The prevalence of primary ciliary dyskinesia (PCD) in adult patients with bronchiectasis in Dnipro (Ukraine)

Abstract

PCD is considered as a cause of bronchiectasis (Bx) in 1–13% patients (pts). In turn, statistics on the prevalence of PCD among adult pts with Bx does not exist in Ukraine and testing is not available in routine clinical practice. The study aimed to define the prevalence of pts with PCD as a cause of Bx in a single Bx center in Dnipro. Data from all pts who visited our Bx outpatient Dnipro clinic from November 2018 until January 2021 were retrospectively analyzed. PICADAR score were calculated to identify potential pts for further PCD diagnosing. nNO was measured by NIOX Vero in stable phase, the level of 77 nL/min was considered as cut-off. Totally, data of 82 pts were analyzed, 19 patients (23.2%) had≥5 scores by PICADAR (the median was 5(5;6) scores) and were considered as eligible for further testing. The age was from 23 to 67 years, median was 37(34;44) years, 5 were men (26.3%). The level of nNO was from 5 to 660 nL/min, the median was 131(19;339) nL/min. 7 pts (36.8% of tested pts and 8.5% of total Bx cohort) had the level of nNO≤77 nL/min: from 5 to 65 nL/min, the median level was 12(11;26) nL/min. The median age of these patients was 38(37;39) years (from 37 to 45 years), one was man (14.3%). The median PICADAR score of pts with nNO>77 nL/min was 5(5;5) scores, of pts with nNO≤77 nL/min – 6(6;9) scores, p=0.0001 by Mann-Witney test.

Conclusions: 36.8% of tested pts and 8.5% of total Bx cohort had the level of nNO considered as positive test for PCD. The age of these pts was from 37 to 45 years, which indicates a significant diagnostic delay. These results confirm the need of PCD testing in adults with Bx.