Interstitial lung disease (ILD) may be associated with hypoxia and pulmonary hypertension (PH). The coexistence of these diseases may produce severe and progressive functional limitation which may be ameliorated by the use of vasoactive therapy. Traditionally vasoactive therapy is not used in such patients due to a perceived lack of benefit and fear of worsening hypoxia.
We reviewed records of patients with ILD who had received vasoactive therapy for PH on a compassionate basis. Data retrieved included demographics, PFTs, radiographic findings, echocardiographic findings, six-minute walk (6MW) results, BNP, and NYHA functional class assessment.
We identified 11 patients with ILD who had received therapy for PH [age 54±8 yrs (mean±SD); 3M/8F; 6Caucasian/5AA]. Baseline characteristics included BMI 26±7 (mean±SD), FVC 50±16% (mean±SD), FEV1 47±17% (mean±SD), DLCO 23±16 (mean±SD). All patients had a restrictive ventilatory defect, mild (N=2), moderate (N=2), severe (N=7) and resting hypoxia 83±9 (mean±SD) %. Patients received epoprostenol (N=7), remodulin (N=3), or bosentan (N=4) over a mean of 29 (range 5-84) months. Over the first three months of therapy NYHA functional class improved from 3.3± 0.6 (mean±SD) to 2.6±0.5 (mean±SD) (p=0.048) and by 1 functional class in 60% of patients. 6MW distance improved from 845± 288 (mean±SD) to 1063±449 (p=NS) (mean±SD) and BNP declined from 521±284 (mean±SD) to 311±430 (mean±SD) (p=NS). Oxygen requirements, RVSP, and Hb did not show any significant change. The patients with severe disease also showed an improvement in 6MW and BNP when analyzed separately.
Our group of patients with combined ILD and PH benefited from vasoactive therapy in terms of improvement in NYHA functional class, 6MW distance, and BNP. Larger, prospective studies are needed to identify the patients most likely to benefit from such intervention.
Vasoactive therapy may help improve functional capacity in selected patients with fibrotic lung disease and PH.
Omar Minai, None.