Abstract
This study is based on 122 patients with interstitial lung disease (ILD) who underwent right heart catheterisation (RHC) to assess the pulmonary arterial pressure (43 patients with connective tissue disease (CTD-ILD), 52 IPF and 27 other ILDs). 19 were excluded from analysis because they had group 2 pulmonary hypertension (PH) or a pulmonary vascular resistance <3 W. The study population therefore comprised 103 patients (37 CTD-ILD, 43 IPF, 23 other). There were 49 men, 54 women. The mean age at RHC was 62 years (15 to 81). The mean follow up from RHC to death or the analysis date was 17.4 months (0 to 88). Pulmonary function tests shows a mean FVC %predicted of 67% (22 to 118), mean FEV1 % predicted 70 (23 to 119) and mean DLCO % predicted 38% (13 to 93). The mean composite physiological index (CPI) was 56 (12 to 91). Their GAP (Gender, Age, Physiology) stages were I: 41 patients, II: 46, III: 16. The mean SPAP was 59 mmHg (18 to 120) and the mean MPAP 33 mmHg (8 to 80). Patients with IPF were significantly older than those with CTD (mean 65 vs 59, p=0.018) and had a lower DLCO (IPF: 35%, CTD: 44%, p=0.01). 67 patients were found to have PH (CTD: 23, IPF: 28, other: 16). The correlated coefficient between SPAP and MPAP was 0.53, with SPAP > 35 mmHg in 56 of the 67 with PH by RHC. GAP and CPI did not correlates with PH. Those with CTD-ILD lived significantly longer than those with IPF (mean for CTD 28 months vs 14 months vs other 29 months, p 0.014). The same was found for the subgroup with PH. The development of PH has a significant impact on survival.
- Copyright ©the authors 2017