Airway obstruction and airway wall instability in cystic fibrosis: the isolated and combined effect of theophylline and sympathomimetics.

Multiple aspects of lung function were measured in 17 cystic fibrosis (CF) patients on four occasions: without therapy (0); with oral theophylline medication (Th); after inhalation of salbutamol (beta 2); and with combined medication (Th + beta 2). In addition to routine measurements, partial and maximum expiratory flow-volume (MEFV) curves were superimposed, and the flow transient equivalent of the MEFV curve was determined. Its volume dimension (volume of airway contribution, VACMEFV) partially reflects airway distensibility. Changes in airway compressibility--the other consequence of airway wall instability--were assessed by observing changes in end-expiratory flow rate. Airway resistance, expired volumes, and early expired flow rates, as well as VACMEFV improved significantly after beta 2 medication. Mean end-expiratory flow also increased after beta 2; in two patients, however, it decreased significantly, indicating that enhanced airway compression dominated over the release of bronchospasm. The alone had only minor effects on lung function. Early expired volume and flow rates as well as VACMEFV showed no significant difference between beta 2 alone and Th + beta 2; airway resistance even decreased significantly with this drug combination. End-expiratory flow rate, however, was significantly lower after Th + beta 2 than after beta 2 alone. Although theophylline does not alter lung function in most patients with CF, sympathomimetics relieve bronchospasm in many, but they enhance airway compressibility and thereby decrease peripheral expiratory airflow in some.(ABSTRACT TRUNCATED AT 250 WORDS)