Chest · 2026
Longitudinal Changes in Lung Morphologic Features and Perfusion Detected by MRI in Patients With Primary Ciliary Dyskinesia From Infancy Through Adulthood.
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Wucherpfennig Lena, Silani Margherita S, Eichinger Monika, Triphan Simon M F, Stahl Mirjam, Graeber Simon Y, Thee Stephanie, Urbantat Ruth M, Döllinger Felix, Hämmerling Susanne, Sommerburg Olaf, Schenk Jens-Peter, Alrajab Abdulsattar, Heussel Claus P, Wege Sabine, Kauczor Hans-Ulrich, Wielpütz Mark O, Mall Marcus A
Аннотация
BACKGROUND: MRI revealed a high prevalence of lung abnormalities in children with primary ciliary dyskinesia (PCD). However, longitudinal imaging data on the onset and progression of PCD are lacking.
RESEARCH QUESTION: When do abnormalities in lung morphologic features and perfusion in patients with PCD first emerge, and how do they progress longitudinally from infancy through adulthood as assessed by MRI?
STUDY DESIGN AND METHODS: One hundred eighty-nine MRI examinations (mean [SD] examinations per patient, 2.4 [1.7] examinations; range, 1-10 examinations) from 75 patients with PCD (mean [SD] age, 17.7 [15.0] years; range, 0-65 years) were included. MRI was assessed using the chest MRI scoring system by 2 independent readers. Spirometry was performed at the time of MRI, and the Bronchiectasis Severity Index (BSI) was calculated in adult patients.
RESULTS: Bronchiectasis or wall thickening, mucus plugging, consolidation, and perfusion abnormalities were highly prevalent at infancy (100%, 80%, 60%, and 50%, respectively). Prevalence of mucus plugging, consolidation, and perfusion abnormalities increased at adulthood (97%, 80%, and 97%, respectively; P < .05). The mean (SD) MRI global score was 14.5 (4.5) at infancy, showed stability from preschool age through school age and adolescence (18.4 [6.7], 19.8 [8.2], and 18.5 [9.6]; P = .386-.529, respectively), and an elevation in adulthood (26.7 [7.2]; P < .001). MRI morphologic features, perfusion, and global score correlated weakly with age (r = 0.20-0.38; P < .05) and showed moderate to strong correlations with FEV1 % predicted (r = -0.44 to -0.71; P < .001) and BSI (r = 0.44-0.49; P < .001).
INTERPRETATION: Our results show an early onset of lung disease in PCD already in infancy and a progressive increase in the prevalence and extent of changes in lung morphologic features and perfusion at adulthood. These results support the use of MRI as an end point in clinical trials in patients with PCD.
RESEARCH QUESTION: When do abnormalities in lung morphologic features and perfusion in patients with PCD first emerge, and how do they progress longitudinally from infancy through adulthood as assessed by MRI?
STUDY DESIGN AND METHODS: One hundred eighty-nine MRI examinations (mean [SD] examinations per patient, 2.4 [1.7] examinations; range, 1-10 examinations) from 75 patients with PCD (mean [SD] age, 17.7 [15.0] years; range, 0-65 years) were included. MRI was assessed using the chest MRI scoring system by 2 independent readers. Spirometry was performed at the time of MRI, and the Bronchiectasis Severity Index (BSI) was calculated in adult patients.
RESULTS: Bronchiectasis or wall thickening, mucus plugging, consolidation, and perfusion abnormalities were highly prevalent at infancy (100%, 80%, 60%, and 50%, respectively). Prevalence of mucus plugging, consolidation, and perfusion abnormalities increased at adulthood (97%, 80%, and 97%, respectively; P < .05). The mean (SD) MRI global score was 14.5 (4.5) at infancy, showed stability from preschool age through school age and adolescence (18.4 [6.7], 19.8 [8.2], and 18.5 [9.6]; P = .386-.529, respectively), and an elevation in adulthood (26.7 [7.2]; P < .001). MRI morphologic features, perfusion, and global score correlated weakly with age (r = 0.20-0.38; P < .05) and showed moderate to strong correlations with FEV1 % predicted (r = -0.44 to -0.71; P < .001) and BSI (r = 0.44-0.49; P < .001).
INTERPRETATION: Our results show an early onset of lung disease in PCD already in infancy and a progressive increase in the prevalence and extent of changes in lung morphologic features and perfusion at adulthood. These results support the use of MRI as an end point in clinical trials in patients with PCD.