SERIAL MEASUREMENTS OF FEV(1) OVER 12 YEARS IN A PATIENT WITH ALPHA-1-PROTEASE INHIBITOR DEFICIENCY - INFLUENCE OF AUGMENTATION THERAPY ANDINFECTIONS

In patients with severe alpha-1-protease inhibitor (alpha 1-Pi) defici ency forced expiratory volume in 1 s (FEV(1)) is an accepted parameter to monitor the progression of emphysema. In a patient with severe alp ha 1-Pi deficiency (PiZZ) more than 1,000 FEV(1) measurements were per formed over a period of 12 years. FEV(1) dramatically decreased initia lly (Delta FEV(1) > 500 ml/year), but stabilized after augmentation th erapy was instituted. Three years later, the FEV(1) decreased again ab ruptly; the deterioration was paralleled by an increasing number of se vere bronchopulmonary infections. This nonlinear decline implies a pos itive influence of augmentation therapy and a deleterious effect of br onchopulmonary infections in the disease (p < 0.0005). Daily variation of FEV(1) in infection-free intervals exceeded 30% and were thus high er than the mean decrease in FEV(1) per year. In this instance, FEV(1) measurements performed once or twice per year may reveal a deteriorat ion, whereas the change of FEV(1) is still within the range of spontan eous variation. More frequent measurements of FEV(1) can be useful to minimize the influence of high intraindividual variation.