Different Cytokine Patterns in BMPR2-Mutation-Positive Patients and Patients With Pulmonary Arterial Hypertension Without Mutations and Their Influence on Survival.
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Authors
Schwiening, Max
Swietlik, Emilia M
Pandya, Divya
Burling, Keith
Barker, Peter
Feng, Oliver Y
Treacy, Carmen M
Abreu, Susana
Wort, S John
Pepke-Zaba, Joanna
Graf, Stefan
Marciniak, Stefan J
Morrell, Nicholas W
Publication Date
2022-06Journal Title
Chest
ISSN
0012-3692
Publisher
Elsevier BV
Type
Article
This Version
AM
Metadata
Show full item recordCitation
Schwiening, M., Swietlik, E. M., Pandya, D., Burling, K., Barker, P., Feng, O. Y., Treacy, C. M., et al. (2022). Different Cytokine Patterns in BMPR2-Mutation-Positive Patients and Patients With Pulmonary Arterial Hypertension Without Mutations and Their Influence on Survival.. Chest https://doi.org/10.1016/j.chest.2022.01.019
Abstract
Pulmonary arterial hypertension (PAH) covers a range of life-limiting illnesses characterized by increased mean pulmonary arterial pressures, which if untreated, lead to right heart failure and death. This is due to remodeling of the small-to-medium sized pulmonary vessels, which obstruct blood flow. Pulmonary arterial hypertension can be further categorized into idiopathic PAH (without any identifiable cause, 6th World Symposium class 1.11) and heritable PAH , (defined by mutations in specific genes, 6th World Symposium class 1.21), the most common affecting bone morphogenetic protein receptor type II (BMPRII)2 3. It is known that BMPR2-mutation positive patients have worse cardiac indices at presentation and a worse overall outcome compared to PAH without mutations4. Possessing a BMPR2 mutation also creates a pro-inflammatory state, through loss of endothelial barrier function5 and loss of antioxidant capability6. This then begs the question as to whether the mutation-positive groups have different underlying pathogenetic mechanisms and require different biomarkers and treatments, analogous to how EGFR-mutation positive non-small cell lung cancer patients respond to tyrosine kinase inhibition while the majority of NSCLC patients do not.
Sponsorship
Medical Research Council (MR/R008051/1)
Academy of Medical Sciences (SGL013\1024)
Medical Research Council (G1002610)
Medical Research Council (MR/R009120/1)
MRC (MR/V028669/1)
Medical Research Council (G0802261)
Medical Research Council (MR/K020919/1)
British Heart Foundation (None)
British Heart Foundation (None)
British Heart Foundation (RE/18/1/34212)
Identifiers
External DOI: https://doi.org/10.1016/j.chest.2022.01.019
This record's URL: https://www.repository.cam.ac.uk/handle/1810/332797
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