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Significance of autoimmune disease in severe pulmonary hypertension complicating extensive pulmonary fibrosis: a prospective cohort study

The association of autoimmune disease (AI) with transplant-free survival in the setting of severe Group 3 pulmonary hypertension and extensive pulmonary fibrosis remains unclear. We report cases of severe pulmonary hypertension (mean pulmonary artery pressure ≥35 mmHg and right ventricular dysfuncti...

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Autores principales: Saggar, Rajeev, Giri, Paresh C., Deng, Chunqin, Johnson, Dana, McCloy, Mary K., Liang, Lloyd, Shaikh, Faisal, Hong, Jason, Channick, Richard N., Shapiro, Shelley S., Lynch, Joseph P., Belperio, John A., Weigt, Samuel S., Ramsey, Allison L., Ross, David J., Sayah, David M., Shino, Michael Y., Derhovanessian, Ariss, Sherman, Alexander E., Saggar, Rajan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8108092/
https://www.ncbi.nlm.nih.gov/pubmed/33996029
http://dx.doi.org/10.1177/20458940211011329
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author Saggar, Rajeev
Giri, Paresh C.
Deng, Chunqin
Johnson, Dana
McCloy, Mary K.
Liang, Lloyd
Shaikh, Faisal
Hong, Jason
Channick, Richard N.
Shapiro, Shelley S.
Lynch, Joseph P.
Belperio, John A.
Weigt, Samuel S.
Ramsey, Allison L.
Ross, David J.
Sayah, David M.
Shino, Michael Y.
Derhovanessian, Ariss
Sherman, Alexander E.
Saggar, Rajan
author_facet Saggar, Rajeev
Giri, Paresh C.
Deng, Chunqin
Johnson, Dana
McCloy, Mary K.
Liang, Lloyd
Shaikh, Faisal
Hong, Jason
Channick, Richard N.
Shapiro, Shelley S.
Lynch, Joseph P.
Belperio, John A.
Weigt, Samuel S.
Ramsey, Allison L.
Ross, David J.
Sayah, David M.
Shino, Michael Y.
Derhovanessian, Ariss
Sherman, Alexander E.
Saggar, Rajan
author_sort Saggar, Rajeev
collection PubMed
description The association of autoimmune disease (AI) with transplant-free survival in the setting of severe Group 3 pulmonary hypertension and extensive pulmonary fibrosis remains unclear. We report cases of severe pulmonary hypertension (mean pulmonary artery pressure ≥35 mmHg and right ventricular dysfunction) and extensive pulmonary fibrosis after pulmonary arterial hypertension-specific therapy. We used multivariate regression to determine the clinical variables associated with transplant-free survival. Of 286 screened patients, 55 demonstrated severe pulmonary hypertension and extensive pulmonary fibrosis and were treated with parenteral prostacyclin therapy. The (+)AI subgroup (n = 34), when compared to the (–)AI subgroup (n = 21), was more likely to be female (77% versus 19%) and younger (58.7 ± 12.1 versus 66.0 ± 10.7 years), and revealed lower forced vital capacity (absolute) (1.9 ± 0.7 versus 2.9 ± 1.1 L), higher D(L)CO (% predicted) (31.1 ± 15.2 versus 23.2 ± 8.0), and increased unadjusted transplant-free survival (1 year (84.6 ± 6.3% versus 45 ± 11.1%)), 3 years (71 ± 8.2% versus 28.6 ± 11.9%), and 5 years (47.6 ± 9.6% versus 6.4 ± 8.2%); (p = 0.01)). Transplant-free survival was unchanged after adjusting for age and gender. The pulmonary hemodynamic profiles improved after parenteral prostacyclin therapy, independent of AI status. The baseline variables associated with mortality included age at pulmonary hypertension diagnosis (heart rate (HR) 1.23 (confidence interval (CI) 1.03–1.47); p = 0.02) and presence of AI (HR 0.26 (confidence interval (CI) 0.10–0.70); p < 0.01). Gas exchange was not adversely affected by parenteral prostacyclin therapy. In the setting of severe Group 3 pulmonary hypertension and extensive pulmonary fibrosis treated with pulmonary arterial hypertension-specific therapy, AI is independently associated with increased transplant-free survival. Pulmonary hypertension/pulmonary fibrosis associated with AI should be considered in future clinical trials of pulmonary arterial hypertension-specific therapy in Group 3 pulmonary hypertension.
