Reversal of inflammatory reprogramming by vasodilator agents in pulmonary hypertension

Background Pulmonary arterial hypertension (PAH) is a deadly disease without effective non-invasive diagnostic and prognostic testing. It remains unclear whether vasodilators reverse inflammatory activation, a part of PAH pathogenesis. Single-cell profiling of inflammatory cells in blood could clari...

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Main Authors: Anna Kirillova, Meena Sethuraman, Xishuang Dong, Almina Kirdar, Gil Speyer, Yassmin Al Aaraj, Annie Watson, Lily K. Schneider, Michael D. Creager, Robert Lafyatis, Satoshi Okawa, Seungchan Kim, Stephen Y. Chan
Format: Article
Language:English
Published: European Respiratory Society 2025-01-01
Series:ERJ Open Research
Online Access:http://openres.ersjournals.com/content/11/1/00486-2024.full
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author Anna Kirillova
Meena Sethuraman
Xishuang Dong
Almina Kirdar
Gil Speyer
Yassmin Al Aaraj
Annie Watson
Lily K. Schneider
Michael D. Creager
Robert Lafyatis
Satoshi Okawa
Seungchan Kim
Stephen Y. Chan
author_facet Anna Kirillova
Meena Sethuraman
Xishuang Dong
Almina Kirdar
Gil Speyer
Yassmin Al Aaraj
Annie Watson
Lily K. Schneider
Michael D. Creager
Robert Lafyatis
Satoshi Okawa
Seungchan Kim
Stephen Y. Chan
author_sort Anna Kirillova
collection DOAJ
description Background Pulmonary arterial hypertension (PAH) is a deadly disease without effective non-invasive diagnostic and prognostic testing. It remains unclear whether vasodilators reverse inflammatory activation, a part of PAH pathogenesis. Single-cell profiling of inflammatory cells in blood could clarify these PAH mechanisms. Methods We evaluated a University of Pittsburgh Medical Center cohort consisting of idiopathic PAH (iPAH) and systemic sclerosis-associated PAH (sscPAH) patients and non-PAH controls. We performed single-cell RNA sequencing of peripheral blood mononuclear cells (PBMCs) from controls (n=3) and from PAH patients (iPAH and sscPAH) naïve to treatment (n=4), PAH patients 3 months after phosphodiesterase-5 inhibitor (PDE5i) treatment (n=7) and PAH patients 3 months after PDE5i+macitentan treatment (n=6). We compared the transcriptomes of five PBMC subtypes from iPAH and sscPAH to observe their serial responses to treatments. Furthermore, we utilised network analysis to illuminate the altered connectivity of biological networks in this complex disease. Results We defined differential gene expression and perturbed network connectivity in PBMCs of PAH patients following treatment with PDE5i or PDE5i+macitentan. Importantly, we identified significant reversal of inflammatory transcripts and pathways in the combined PAH patient cohort after vasodilator therapy in every PBMC type assessed. The “glucagon signalling in metabolic regulation” pathway in monocytes was reversed after vasodilator therapy via two independent analysis modalities. Conclusion Via a systems-biology approach, we define inflammatory reprogramming in the blood of PAH patients and the anti-inflammatory activity of vasodilators. Such findings establish diagnostic and prognostic blood-based tools for tracking inflammatory progression of PAH and response to therapy.
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spelling doaj-art-39b41eeb1abf484cad4971b9f38c68022025-08-20T03:02:16ZengEuropean Respiratory SocietyERJ Open Research2312-05412025-01-0111110.1183/23120541.00486-202400486-2024Reversal of inflammatory reprogramming by vasodilator agents in pulmonary hypertensionAnna Kirillova0Meena Sethuraman1Xishuang Dong2Almina Kirdar3Gil Speyer4Yassmin Al Aaraj5Annie Watson6Lily K. Schneider7Michael D. Creager8Robert Lafyatis9Satoshi Okawa10Seungchan Kim11Stephen Y. Chan12 Center for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Division of Cardiology, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA Center for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Division of Cardiology, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA Department of Electrical and Computer Engineering, Center for Computational Systems Biology, Prairie View A&M University, Prairie View, TX, USA Center for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Division of Cardiology, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA Research Computing, Arizona State University, Tempe, AZ, USA Center for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Division of Cardiology, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA Center for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Division of Cardiology, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA Center for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Division of Cardiology, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA Center for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Division of Cardiology, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA Division of Rheumatology, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA Center for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Division of Cardiology, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA Department of Electrical and Computer Engineering, Center for Computational Systems Biology, Prairie View A&M University, Prairie View, TX, USA Center for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Division of Cardiology, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA Background Pulmonary arterial hypertension (PAH) is a deadly disease without effective non-invasive diagnostic and prognostic testing. It remains unclear whether vasodilators reverse inflammatory activation, a part of PAH pathogenesis. Single-cell profiling of inflammatory cells in blood could clarify these PAH mechanisms. Methods We evaluated a University of Pittsburgh Medical Center cohort consisting of idiopathic PAH (iPAH) and systemic sclerosis-associated PAH (sscPAH) patients and non-PAH controls. We performed single-cell RNA sequencing of peripheral blood mononuclear cells (PBMCs) from controls (n=3) and from PAH patients (iPAH and sscPAH) naïve to treatment (n=4), PAH patients 3 months after phosphodiesterase-5 inhibitor (PDE5i) treatment (n=7) and PAH patients 3 months after PDE5i+macitentan treatment (n=6). We compared the transcriptomes of five PBMC subtypes from iPAH and sscPAH to observe their serial responses to treatments. Furthermore, we utilised network analysis to illuminate the altered connectivity of biological networks in this complex disease. Results We defined differential gene expression and perturbed network connectivity in PBMCs of PAH patients following treatment with PDE5i or PDE5i+macitentan. Importantly, we identified significant reversal of inflammatory transcripts and pathways in the combined PAH patient cohort after vasodilator therapy in every PBMC type assessed. The “glucagon signalling in metabolic regulation” pathway in monocytes was reversed after vasodilator therapy via two independent analysis modalities. Conclusion Via a systems-biology approach, we define inflammatory reprogramming in the blood of PAH patients and the anti-inflammatory activity of vasodilators. Such findings establish diagnostic and prognostic blood-based tools for tracking inflammatory progression of PAH and response to therapy.http://openres.ersjournals.com/content/11/1/00486-2024.full
spellingShingle Anna Kirillova
Meena Sethuraman
Xishuang Dong
Almina Kirdar
Gil Speyer
Yassmin Al Aaraj
Annie Watson
Lily K. Schneider
Michael D. Creager
Robert Lafyatis
Satoshi Okawa
Seungchan Kim
Stephen Y. Chan
Reversal of inflammatory reprogramming by vasodilator agents in pulmonary hypertension
ERJ Open Research
title Reversal of inflammatory reprogramming by vasodilator agents in pulmonary hypertension
title_full Reversal of inflammatory reprogramming by vasodilator agents in pulmonary hypertension
title_fullStr Reversal of inflammatory reprogramming by vasodilator agents in pulmonary hypertension
title_full_unstemmed Reversal of inflammatory reprogramming by vasodilator agents in pulmonary hypertension
title_short Reversal of inflammatory reprogramming by vasodilator agents in pulmonary hypertension
title_sort reversal of inflammatory reprogramming by vasodilator agents in pulmonary hypertension
url http://openres.ersjournals.com/content/11/1/00486-2024.full
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