ABSTRACT
The aim of this study was to prospectively evaluate the prevalence of pulmonary hypertension (PH) in patients with idiopathic pulmonary fibrosis (IPF). One hundred thirty-nine patients (101 male, mean age = 68.6 ± 9 years), with confirmed IPF and who were admitted to eight Pulmonary Departments in Greece between November 2005 and December 2006 were included in the study. Pulmonary artery systolic pressure (PASP) was estimated by echocardiography, and PH was defined as PASP > 36 mmHg. We compared demographics, pulmonary function tests, NYHA functional status, 6-min walk distance (6MWD), B-type natriuretic peptide (BNP), PaO(2), and P(A-a)O(2) at rest data between patients with PH and without PH (PASP ≤ 36 mmHg). Increased estimated right ventricular systolic pressure was present in 55% of patients (mean PASP = 47.1 ± 11.2 mmHg vs. 30.3 ± 3.8 mmHg, respectively). Patients with PH had a lower but not statistically significant DL(CO) (47.1 ± 18.8 vs. 52.5 ± 20.1), lower PaO(2) at rest (64.6 ± 12.2 vs. 71.1 ± 11.3, P = 0.004), and lower mean 6MWD (282 ± 118 vs. 338 ± 91, P = 0.007). Significant differences were also observed in the NYHA functional status between the two groups (P = 0.02). Statistically significant correlations were observed between PASP and PaO(2) at rest (r = -0.331, P = 0.00), P(A-a)O(2) at rest (r = 0.494, P = 0.00)(,) 6MWD (r = -0.264, P = 0.01), SpO(2) at rest (r = -0.293, P = 0.00), SpO(2) at the end of exercise (r = -0.364, P = 0.00), and also BNP values (r = 0.319, P = 0.01). Moreover, PaO(2) (P = 0.02), P(A-a)O(2) (P = 0.005), and SpO(2) at the end of exercise (P = 0.023) were independent predictors of the presence of estimated PH. Using Doppler echocardiography as a screening tool for the estimation of PH, we found that PH is common in patients with IPF. Gas exchange parameters at rest and exercise desaturation might indicate underlying PH in IPF.
Subject(s)
Hypertension, Pulmonary/epidemiology , Idiopathic Pulmonary Fibrosis/epidemiology , Aged , Echocardiography , Echocardiography, Doppler , Exercise Test , Familial Primary Pulmonary Hypertension , Female , Greece/epidemiology , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/diagnostic imaging , Lung/diagnostic imaging , Lung/physiopathology , Male , Middle Aged , Natriuretic Peptide, Brain/blood , Oxygen/blood , Prevalence , Prospective Studies , Respiratory Function Tests , Walking/physiologyABSTRACT
OBJECTIVE: The aim of this study was to describe sleep quality and associated daytime consequences in idiopathic pulmonary fibrosis (IPF). SUBJECTS AND METHODS: Fifteen patients with IPF and 15 control subjects matched on age and anthropometric variables were included in the study. Sleep quality and its daytime consequences were assessed by clinical interview, the Pittsburgh Sleep Quality Index (PSQI), the Functional Outcomes in Sleep Questionnaire (FOSQ), the Fatigue Severity Scale (FSS), the Epworth Sleepiness Scale and attended all-night polysomnography. RESULTS: Polysomnography revealed a decrease in sleep efficiency and slow wave sleep, and an increase in stage 1 sleep and arousal index in IPF patients compared to controls. Daytime tachypnea persisted during sleep. Oxygen saturation below 90% was observed during 34.3 +/- 37.3% of the total sleep time (TST). Quality of sleep and daytime function were moderately to significantly impaired based on the PSQI and FOSQ. The total FOSQ score was negatively correlated with TST with oxygen saturation below 90% (p = 0.01, r = -0.62). FSS scores were correlated with TST at oxygen saturation below 90% and mean oxygen saturation during sleep (p = 0.002, r = 0.74, and p = 0.007, r = -0.66, respectively). CONCLUSIONS: Our data suggest significant sleep disruption and consequent impairment of physical and social functioning in patients with IPF. In the absence of effective treatments for IPF, the improvement of sleep quality should be a primary therapeutic goal.
