Abstract
Background: The purpose of this study was to evaluate the prevalence and outcomes of pulmonary hypertension (PH) in chronic hypersensitivity pneumonitis (HP) and to examine the relationship between pulmonary function tests (PFT) and PH.
Methods: A retrospective review of 120 patients with HP seen at two centres of pulmonary diseases over a 5-year interval and identification of patients with chronic HP for whom both PFT and Doppler echocardiography (DE) data were available.
Results: Chronic HP was identified in 83 patients and in 72 patients data of Doppler echocardiography were available. PH (sPAP ≥ 40 mmHg) was detected in 26 patients (36%), and was associated with a greater risk of death (median survival, 30 months vs 98 months, respectively; p = 0.002). In patients with PH, a significantly decreased PaO2, predicted% of DLCO, predicted% of FVC and increased PaCO2 were detected. There was only a weak correlation between pulmonary function testing and the underlying PH.
Conclusions: Using DE for evaluation, PH is common in patients with chronic HP and significantly impacts survival. Because of the poor correlation between pulmonary function testing and PH correspondingly with other interstitial lung diseases (ILD), other factors than fibrosis may play a role in the etiology.
- © 2011 ERS