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spelling pubmed-81080922021-05-14 Significance of autoimmune disease in severe pulmonary hypertension complicating extensive pulmonary fibrosis: a prospective cohort study Saggar, Rajeev Giri, Paresh C. Deng, Chunqin Johnson, Dana McCloy, Mary K. Liang, Lloyd Shaikh, Faisal Hong, Jason Channick, Richard N. Shapiro, Shelley S. Lynch, Joseph P. Belperio, John A. Weigt, Samuel S. Ramsey, Allison L. Ross, David J. Sayah, David M. Shino, Michael Y. Derhovanessian, Ariss Sherman, Alexander E. Saggar, Rajan Pulm Circ Original Research Article The association of autoimmune disease (AI) with transplant-free survival in the setting of severe Group 3 pulmonary hypertension and extensive pulmonary fibrosis remains unclear. We report cases of severe pulmonary hypertension (mean pulmonary artery pressure ≥35 mmHg and right ventricular dysfunction) and extensive pulmonary fibrosis after pulmonary arterial hypertension-specific therapy. We used multivariate regression to determine the clinical variables associated with transplant-free survival. Of 286 screened patients, 55 demonstrated severe pulmonary hypertension and extensive pulmonary fibrosis and were treated with parenteral prostacyclin therapy. The (+)AI subgroup (n = 34), when compared to the (–)AI subgroup (n = 21), was more likely to be female (77% versus 19%) and younger (58.7 ± 12.1 versus 66.0 ± 10.7 years), and revealed lower forced vital capacity (absolute) (1.9 ± 0.7 versus 2.9 ± 1.1 L), higher D(L)CO (% predicted) (31.1 ± 15.2 versus 23.2 ± 8.0), and increased unadjusted transplant-free survival (1 year (84.6 ± 6.3% versus 45 ± 11.1%)), 3 years (71 ± 8.2% versus 28.6 ± 11.9%), and 5 years (47.6 ± 9.6% versus 6.4 ± 8.2%); (p = 0.01)). Transplant-free survival was unchanged after adjusting for age and gender. The pulmonary hemodynamic profiles improved after parenteral prostacyclin therapy, independent of AI status. The baseline variables associated with mortality included age at pulmonary hypertension diagnosis (heart rate (HR) 1.23 (confidence interval (CI) 1.03–1.47); p = 0.02) and presence of AI (HR 0.26 (confidence interval (CI) 0.10–0.70); p < 0.01). Gas exchange was not adversely affected by parenteral prostacyclin therapy. In the setting of severe Group 3 pulmonary hypertension and extensive pulmonary fibrosis treated with pulmonary arterial hypertension-specific therapy, AI is independently associated with increased transplant-free survival. Pulmonary hypertension/pulmonary fibrosis associated with AI should be considered in future clinical trials of pulmonary arterial hypertension-specific therapy in Group 3 pulmonary hypertension. SAGE Publications 2021-05-02 /pmc/articles/PMC8108092/ /pubmed/33996029 http://dx.doi.org/10.1177/20458940211011329 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by-nc/4.0/Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Original Research Article
Saggar, Rajeev
Giri, Paresh C.
Deng, Chunqin
Johnson, Dana
McCloy, Mary K.
Liang, Lloyd
Shaikh, Faisal
Hong, Jason
Channick, Richard N.
Shapiro, Shelley S.
Lynch, Joseph P.
Belperio, John A.
Weigt, Samuel S.
Ramsey, Allison L.
Ross, David J.
Sayah, David M.
Shino, Michael Y.
Derhovanessian, Ariss
Sherman, Alexander E.
Saggar, Rajan
Significance of autoimmune disease in severe pulmonary hypertension complicating extensive pulmonary fibrosis: a prospective cohort study
title Significance of autoimmune disease in severe pulmonary hypertension complicating extensive pulmonary fibrosis: a prospective cohort study
title_full Significance of autoimmune disease in severe pulmonary hypertension complicating extensive pulmonary fibrosis: a prospective cohort study
title_fullStr Significance of autoimmune disease in severe pulmonary hypertension complicating extensive pulmonary fibrosis: a prospective cohort study
title_full_unstemmed Significance of autoimmune disease in severe pulmonary hypertension complicating extensive pulmonary fibrosis: a prospective cohort study
title_short Significance of autoimmune disease in severe pulmonary hypertension complicating extensive pulmonary fibrosis: a prospective cohort study
title_sort significance of autoimmune disease in severe pulmonary hypertension complicating extensive pulmonary fibrosis: a prospective cohort study
topic Original Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8108092/
https://www.ncbi.nlm.nih.gov/pubmed/33996029
http://dx.doi.org/10.1177/20458940211011329
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