What question did this study set out to answer?

To explore the incidence and predictors of pulmonary exacerbations in individuals with primary ciliary dyskinesia (PCD).

January 22, 2026ERJ Open ResearchOpen Access

Incidence and predictors of self-reported pulmonary exacerbations in primary ciliary dyskinesia: an international prospective cohort study

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Cohort study examines predictors of pulmonary exacerbations in primary ciliary dyskinesia, indicating healthcare needs.

To explore the incidence and predictors of pulmonary exacerbations in individuals with primary ciliary dyskinesia (PCD).
Conducted a prospective cohort study from June 2020 to May 2022.
Participants reported respiratory symptoms via online questionnaires.
Used multivariable negative binomial regression to analyze incidence rates and predictors.
660 individuals reported 1026 exacerbations, yielding an incidence rate of 3.1 exacerbations per person per year.
Higher incidence found in adult females (IRR 2.0) and with Pseudomonas aeruginosa isolation.
Healthcare professionals were consulted during only 13% of exacerbation weeks.

Schreck et al. (2025) studied this question.

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