Newly diagnosed cystic fibrosis in adults: Pattern and distribution of bronchiectasis in 12 cases

• Published in: Clinical radiology 1999-08, Vol.54 (8), p.507-512
• Main Authors: Mason, Andrew C, Nakielna, Barbara E.M
• Format: Article
• Language: English
• Subjects: Adult; Biological and medical sciences; bronchiectasis; Bronchiectasis - diagnostic imaging; Bronchiectasis - etiology; CFTR gene mutations; cystic fibrosis; Cystic Fibrosis - complications; Cystic Fibrosis - diagnostic imaging; Cystic Fibrosis - genetics; Cystic Fibrosis - physiopathology; Cystic Fibrosis Transmembrane Conductance Regulator - genetics; Female; Forced Expiratory Volume; Humans; lung; Male; Medical sciences; Middle Aged; Pneumology; Respiratory system : syndromes and miscellaneous diseases; Retrospective Studies; Tomography, X-Ray Computed; Vital Capacity
• ISSN: 0009-9260; 1365-229X
• DOI: 10.1016/S0009-9260(99)90847-4

OBJECTIVE: The aim of this study was to analyse the type and distribution of bronchiectasis at computed tomography (CT) in adults with recently diagnosed cystic fibrosis. METHODS: The CT examinations of 12 consecutive patients diagnosed with cystic fibrosis in adulthood (six male, six female; age range at diagnosis 25–63 years) were analysed retrospectively using a modified Bhalla scoring system. Bronchiectasis was catergorized by Reid type and by location within the affected lobe (peripheral, central or mixed). RESULTS: Bronchiectasis was identified in 70 of 71 lobes. Two or more bronchopulmonary segments were involved in 67 of 71 lobes. The majority of lobes demonstrated cylindrical bronchiectasis as the predominant type ( n = 51), with varicose ( n =11) and cystic ( n = 8) less commonly seen. Bronchiectasis was mainly central in 16 lobes, and both central and peripheral in 54 lobes. The severity of bronchial dilatation was greatest for the upper lobes (mean score, 1.75 right, 1.58 left) compared with the middle lobe/lingula (mean score, 1.42 right, 1.17 left) and lower lobes (mean score, 1.09 right, 1.17 left). CONCLUSION: Bronchiectasis in patients with cystic fibrosis diagnosed in adulthood is usually widespread, predominantly cylindrical, and is more severe in the upper lobes. The diagnosis of cystic fibrosis should be considered in adults with ‘idiopathic’ bronchiectasis showing these features on CT.