1. Different Cytokine Patterns in BMPR2-Mutation-Positive Patients and Patients With Pulmonary Arterial Hypertension Without Mutations and Their Influence on Survival
- Author
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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, Soon, Elaine, Feng, Oliver [0000-0003-0039-7039], Marciniak, Stefan [0000-0001-8472-7183], Soon, Elaine [0000-0002-5744-5014], and Apollo - University of Cambridge Repository
- Subjects
Pulmonary and Respiratory Medicine ,Pulmonary Arterial Hypertension ,Mutation ,Cytokines ,Humans ,Familial Primary Pulmonary Hypertension ,Bone Morphogenetic Protein Receptors, Type II ,Cardiology and Cardiovascular Medicine ,Critical Care and Intensive Care Medicine - 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.
- Published
- 2022