OBSTRUCTIVE DISEASE OF THE AIRWAYS IN CYSTIC FIBROSIS

Abstract

A study of the interrelationships of instantaneous air flow, lung volume, and transpulmonary pressure over the range of the vital capacity has demonstrated striking differences in the determinants of maximum expiratory flow in cystic fibrosis and asthma. At high lung volumes, maximum expiratory flow rates in asthma are limited by the mechanical characteristics of the lungs and airways, whereas in cystic fibrosis and in the normal they are dependent on effort. At lower lung volumes, maximum expiratory flow rates are relatively more reduced in cystic fibrosis than in asthma and pressures in excess of those required to produce maximum flow actually depress flow. Also, forced expiration is associated with a transient reversal in the slope of the single breath nitrogen curve in cystic fibrosis and not in asthma. From these studies it is concluded that: (1) airway obstruction is less uniform and involves larger airways in cystic fibrosis than in asthma, and (2) increased expiratory pressure is associated with collapse of some of the larger airways over most of the range of the vital capacity in cystic fibrosis. A major clinical implication of these studies is that the effectiveness of cough is impaired by large airway collapse in cystic fibrosis.