Aetiologies of bronchiectasis

CategoryCause/notesClinical phenotypeSpecific treatment
Post-infectionViral, bacterial, fungal, mycobacteria (usually classified separately)Past history of severe infection; classically unilobar bronchiectasisNo specific treatment
NTMM. avium and M. abscessus most frequentMiddle-aged or elderly; females with low BMI; middle lobe and lingual nodular bronchiectasis; cavitation; tree-in-budAntibiotic treatment
Post-TBM. tuberculosisUpper lobe most frequentlyNo specific therapy
ABPAHypersensitivity to A. fumigatusHistory of asthma (not universal); thick sputum; S. aureus in sputum; central bronchiectasis; fleeting infiltratesSteroids±antifungals
COPDSmoking, biomass exposureFixed airflow obstruction; smoking history; bilateral lower lobe; tubular bronchiectasisNo specific therapy
AsthmaNot universally accepted as a cause of bronchiectasisLong history of asthma; frequent exacerbations; neutrophilic airway inflammationInhaled corticosteroids, biologics e.g. anti-IgE and anti-IL5
Aspiration/inhalationForeign body aspiration, gastric contents aspiration, inhalation of corrosive substancesLower lobe bronchiectasisSpeech and language therapy, fundoplication, removal of exacerbating drugs
ObstructionBenign tumours, enlarged lymph nodesSingle lobe bronchiectasisRemoval of obstruction via bronchoscopy or thoracic surgery
Congenital defects of large airwaysMarfan syndrome, Mounier-Kuhn syndrome (tracheobronchomegaly), Williams–Campbell syndrome (bronchial cartilage deficiency)Specific features depending on the congenital defectDependant on the underlying disorder
AATDUnopposed protease activityCombined emphysema and bronchiectasisAugmentation therapy is available in some countries
Yellow nail syndromeLymphatic obstructionDystrophic nails, pleural effusions, rhinosinusitisLocal treatment for nails e.g. vitamin-E, management of lymphoedema
Immunological defectsPrimary: common variable immune deficiency, agammaglobulinemia, hyper-IgE syndrome; secondary: chemotherapy, immunosuppressant therapy, malignancy, HIV/AIDSVaried clinical pattern depending on the underlying cause; patient may give a history of non-respiratory infectionsIg replacement, prophylactic antibiotics, treatment of underlying disorder, removal of iatrogenic immunosuppression
Young's syndromeCause not knownBronchiectasis, rhinosinusitis and reduced fertilitySee ciliary disorders below
PCDGeneticMiddle lobe and lower lobe bronchiectasis; rhinosinusitis; middle ear infections; situs inversus in some casesRecognition and treatment of associated problems (including rhinosinusitis, middle ear disease, infertility, ectopic pregnancy), genetic counselling, intensive airway clearance
Systemic inflammatory diseaseRheumatoid arthritis, sarcoidosis, systemic lupus erythematosus, Sjögren syndromeVaried clinical pattern, often rapidly progressiveNo specific treatment
Inflammatory bowel diseaseUlcerative colitis, Crohn's syndrome, coeliac diseaseVaried clinical pattern often high sputum volumes and steroid responsiveInhaled and systematic corticosteroids, treatment of the underlying condition
Adult CFCFTR mutationsUpper lobe bronchiectasis; P. aeruginosa or S. aureus in sputum; non-respiratory manifestationsSpecialist multidisciplinary care in adult CF centres, recognition and treatment of non-respiratory manifestations, CFTR modulator/corrector therapy
Diffuse panbronchiolitisIdiopathic inflammatory diseaseMostly patients of Far Eastern ethnic originMacrolide antibiotics

NTM: nontuberculous mycobacteria; TB: tuberculosis; ABPA: allergic bronchopulmonary aspergillosis; COPD: chronic obstructive pulmonary disease; AATD: α1-antitrypsin deficiency; PCD: primary ciliary dyskinesia; CF: cystic fibrosis; M. avium: Mycobacterium avium; M. abscessus: Mycobacterium abscessus; M. tuberculosis: Mycobacterium tuberculosis; A. fumigatus: Aspergillus fumigatus; S. aureus: Staphylococcus aureus; BMI: body mass index; Ig: immunoglobulin; IL: interleukin; CFTR: cystic fibrosis transmembrane conductance regulator; P. aeruginosa: Pseudomonas aeruginosa.