Systemic sclerosis & Raynaud's

Metabolomic Differences in Connective Tissue Disease–Associated Versus Idiopathic Pulmonary Arterial Hypertension in the PVDOMICS Cohort

Arthritis RheumatolPublished Prospective cohort study

Authors' conclusion

CTD-PAH is characterized by aberrant lipid metabolism that may signal shifted metabolic substrate utilization.

Study context

Population
Adults with CTD-PAH or idiopathic PAH (IPAH) from the PVDOMICS study115 with paired samples
Participants
141 vs 165
Exposure
CTD-PAH vs IPAH
  • CTD-PAHn = 141
  • IPAHn = 165reference
Outcome
Differential metabolomic profiles of plasma, CTD-PAH vs IPAH
Methodological detailsmetabolomic profiling, machine learning, paired mixed venous and wedged samples in a subset
  • Metabolomic profiling
  • Machine learning
  • Paired mixed venous and wedged samples in a subset

Main finding

As reported in the abstract

Plasma metabolomic profiles
  • Metabolomic profiles distinguished CTD-PAH from IPAH
  • CTD-PAH: aberrant lipid metabolism with lower sex steroid hormones and higher free fatty acids (FAs) and FA intermediates

Observational data: associations, not effects.

Further findings

Metabolite gradients across the right ventricular-pulmonary vascular (RV-PV) circulation

Acylcholines taken up by the RV-PV circulation, particularly in CTD-PAH; free FAs and acylcarnitines released

Metabolite-phenotype associations

In both subtypes, dysregulated lipid metabolites associated with hemodynamic and RV measurements and transplant-free survival

Original publication

Metabolomic Differences in Connective Tissue Disease–Associated Versus Idiopathic Pulmonary Arterial Hypertension in the PVDOMICS Cohort. Arthritis Rheumatol. Published 30 October 2023. doi:10.1002/art.42632

Composed by AI from the published abstract and checked against it. How Briefs are made →

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