Quantitative assessment of pulmonary function in lymphangioleiomyomatosis patients using high-resolution computed tomography and pulmonary function tests.

BACKGROUND

To explore the feasibility of using quantitative high-resolution computed tomography (HRCT) to evaluate pulmonary function in patients with pulmonary lymphangioleiomyomatosis (PLAM).

METHODS

Pulmonary function tests (PFTs) were performed in 30 patients with pathologically confirmed PLAM with the use of HRCT. These results were correlated with quantitative HRCT in 21 patients.

RESULTS

There were significant correlations between the HRCT parameters for lung function and PFT parameters. Among these parameters, emphysema volume (EV), pulmonary volume with a pixel index less than the trigger threshold (-950 HU) to account for a proportion of total lung volume [PI-950 (%)] and forced expiratory volume in 1 second/forced vital capacity [FEV1/FVC (%)] had the strongest correlations, reaching values between -0.71 and -0.68. HRCT lung function might therefore also be helpful for predicting changes in lung function before and after treatment.

CONCLUSIONS

HRCT is helpful for the assessment of pulmonary function in PLAM patients and can assist in the clinical evaluation of lung function and treatment response in patients with this disease.