Abstract
Introduction
PAH specific therapies have been trialled in several lung diseases without success. It has also been shown that a low FVC is associated with a poor prognosis in CTD-PAH.
Methods
From the Royal Free Hospital pulmonary hypertension associated with connective tissue disease database we identified patients with lung function tests within 6 months of right heart catheterisation performed to confirm pulmonary hypertenson. Patients who had repeat haemodynamic studies within a year of diagnosis on first line therapy (bosentan) were included.
Results
There were no significant haemodynamic differences between groups at baseline (ANOVA). Follow up catheter studies demonstrated a good haemodynamic response with improvements in mean PA pressure (FVC >94%) and pulmonary vascular resistance (FVC >70%).
The changes seen in the FVC <70% group did not reach significance (e.g. p<0.09 for PVR).
Conclusion
A low FVC does not preclude a good treatment response, suggesting that PAH can co-exist with lung fibrosis in connective tissue diseases. Treatment of both pathologies may be needed to improve outcomes in this difficult group.
- © 2012 ERS
