Use of zonal distribution of lung crackles during inspiration and expiration to assess disease severity in idiopathic pulmonary fibrosis.

PURPOSE OF THE STUDY: To measure the number and distribution of crackles in patients with idiopathic pulmonary fibrosis (IPF) and assess how this relates to measures of disease severity. STUDY DESIGN: Fourteen patients with IPF had both the number of crackles per litre of lung volume and lung function measured every 3 months for 1 year. Crackle counts were expressed according to position (upper and lower zones) and whether they occurred during inspiration and expiration. RESULTS: At baseline, crackle count per unit volume was higher at the bases than the apices and higher during inspiration than during expiration. There was a significant relationship between lung function and number of crackles per unit volume. Upper zone crackles during inspiration (crackle count vs forced vital capacity (FVC): r=0.69, p=0.007) and lower zone crackles during expiration (crackle count vs FVC: r=0.55, p=0.04) demonstrated the strongest relationship with lung function. CONCLUSIONS: Number and distribution of crackles in IPF relate to physiological measures of disease severity. Inspiratory lower zone crackles were universal and extensive but the presence, hence, development of inspiratory upper zone crackles and expiratory lower zone crackles correlated with measures of poorer lung function. The presence or appearance of these assessed using chest auscultation provides a clinician with simple measure of disease severity, and possibly progression, prompting further physiological assessment and review of treatment.

Objective cough frequency in Idiopathic Pulmonary Fibrosis.

BACKGROUND: Cough is a common presenting symptom in patients with Idiopathic Pulmonary Fibrosis (IPF). This study measured cough rates in IPF patients and investigated the association between cough and measures of health related quality of life and subjective cough assessments. In addition, IPF cough rates were related to measures of physiological disease severity and compared to cough rates in health and other respiratory conditions. METHODS: Nineteen IPF patients, mean age 70.8 years +/- 8.6, five female (26.3%) were studied. Subjects performed full pulmonary function testing, 24 hour ambulatory cough recordings, completed a cough related quality of life questionnaire (Leicester Cough Questionnaire) and subjectively scored cough severity with a visual analogue scale. Ambulatory cough recordings were manually counted and reported as number of coughs per hour. RESULTS: The 24hr cough rates were high (median 9.4, range 1.5-39.4), with day time rates much higher than night time (median 14.6, range 1.9-56.6 compared to 1.9, range 0-19.2, p = 0.003). Strong correlations were found between objective cough frequency and both the VAS (day r = 0.80, p < 0.001, night r = 0.71, p = 0.001) and LCQ (r = -0.80, p < 0.001), but not with measures of pulmonary function. Cough rates in IPF were higher than healthy subjects (p < 0.001) and asthma patients (p < 0.001) but similar to patients with chronic cough (p = 0.33). CONCLUSIONS: This study confirms objectively that cough is a major, very distressing and disabling symptom in IPF patients. The strong correlations between objective cough counts and cough related quality of life measures suggest that in IPF patient's, perception of cough frequency is very accurate.