pulmonary disorders
Bronchiectasis
Treatment of Bronchiectasis
Prevention of exacerbations with regular vaccinations and sometimes suppressive antibiotics Measures to help clear airway secretions Bronchodilators and sometimes inhaled corticosteroids if reversible airway obstruction is present Antibiotics and bronchodilators for acute exacerbations Early treatment with antivirals of any viral infections, particularly influenza and COVID-19 Sometimes surgical resection for localized disease with intractable symptoms or bleeding Lung transplantation in carefully selected patients who have advanced disease despite maximal therapy The key treatment goals are to control symptoms and improve quality of life, reduce the frequency of exacerbations, and preserve lung function ( Treatment references ). As for all patients with chronic pulmonary disease, Treatment None Smoking Cessation None Annual Influenza Vaccine None Pneumococcal Vaccine None COVID-19 Vaccine None Airway clearance techniques are used to reduce chronic cough in patients with significant sputum production and mucous plugging and to reduce symptoms during exacerbations. Such techniques include regular exercise, Chest Physiotherapy with postural drainage and chest percussion, positive expiratory pressure devices, intrapulmonary percussive ventilators, pneumatic vests, and autogenic drainage (a breathing technique thought to help move secretions from peripheral to central airways). Patients should be taught these techniques by a respiratory therapist and should use whichever one is most effective and sustainable for them; no evidence favors one particular technique. Patients should be advised to continue their airway clearance techniques for at least 10 minutes and may stop when they produce 2 clear coughs or huffs ( For patients with reversible airway obstruction, bronchodilator therapy (eg, with some combination of a long-acting beta-adrenergic agonist, a long-acting muscarinic antagonist, and a short-acting beta-adrenergic drug as indicated by symptoms and severity of lung obstruction, Treatment of Stable COPD ) can help improve lung function and quality of life. Inhaled corticosteroids may also be used in patients with frequent exacerbations or marked variability in lung function measurements (ie, reversible airway obstruction following bronchodilator administration), but their role remains controversial. Patients with cystic fibrosis may receive nebulized treatments, including a mucolytic (rhDNase, also calleddornase alfa) and hypertonic (7%) saline, to help reduce sputum viscosity and enhance airway clearance. In patients without CF, evidence of benefit with these measures is inconclusive, so only humidification and saline are recommended as inhaled treatments. Inhaled rhDNase may be harmful in patients with bronchiectasis not caused by CF. when they are first detected in sputum cultures ( Treatment references ). Chronic macrolide therapy reduces acute exacerbations in patients with bronchiectasis, and can slow the decline in lung function in patients with CF ( Treatment references ). Macrolides are thought to be beneficial mainly due to their anti-inflammatory or immunomodulatory effects. Inhaled antibiotics (eg,amikacin,aztreonam,ciprofloxacin,gentamicin,tobramycin) can reduce sputum bacterial load, and may also reduce the frequency of exacerbations. The evidence supporting their use and benefit is strongest in patients with CF. Patients with bronchiectasis who develop influenza or SARS-CoV-2 infection should be treated with antiviral medications to prevent complications and development of severe disease. For influenza, treatment is typically withoseltamivirregardless of the duration of viral symptoms. SARS-CoV-2 infections should be treated as soon as possible in patients with bronchiectasis, especially in those with underlying immune suppression. The preferred regimen isritonavir-boosted nirmatrelvir started within 5 days of symptom onset, but alternative options are available. Oral corticosteroids are not recommended for outpatients with COVID-19 in the absence of a co-existing condition, such as asthma or COPD, that is known to benefit from corticosteroids. Patients with bronchiectasis should be monitored closely for bacterial co-infection, and any exacerbation triggered by viral infection should be treated as outlined above. Treatment sessions for bronchiectasis should be done in the following order: Inhaled short-acting bronchodilators Mucolytic therapy (if prescribed) Airway clearance technique Any prescribed inhaled or nebulized antibiotics, long-acting bronchodilators, or corticosteroids Underlying conditions should be treated