Primary ciliary dyskinesia: evolution of pulmonary function

Pulmonary function tests were obtained in 11 patients with primary ciliary dyskinesia. Their mean age was 15 years (range 6-32). Their pulmonary function was obstructive, with a vital capacity (mean +/- SD) of 75% +/- 20% predicted, a forced expiratory volume in 1s (FEV1) of 63% +/- 20% predicted and a raised residual volume of 169% +/- 50% predicted. After inhalation of 200 mu g of salbutamol the mean change in FEV1 was + 13.2% +/- 9.6% of the baseline value. In the 10 oldest patients, lung function had been measured at regular intervals during 3-20 years. Interestingly, during childhood and adolescence the evolution was not unfavourable: vital capacity increased by 8% +/- 20% and FEV1 remained stable (mean change 0.3% +/- 12%). Only 2 patients had an unfavourable evolution. Conclusion At time of diagnosis, patients with primary ciliary dyskinesia have partially reversible obstructive airway disease. During regular follow up and therapy, there is no evidence of a further decline in lung function. Patients with associated immunodeficiency or important damage at the start of therapy may have a worse prognosis.