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Bronchiectasis

Definition
Bronchiectasis is a chronic lung condition, which presents with permanent and abnormal dilation of the bronchi, and is characterized by mild to moderate airway obstruction and productive cough.
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Pathophysiology
Causes of bronchiectasis include sequelae of previous bacterial infection or mycobacterial infection (e.g., following pneumonia, whooping cough, or tuberculosis), as well as allergic bronchopulmonary aspergillosis, rheumatoid arthritis, ulcerative colitis, toxic lung injury, primary ciliary dyskinesia, COPD, and AAT deficiency. Impairment of airway defense mechanisms due to inflammation and persistent infection results in bronchiectasis.
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Disease course
Clinical manifestations include productive cough, excessive sputum, hemoptysis, dyspnea, chest pain, and recurrent chest infections. The disease can progress to chronic respiratory failure.
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Prognosis and risk of recurrence
The in-hospital and 1-year mortality in patients with acute exacerbation of bronchiectasis is 9% and 30%, respectively.
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Key sources
The following summarized guidelines for the evaluation and management of bronchiectasis are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2021), the European Respiratory Society (ERS 2021; 2017), the British Thoracic Society (BTS 2020), and the Canadian Thoracic Society (CTS 2012).
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Guidelines

1.Screening and diagnosis

Diagnostic criteria: define clinically significant bronchiectasis as the presence of both permanent bronchial dilatation on CT scanning and the clinical syndrome of cough, sputum production and/or recurrent respiratory infections.
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2.Diagnostic investigations

Initial investigations
Consider obtaining the following minimum bundle of etiological tests in adults with a new diagnosis of bronchiectasis:
CBC
serum immunoglobulins (total IgG, IgA and IgM)
testing for allergic bronchopulmonary aspergillosis
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Obtain three sequential daily sputum cultures for mycobacterial cultures or a single BAL in patients with radiological features of nontuberculous mycobacterial infection or clinical features such as weight loss, hemoptysis, rapid deterioration or symptoms non-responsive to standard therapy
consider screening for evidence of connective tissue disease by history and physical examination, and not routinely testing for autoantibodies in patients without signs and symptoms of connective tissue disease. (Ungraded)
consider testing for AAT deficiency in patients with basal emphysema or early onset airflow obstruction. (Ungraded)
consider measuring antibody responses to S. pneumoniae 23 valent polysaccharide vaccine (PPV23) in order to identify individuals with specific polysaccharide antibody deficiency (ungraded)
consider testing for cystic fibrosis with measurement of sweat chloride, other biomarkers of CFTR-mediated chloride ion transport and CFTR gene mutation analysis in young adults or in patients with specific clinical features of cystic fibrosis, such as upper lobe predominance of bronchiectasis on chest CT, the presence of nasal polyposis and/or chronic rhinosinusitis, recurrent pancreatitis, male primary infertility and/or malabsorption. (ungraded)
consider testing for primary ciliary dyskinesia with nasal nitric oxide, high-speed video analysis, transmission electron microscopy, immunofluorescence and/or genetic testing in patients with several of the following features: persistent wet cough since childhood, situs anomalies, congenital cardiac defects, nasal polyposis and/or chronic rhinosinusitis, chronic middle ear disease with or without hearing loss, a history of neonatal respiratory distress or neonatal intensive care admittance in term infants. (ungraded)

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  • Testing for AAT deficiency

3.Medical management

Inhaled bronchodilators
Consider using long-acting bronchodilators on an individual basis for patients with significant breathlessness.
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Consider avoiding routine long-acting bronchodilators for adult patients with bronchiectasis.
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  • ICSs

  • Pseudomonas eradication

  • Long-term inhaled antibiotics

  • Long-term oral antibiotics

  • Mucoactive agents

  • Statin therapy

  • Management of acute exacerbations

4.Nonpharmacologic interventions

Airway clearance techniques: refer patients with chronic productive cough or difficulty expectorating sputum to a trained respiratory physiotherapist who can teach airway clearance techniques.
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  • Pulmonary rehabilitation programs

5.Surgical interventions

Indications for surgery: consider avoiding surgical treatments for adult patients with bronchiectasis with the exception of patients with localized disease and a high exacerbation frequency despite optimisation of all other aspects of their bronchiectasis management.
D

6.Specific circumstances

Patients with Sjögren's disease: manage patients withSjögren's disease and clinically relevant bronchiectasis similarly to primary or secondary bronchiectasis of other etiologies and consider using any of the following:
mucolytic agents/expectorants
nebulized saline or hypertonic saline
oscillatory positive expiratory pressure
postural drainage
mechanical high-frequency chest wall oscillation therapies
chronic macrolides in those without non-tuberculous mycobacterium colonization or infection
B

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  • Pediatric patients (evaluation)

  • Pediatric patients (nutrition and exercising)

  • Pediatric patients (counseling)

  • Pediatric patients (ICSs)

  • Pediatric patients (antibiotics)

  • Pediatric patients (mucoactive agents)

  • Pediatric patients (surgical interventions)

  • Pediatric patients (routine immunizations)

  • Pediatric patients (monitoring)