Lung function in children and adolescents with idiopathic interstitial pulmonary fibrosis

Abstract

Lung function of 65 patients who had idiopathic interstitial pulmonary fibrosis (IIPF) that had been treated with prednisone was evaluated by tests of ventilatory function, lung mechanics, and gas exchange at rest and during exercise. Ages on initial investigation ranged from 5 to 20 years. In 35 of 65 patients the studies were repeated an average of four times over a period of 1 to 9 years. Results of the first testing were as follows: vital capacity (VC)-significantly reduced in all patients; inspiratory capacity (IC)-significantly reduced in all patients; total lung capacity (TLC)-reduced in 91%; functional residual capacity (FRC)-reduced in 31%; residual volume (RV)-reduced in 6%; elastic recoil of the lungs (Pstl)-significantly increased in 97% at 100% TLC, significantly increased in 52% at 90% TLC, reduced in 68% at 60% TLC; 7) static compliance (Cst)-reduced in 83%; 8) dynamic compliance (Cdyn)-reduced in 88%; 9) specific airway conductance at FRC level (Gaw/TGVex)-significantly increased in 50%; 10) maximum expiratory flow rates at 60% TLC (Vmax 60% TLC, in TLC/s)-significantly reduced in 33%; 11) upstream airway conductance (Gus 60% TLC, in TLC/s/cm H2O)-reduced in 32%; 12) diffusing capacity of the lungs for carbon monoxide (DLco) related to body-surface area-abnormal in 58% (when corrected for lung size, i.e., DLco/TLC, abnormal in only 8%); 13) PaO2 at rest and after 6 minutes submaximal exercise-reduced in 25% and 63%, respectively. Changes in lung function that occurred with growth were assessed in terms of percentages of predicted values. Results showed that the VC and IC remained significantly reduced. An actual reduction of TLC, FRC, RV, breathing frequency, DLCO, and Pstl at 100% and 90% TLC was observed. Increases were seen in Pstl at 60% TLC, Gaw/TGVex, Vmax, and Cst. Indices of lung elasticity suggested that regions of fibrosis and emphysema had become present. Smaller patients were also noted to have stiffer lungs.