Pulmonary vascular disease & thromboembolism
Serum and Pulmonary Expression Profiles of the Activin Signaling System in Pulmonary Arterial Hypertension
Authors' conclusion
These findings offer new insights into the activin signaling system in PAH and show that activin A and FSTL3 are prognostic biomarkers for PAH.
Study context
- Population
- Patients with newly diagnosed idiopathic, heritable, or anorexigen-associated pulmonary arterial hypertension
- Participants
- 80 patients
- Exposure
- Serum activin pathway members as prognostic biomarkers
- Pulmonary arterial hypertension (PAH)n = 80
- Outcome
- Death or lung transplantation
- Follow-up
- Median 69 (IQR 50-81) months
Methodological detailsexternal validation cohort
- External validation cohort
Main finding
As reported in the abstract
Hazard ratio (95% CI)P value
Baseline activin Atransplant-free survival; adjusted for age and sex
1.001(1.000–1.001)
P = 0.037Baseline FSTL3transplant-free survival; adjusted for age and sex
1.263(1.049–1.520)
P = 0.014Follow-up activin Atransplant-free survival; adjusted for age and sex
1.003(1.001–1.005)
P = 0.001Follow-up FSTL3transplant-free survival; adjusted for age and sex
1.365(1.185–1.573)
P < 0.001Baseline activin A <393 pg/mLtransplant-free survival; adjusted for NYHA class, 6-minute walk distance, NT-proBNP
0.14(0.03–0.61)
P = 0.009Baseline FSTL3 <16.6 ng/mLtransplant-free survival; adjusted for NYHA class, 6-minute walk distance, NT-proBNP
0.17(0.06–0.45)
P < 0.001Follow-up activin A <393 pg/mLtransplant-free survival; adjusted for NYHA class, 6-minute walk distance, NT-proBNP
0.23(0.07–0.78)
P = 0.019Follow-up FSTL3 <16.6 ng/mLtransplant-free survival; adjusted for NYHA class, 6-minute walk distance, NT-proBNP
0.27(0.09–0.78)
P = 0.015- Death or lung transplantation
- 26 of 80 patients (32.5%)
- ROC-derived threshold, activin A
- 393 pg/mL
- ROC-derived threshold, FSTL3
- 16.6 ng/mL
Observational data: associations, not effects.
Further findings
Lung tissue histology (PAH vs control)
- Nuclear accumulation of phosphorylated Smad2/3
- Higher immunoreactivities for ACTRIIB, ALK2, ALK4, ALK5, ALK7, Cripto and FSTL3 in vascular endothelial and smooth muscle layers
- Lower immunostaining for inhibin-α and follistatin
Original publication
Serum and Pulmonary Expression Profiles of the Activin Signaling System in Pulmonary Arterial Hypertension. Circulation. Published 25 April 2023. doi:10.
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