the presence of mild to moderate interstitial lung disease and the potential clinical significance of the antifibrotic effect of soluble guanylate cyclase stimulators demonstrated in preclinical studies. |
| first_indexed | 2024-12-16T18:37:00Z |
| format | Article |
| id | doaj.art-01dffd27436947bda6e6c53158b5350d |
| institution | Directory Open Access Journal |
| issn | 2046-1402 |
| language | English |
| last_indexed | 2024-12-16T18:37:00Z |
| publishDate | 2019-12-01 |
| publisher | F1000 Research Ltd |
| record_format | Article |
| series | F1000Research |
| spelling | doaj.art-01dffd27436947bda6e6c53158b5350d2022-12-21T22:21:08ZengF1000 Research LtdF1000Research2046-14022019-12-01810.12688/f1000research.20313.122318The role of pulmonary arterial hypertension-targeted therapy in systemic sclerosis [version 1; peer review: 2 approved]Michael H Lee0Todd M Bull1Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado School of Medicine, Colorado, USADivision of Pulmonary Sciences and Critical Care Medicine, University of Colorado School of Medicine, Colorado, USAPulmonary arterial hypertension, categorized as group 1 pulmonary hypertension by the World Health Organization classification system, represents a major complication of systemic sclerosis resulting from pulmonary vascular involvement of the disease. The high mortality seen in systemic sclerosis-associated pulmonary arterial hypertension is likely due to the impairment of right ventricular systolic function and the coexistence of other non-group-1 pulmonary hypertension phenotypes that may negatively impact clinical response to pulmonary arterial hypertension-targeted therapy. This review highlights two areas of recent advances regarding the management of systemic sclerosis patients with pulmonary hypertension: the tolerability of pulmonary arterial hypertension-targeted therapy in the presence of mild to moderate interstitial lung disease and the potential clinical significance of the antifibrotic effect of soluble guanylate cyclase stimulators demonstrated in preclinical studies.https://f1000research.com/articles/8-2124/v1 |
| spellingShingle | Michael H Lee Todd M Bull The role of pulmonary arterial hypertension-targeted therapy in systemic sclerosis [version 1; peer review: 2 approved] F1000Research |
| title | The role of pulmonary arterial hypertension-targeted therapy in systemic sclerosis [version 1; peer review: 2 approved] |
| title_full | The role of pulmonary arterial hypertension-targeted therapy in systemic sclerosis [version 1; peer review: 2 approved] |
| title_fullStr | The role of pulmonary arterial hypertension-targeted therapy in systemic sclerosis [version 1; peer review: 2 approved] |
| title_full_unstemmed | The role of pulmonary arterial hypertension-targeted therapy in systemic sclerosis [version 1; peer review: 2 approved] |
| title_short | The role of pulmonary arterial hypertension-targeted therapy in systemic sclerosis [version 1; peer review: 2 approved] |
| title_sort | role of pulmonary arterial hypertension targeted therapy in systemic sclerosis version 1 peer review 2 approved |
| url | https://f1000research.com/articles/8-2124/v1 |
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