to slow the progression of lung disease. For patients with underlying immunodeficiency states: Scheduled intravenous immunoglobulin (which may reduce the frequency of lower respiratory infections [ Treatment references ]) For patients with Treatment : Antibiotics and inhaled bronchodilators as well as comprehensive support, and dietary supplementation. Most patients with cystic fibrosis benefit from For patients with Allergic Bronchopulmonary Aspergillosis (ABPA) : Corticosteroids and sometimes azole antifungals For patients with alpha-1 antitrypsin deficiency: Replacement therapy Acute exacerbations are treated with antibiotics, inhaled bronchodilators (particularly if patients are wheezing), and increased attempts at mucus clearance, using mechanical techniques, treatment of dehydration (if present), humidification, and nebulized saline (and mucolytics for patients with CF). Inhaled or oral corticosteroids are frequently given to treat airway inflammation and worsening airway obstruction. Antibiotic choice depends on previous culture results and whether or not patients have CF ( Treatment references ). Initial antibiotics for patients without CF and with no prior culture results should be effective againstH. influenzae,M. catarrhalis,S. aureus, andS. pneumoniae. Examples includeamoxicillin/clavulanic acid,azithromycin,clarithromycin, and sulfamethoxazole/trimethoprim. Patients with knownP. aeruginosacolonization or more severe exacerbations should receive antibiotics effective against this organism (eg,ciprofloxacin,levofloxacin) until repeat culture results are available. Antibiotics should be adjusted based on culture results and are given for a typical duration of 14 days, especially ifP. aeruginosais detected. Shorter courses are reserved for patients with mild disease. Initial antibiotic selection for patients with CF is guided by previous sputum culture results (done routinely in all patients with CF). During childhood, common infecting organisms areS. aureusandH. influenzae, and quinolone antibiotics such asciprofloxacinandlevofloxacinmay be used. In the later stages of CF, infections involve highly resistant strains of certain gram-negative organisms includingP. aeruginosa,Burkholderia cepacia, andStenotrophomonas maltophilia. In patients with infections caused by these organisms, treatment is with multiple antibiotics (eg,tobramycin,aztreonam,ceftazidime,cefepime). Intravenous administration is frequently required. Significant hemoptysis is usually treated with bronchial artery embolization, but surgical resection may be considered if embolization is ineffective and pulmonary function is adequate. Superinfection with Nontuberculous Mycobacterial Infections such as Surgical resection is rarely indicated but may be considered when bronchiectasis is localized, medical therapy has been optimized, and the symptoms are intolerable. In certain patients with diffuse bronchiectasis, especially cystic fibrosis, Lung and Heart-Lung Transplantation is also an option. 1.Polverino E, Gemine PC, McDonnell MJ, et al: European Respiratory Society guidelines for the management of adult bronchiectasis.Eur Respir J50: 1700629, 2017. doi: 10.1183/13993003.00629-2017 2.Nicholson CH, Holland AE, Lee AL:The Bronchiectasis Toolbox- A Comprehensive Website for the Management of People with Bronchiectasis.Med Sci(Basel) 5, 13, 2017. 3.Hill AT, Sullivan AL, Chalmers JD, et al: British Thoracic Society Guideline for bronchiectasis in adults.Thorax74(Suppl 1):1–69, 2019. doi: 10.1136/thoraxjnl-2018-212463 4.Wong C, Jayaram L, Kraals N, et al:Azithromycinfor the prevention of exacerbations in non-cystic fibrosis bronchiectasis (EMBRACE): A randomised, double blind, placebo controlled trial.Lancet380: 660–667, 2012. 5.Altenburg J, de Graaf CS, Stienetra Y, et al: Effect ofazithromycinmaintenance treatment on infectious exacerbations among patients with non-cystic fibrosis bronchiectasis: The BAT randomized controlled trial.JAMA309: 1251–1259, 2013. 6.Serisier DJ, Martin ML, McGuckin MA, et al: Effect of long-term, low doseerythromycinon pulmonary exacerbations among patients with non-cystic fibrosis bronchiectasis: the BLESS randomized controlled trial.JAMA309: 1260–1267, 2013. 7.Quinti I, Sorellina A, Guerra A, et al: Effectiveness of immunoglobulin replacement therapy on clinical outcome in patients with primary antibody deficiencies: Results from a multicenter prospective cohort trial.J Clin Immunol31: 315–322, 2011. 8.Flume PA, Mogayzel PJ Jr, Robinson KA, et al: Cystic fibrosis pulmonary guidelines: Treatment of pulmonary exacerbations.Am J Respir Crit Care Med80:802–808, 2009. doi: 10.1164/rccm.200812-1845PP 