Guideline sources The following summarized guidelines for the evaluation and management of bronchiectasis are prepared by our editorial team based on guidelines from the European Respiratory Society (ERS 2021; 2017), the American College of Rheumatology (ACR 2021), the British Thoracic Society (BTS 2020), and the Canadian Thoracic Society (CTS 2012). 1 2 3 4 5 6 7 8 8 9 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. 6 Epidemiology 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. 7 8 Di https://web.pathway.md/diseases/recSF4xa2pybKp3wS 1/7 6/29/23, 3:21 AM Bronchiectasis Pathway 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. 8 Prognosis and risk of recurrence The in-hospital and 1-year mortality in patients with acute exacerbation of bronchiectasis is 9% and 30%, respectively. 9 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. 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. C 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) https://web.pathway.md/diseases/recSF4xa2pybKp3wS 2/7 6/29/23, 3:21 AM Bronchiectasis Pathway 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). Testing for alpha-1 antitrypsin deficiency: avoid obtaining targeted testing for AAT deficiency in patients with bronchiectasis. D 3. Medical management Inhaled bronchodilators: Consider using long-acting bronchodilators on an individual basis for patients with significant breathlessness. C Consider avoiding routine long-acting bronchodilators for adult patients with bronchiectasis. D Inhaled corticosteroids: consider avoiding routine treatment with ICSs to adults with bronchiectasis. D Pseudomonas eradication: consider offering antibiotic eradication therapy to adults with bronchiectasis in whom P. aeruginosa is newly isolated in sputum cultures. C Long-term inhaled antibiotics: Consider initiating long-term treatment with an inhaled antibiotic for adults with bronchiectasis and chronic P. aeruginosa infection who have three or more exacerbations per year, we suggest. C Consider initiating long-term treatment with an inhaled antibiotic for adults with bronchiectasis not infected with P. aeruginosa in whom oral antibiotic prophylaxis is contraindicated, not tolerated or ineffective. C Long-term oral antibiotics: As per BTS 2020 guidelines: Consider offering the following options for long-term macrolide treatment, for a minimum of 6 months, to reduce exacerbations in patients with 3 exacerbations per year: B azithromycin 250 mg daily; consider starting azithromycin at 250 mg thrice weekly to minimize side effect risk with subsequent titration according to clinical response azithromycin 500 mg thrice weekly erythromycin ethylsuccinate 400 mg BID. C Consider offering long-term macrolides for a longer period of therapy (such as 1 year) with the aim of improving quality of life in patients with bronchiectasis. C As per ERS 2017 guidelines, consider offering long-term antibiotic treatment for adults with bronchiectasis who have three or more exacerbations per year. C https://web.pathway.md/diseases/recSF4xa2pybKp3wS 3/7 6/29/23, 3:21 AM Bronchiectasis Pathway Landmark trials: BAT In adult patients with non-cystic fibrosis bronchiectasis and 3 lower respiratory tract infections in the preceding year, azithromycin was superior to placebo with respect to exacerbation, at least 1. Altenburg J et al. JAMA. 2013 Mar 27. Mucoactive agents: consider offering long-term mucoactive treatment ( 3 months) in adult patients with bronchiectasis who have difficulty in expectorating sputum and poor quality of life, where standard airway clearance techniques have failed to control symptoms. C Statin therapy: avoid offering statins to treat bronchiectasis. D Management of acute exacerbations: consider treating acute exacerbations of bronchiectasis with 14 days of antibiotics. C 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. B Pulmonary rehabilitation programs: refer adult patients with bronchiectasis and impaired exercise capacity to a pulmonary rehabilitation program, and advise regular exercise. Tailor interventions to the patient's symptoms, physical capability, and disease characteristics. A 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 Sj gren's disease: Manage patients with Sj gren'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 https://web.pathway.md/diseases/recSF4xa2pybKp3wS 4/7 6/29/23, 3:21 AM Bronchiectasis Pathway Pediatric patients (evaluation): Consider obtaining a minimum panel of the following tests in pediatric/adolescent patients with suspected or confirmed bronchiectasis: chest CT (to diagnose bronchiectasis) sweat test pulmonary function tests CBC immunological tests (total IgG, IgA, IgM, IgE, specific antibodies to vaccine antigens) lower airway bacteriology. C Show 5 more Pediatric patients (nutrition and exercising): Consider optimizing nutrition including vitamin D status in pediatric/adolescent patients with bronchiectasis. C Consider encouraging exercising on an ongoing basis in pediatric/adolescent patients with bronchiectasis, recognizing that short periods of exercise training are unlikely to have a long- term effect. C Pediatric patients (counseling): teach and provide regular airway clearance techniques or