The following is a summary of “Lung function from school age to adulthood in primary ciliary dyskinesia” published in the October 2022 issue of Respiratory by Halbeisen et al.

Primary ciliary dyskinesia (PCD) causes symptoms to show up early in life, and the disease may get worse over time, but there aren’t many long-term studies on how well the lungs work. This multinational cohort study shows how the lung function of children, teens, and young adults with PCD changes over time. Researchers looked at data from 486 patients with repeated lung function measurements taken between the ages of 6 and 24 from the International PCD Cohort. 

They used references from the Global Lung Function Initiative 2012 to calculate z-scores for forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), and the FEV1/FVC ratio. They talked about how the lungs worked at the start and how they changed over time. Researchers also discussed their relationships with possible causes in mixed-effects linear regression models. Overall, FEV1, FVC, and FEV1/FVC z-scores went down over time (the average annual decline in FEV1 was 0.07 z-scores), but not all patients’ scores went down at the same rate. FEV1 z-scores got better for 21% of patients over time, stayed the same for 40%, and got worse for 39%. Low body mass index was linked to poor lung function at the start and to more decline. 

Results were different by country and type of ultrastructural defect, but there was no evidence that they were different by gender, calendar year of diagnosis, age at diagnosis, diagnostic certainty, or side of the body where the defect was found. The study shows that the average lung function of people with PCD gets worse from childhood to young adulthood. This is true even for people who get treatment at specialized centers. It is important to come up with ways to stop this trend and improve the prognosis.