PURPOSE: The aim of this study was to characterize patients with IPF and PAH in comparison to patients with IPF alone.
METHODS: Thirty six IPF patients were enrolled in this prospective cross-section observation study. The IPF was diagnosed on the basis of clinical data, plain chest radiography, data from the high resolution CT scans (HRCT) of the chest and presence of restrictive pulmonary dysfunction. All patients underwent arterial blood gas analysis on room air after 30 minutes of rest. All patients performed 6 Minute Walk Test indoors and Trans Thoracic Echocardiography (TTE).
RESULTS: During the study period, 36 patients with IPF were evaluated for the presence of pulmonary arterial hypertension. Twelve patients (33.3%) had pulmonary arterial hypertension (systolic PAP> 40mmgh). Their (n=12) mean age was 49.83+10.05 years; there were 5 males (41.6%) and 7 females (58.3%). Patients with IPF and PAH were more hypoxemic (PaO2=68.25+ 11.4, SaO2% = 89.92+ 3.53) than those without PAH (PaO2=73.75 + 9.02, SaO2% = 92.08 + 3.50) but the difference was statistically non-significant (P value=0.17, 0.6 respectively). There was no statistical significant difference as regards pH, paCO2, mean FVC % pred., mean FEV1% pred., mean FEV1/FVC% and mean FEF25-75 % pred. As regards 6MWT the total distance achieved by patients with IPF-PAH was significantly lower than patients with IPF alone (286.56 + 47.16,and 340.20 + 49.63 respectively ) and also post 6MWT SaO2% was significantly lower in patients with IPF-PAH than in patients with only IPF (75.5+8.54, 82.67+2.39 respectively) and( Pvalue=0.000). In the present study, there was a significant positive correlation between dyspnea scale and Pulmonary arterial systolic pressure in patients with IPF-PAH (R=0.67and p=0.017). Also there was a significant negative correlation between distance walked during 6MWT and Pulmonary arterial systolic pressure in patients with IPF-PAH(R=-0.737and p=0.006) and post 6MWT SaO2 % and Pulmonary arterial systolic pressure in patients with IPF-PAH (R=-0.723 and p=0.008) .
CONCLUSIONS: The prevalence of PAH in our IPF patients group was 33.3% as detected by transthoracic Echocardiography.
CLINICAL IMPLICATIONS: Pulmonary arterial hypertension (PAH) in patients with idiopathic pulmonary fibrosis (IPF) has been the subject of growing attention since it was demonstrated that its presence carries high mortality.
DISCLOSURE: The following authors have nothing to disclose: Nabila Laz, Yasser Ahmed
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