Abstract

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<0.001) and FEV1 (-2.68 vs -1.96, p=0.002) z-scores at first post-lobectomy assessment and steeper lung function decline over time after lobectomy (FVC z-score slope: -0.04/year vs – 0.01/year, p=0.048, FEV1 z-score slope: -0.05/year vs -0.03/year, p=0.156). Among cases, females and patients with multiple lobe resections had significantly lower lung function. A minority of PCD patients demonstrated improvement of lung function after surgical intervention.

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.