Dyspnea and functional limitation in interstitial lung diseases (ILD) are not always adequately explained by the degree of compromise in pulmonary function alone. Pulmonary hypertension (PH) is felt to be a major contributor to morbidity and mortality in these patients. It is not clear whether treatment with newer vaso-active agents benefits patients with PH in the setting of moderate or severe ILD.
Methods
Medical records of patients followed at our institution between July 2001 and June 2005 were reviewed to identify patients with moderate or severe fibrotic ILD and PH. Data regarding demographics, hemodynamics, and clinical characteristics at baseline and during follow-up were collected.
Results
We identified 19 patients who met our inclusion criteria and in whom vaso-active therapy [epoprostenol (N=10), bosentan (N=9)] was initiated. Most patients [(15/19(79%)] showed an initial positive response to therapy and improved their 6-min walk distance (6MWD) by >50 m (responders) and 12/15 (80%) improved by at least 1 WHO functional class. At 1-year follow-up, 7 of 15 (47%) ‘responders’ had deteriorated significantly. None of the patients died during 1 year of follow-up.
Conclusions
Epoprostenol and bosentan produced short-term functional benefit in our patients with PH and moderate or severe restrictive ILD. The generalizability of these results awaits the results of larger, prospective, randomized trials in such patients.
