Prevalence and Impact of Lung Resection in Primary Ciliary Dyskinesia: a cohort and nested case-control study

Rational Lung resection in Primary Ciliary Dyskinesia (PCD) is a controversial therapeutic modality. We aimed to assess the prevalence of lung resection in PCD across countries and compare disease course in lobectomised and non-lobectomised patients. Methods: In a study nested in the international iPCD cohort (n=2855), we identified lobectomised (cases) and non-lobectomised age, center and sex-matched (controls) PCD patients and compared their clinical characteristics, lung function and BMI cross-sectionally and longitudinally. Results: Data from 163 lobectomised PCD patients from 20 centers (15 countries) were available. The overall prevalence of lung resection in iPCD cohort was 5.7% (8.9% among adults), with wide variation among centers (range: 0% to 20%). Compared to the rest of iPCD cohort, lobectomised patients were more often females, older at diagnosis, and had more often situs solitus. Compared to controls (n=265), lobectomised patients had lower FVC (-2.15 vs -1.34, p Conclusion: Prevalence of lung resection in PCD varies widely between countries and is more frequent in patients with a delayed diagnosis. Most lobectomised PCD patients have poorer and steeper decline of lung function after intervention compared to controls.