maneuvers in pediatric/adolescent patients with bronchiectasis. B Show 3 more Pediatric patients (inhaled corticosteroids): avoid using routine corticosteroids with or without long-acting -agonists in either the short or long-term, irrespective of stability or exacerbation, in pediatric/adolescent patients with bronchiectasis. D Pediatric patients (antibiotics): administer a systemic course of an appropriate antibiotic for 14 days in pediatric/adolescent patients with bronchiectasis and an acute respiratory exacerbation. B Show 2 more Pediatric patients (mucoactive agents): Do not use recombinant human deoxyRNAase routinely in pediatric/adolescent patients with bronchiectasis. D Avoid using bromhexine, inhaled mannitol or hypertonic saline routinely in pediatric/adolescent patients with bronchiectasis. D Pediatric patients (surgical interventions): Take into account the following factors when considering surgery in pediatric/adolescent patients with bronchiectasis: age symptoms and disease burden localization of the bronchiectatic areas on chest CT underlying etiology (influencing recurrence of disease) facility where surgery is undertaken (surgical expertise and availability of pre- and postoperative care) https://web.pathway.md/diseases/recSF4xa2pybKp3wS 5/7 6/29/23, 3:21 AM Bronchiectasis Pathway optimization of the patient's clinical state. B Pediatric patients (routine immunizations): consider ensuring that pediatric/adolescent patients with bronchiectasis are fully immunized according to their national immunization programs, including pneumococcal and annual seasonal influenza vaccines if these are not part of this program. C Pediatric patients (monitoring): consider reviewing pediatric/adolescent patients with bronchiectasis every 3-6 months in outpatient clinics to monitor their general wellbeing, respiratory status, including lung function when age appropriate and to detect any complications. C Show 5 more Clinical findings Symptoms Past medical history Acute cough Allergic bronchopulmonary aspergillosis Chest pain Aspiration pneumonia Chills Cilia abnormalities Coughing up yellow or green mucus daily Cystic fibrosis Dyspnea IBD Fatigue Immunocompromising condition Fever Measles Hemoptysis Recurrent pneumonia Sputum production Repeated infections Rheumatoid arthritis Respiratory exam Tuberculosis Whooping cough Dyspnea Wheezing Integument exam Digital clubbing Studies 2022 LDPEEPPC In patients undergoing selective lung resection surgery, PEEP titration to the lowest driving pressure was superior to conventional low level of PEEP with respect to the rate of development of 4 Melbourne Group Scale variables within 3 postoperative days. Junjie Yu et al. Anaesth Crit Care Pain Med. 2022 Sep 19. 2014 EMBRACE (azithromycin) https://web.pathway.md/diseases/recSF4xa2pybKp3wS 6/7 6/29/23, 3:21 AM Bronchiectasis Pathway In adult patients with non-cystic fibrosis bronchiectasis with 1 pulmonary exacerbation requiring antibiotic treatment in the past year, azithromycin was superior to placebo with respect to a event- based exacerbations. Gladstone DJ et al. N Engl J Med. 2014 Jun 26. Show 2 more References 1. Anne B Chang, Rebecca Fortescue, Keith Grimwood et al. European Respiratory Society guidelines for the management of children and adolescents with bronchiectasis. Eur Respir J. 2021 Aug 26;58 2 2002990. Open 2. Polverino E, Goeminne PC, McDonnell MJ et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J. 2017 Sep 9;50 3 . pii: 1700629. Open 3. David Smith, Ingrid Du Rand, Charlotte Louise Addy et al. British Thoracic Society guideline for the use of long-term macrolides in adults with respiratory disease. Thorax. 2020 May;75 5 370 404. Open 4. Darcy D Marciniuk, P Hernandez, M Balter et al. Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline. Can Respir J. 2012 Mar- Apr;19 2 109 16. Open 5. Augustine S Lee, R Hal Scofield, Katherine Morland Hammitt et al. Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sj gren's. Chest. 2021 Feb;159 2 683 698. Open 6. Paul T King. The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis. 2009;4 411 9. Open 7. No authors listed. Bronchiectasis. Breathe Sheff). 2018 Mar;14 1 73 80.2018 Mar;14 1 73 80. Open 8. Maeve P Smith. Diagnosis and management of bronchiectasis. CMAJ. 2017 Jun 19;189 24 E828 E835.2017 Jun 19;189 24 E828 E835. Open 9. James D Finklea, Gul Khan, Sheree Thomas et al. Predictors of mortality in hospitalized patients with acute exacerbation of bronchiectasis. Respir Med. 2010 Jun;104 6 816 21. Open 10. Hill AT, Sullivan AL, Chalmers JD et al. British Thoracic Society Guideline for bronchiectasis in adults. Thorax. 2019 Jan;74 Suppl 1 1 69. Open 11. Maeve P Smith. Diagnosis and management of bronchiectasis. CMAJ. 2017 Jun 19;189 24 E828 E835. Open 12. No authors listed. Bronchiectasis. Breathe Sheff). 2018 Mar;14 1 73 80. Open 13. Paul T King. The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis. 2009;4 411 9.2009;4 411 9. Open 14. James D Finklea, Gul Khan, Sheree Thomas et al. Predictors of mortality in hospitalized patients with acute exacerbation of bronchiectasis. Respir Med. 2010 Jun;104 6 816 21.2010 Jun;104 6 816 21. Open https://web.pathway.md/diseases/recSF4xa2pybKp3wS 7/7