Medical treatments for bronchiectasis in children

Treatment	Indications	Typical regimen	Mechanism of action	Evidence
Antibiotics	Acute exacerbations (all patients)	Amoxicillin-clavulanate for 14 days for mild exacerbations IV antibiotics for severe exacerbations or for patients who do not respond to a long course (4 weeks) of oral antibiotics	Decrease the bacterial load and interrupt the cycle of infection and inflammation Macrolide antibiotics (eg, azithromycin) are an alternative to amoxicillin-clavulanate and also have antiinflammatory effects	Antibiotics are superior to placebo to treat nonsevere exacerbations^[1]; treatment response is more rapid for amoxicillin-clavulanate compared with azithromycin^[2]
	Eradication of P. aeruginosa when first detected in sputum cultures	IV antipseudomonal antibiotics for 2 weeks, followed by inhaled tobramycin or colistin for 1 to 4 months	Pseudomonas can be eradicated when first detected but not after chronic infection is established	Expert consensus, based on indirect evidence from bronchiectasis in adults and early eradiation protocols in patients with cystic fibrosis^[3]
	Chronic treatment (for selected patients with frequent exacerbations)	Azithromycin 30 mg/kg/week orally, divided into daily, thrice-weekly, or weekly doses (maximum 1500 mg/week)*		Evidence is strongest for macrolides, which demonstrate 50% reduction in exacerbations^[4]
Airway clearance techniques	All children with bronchiectasis (guided by an expert physiotherapist)	Any of several techniques, including chest percussion, positive airway pressure, forced expirations, and exercise (choice depends on child's age and developmental stage)	Improves mucus clearance; may decrease cough frequency	RCTs in adults with stable-state bronchiectasis found that airway clearance techniques and exercise training improved exercise capacity, dyspnea, and fatigue with fewer pulmonary exacerbations^[5,6] No RCTs for exacerbations or in children
Mucolytics	Acute exacerbation (adjunctive therapy for patients with high sputum load)	Inhaled hypertonic saline (6 to 7%) twice daily, before airway clearance therapy (chest physiotherapy) Administer a bronchodilator prior to each treatment	Improves mucus clearance by reducing viscosity, enabling ciliary clearance and promoting cough	RCT in children with bronchiectasis showed higher mean improvement in predicted FEV₁ when inhaled hypertonic saline was added to conventional airway clearance therapy^[7] RCTs in adults with bronchiectasis had equivocal results for inhaled mannitol and NAC^[8,9,10]; rhDNAse can be harmful
Mucolytics	Chronic treatment (for selected patients with moderate or severe daily symptoms or difficulty in expectoration)	Inhaled hypertonic saline or dry-powder mannitol, administered as described above
Bronchodilators	Patients with coexistent asthma or bronchial hyperreactivity	Albuterol, etc	Improves airflow; may improve mucus clearance	Based on indirect evidence from management of asthma and clinical experience in children and adults with bronchiectasis and coexistent asthma^[3]
Inhaled glucocorticoids	Patients with coexistent asthma, airway eosinophilia, or bronchial hyperreactivity	Inhaled glucocorticoids, as indicated for the asthma	Decreases airway eosinophilic inflammation	Meta-analysis found insufficient evidence to support routine use in adults with bronchiectasis^[11]; no RCTs in children
Systemic glucocorticoids	Patients with acute asthma exacerbation Patients with ABPA	Brief course of oral glucocorticoids Oral glucocorticoids as indicated for treatment of ABPA	Antiinflammatory effects	No RCTs in adults or children with bronchiectasis^[12]; evidence from other populations suggests that systemic steroids may increase risk of P. aeruginosa, in addition to other adverse effects of systemic steroids
Vaccines	All patients with bronchiectasis	Ensure completion of all routine childhood vaccinations Annual influenza vaccine (inactivated form)^¶ Pneumococcal vaccination as recommended for high-risk patients^Δ	Provide immunity against influenza, pneumococcal respiratory infections, and other vaccine-preventable diseases	RCT in adults with bronchiectasis found that the 23-valent pneumococcal polysaccharide vaccine reduced acute pulmonary exacerbations^[13]

IV: intravenous; P. aeruginosa: Pseudomonas aeruginosa; RCT: randomized controlled trial; FEV₁: forced expiratory volume in 1 second; NAC: N-acetyl cysteine; rhDNAse, recombinant human deoxyribonuclease (dornase alpha); ABPA: allergic bronchopulmonary aspergillosis.
* For children who are chronically infected with P. aeruginosa and have frequent exacerbations, oral macrolides should be used as maintenance treatment. Inhaled tobramycin or colistin may be used as an alternative (in those who cannot tolerate macrolides) or in addition to the oral regimen.
¶ For influenza vaccination, the inactivated influenza vaccine (intramuscular) should be used for any child who might be immunocompromised. The live attenuated influenza vaccine (intranasal) should not be given if there is any possibility that the child is immunocompromised.
Δ All children with bronchiectasis should receive the standard pneumococcal conjugate vaccine series (usually the 13-valent conjugate [PCV13]), as is recommended for healthy children. In addition, vaccination with the 23-valent pneumococcal polysaccharide vaccine [PPV23] is recommended in some countries, including the United States, for children with bronchiectasis and other chronic respiratory illnesses.

References:

Goyal V, Grimwood K, Ware RS, et al. Efficacy of oral amoxicillin-clavulanate or azithromycin for non-severe respiratory exacerbations in children with bronchiectasis (BEST-1): a multicentre, three-arm, double-blind, randomised placebo-controlled trial. Lancet Respir Med 2019; 7:791.
Goyal V, Grimwood K, Byrnes CA, et al. Amoxicillin-clavulanate versus azithromycin for respiratory exacerbations in children with bronchiectasis (BEST-2): a multicentre, double-blind, non-inferiority, randomised controlled trial. Lancet 2018; 392:1197.
Chang AB, Fortescue R, Grimwood K, et al. Task Force report: European Respiratory Society guidelines for the management of children and adolescents with bronchiectasis. Eur Respir J 2021; 58:2002990.
Valery PC, Morris PS, Byrnes CA, et al. Long-term azithromycin for Indigenous children with non-cystic-fibrosis bronchiectasis or chronic suppurative lung disease (Bronchiectasis Intervention Study): a multicentre, double-blind, randomised controlled trial. Lancet Respir Med 2013; 1:610.
Murray MP, Pentland JL, Hill AT. A randomised crossover trial of chest physiotherapy in non-cystic fibrosis bronchiectasis. Eur Respir J 2009; 34:1086.
Muñoz G, de Gracia J, Buxó M, et al. Long-term benefits of airway clearance in bronchiectasis: a randomised placebo-controlled trial. Eur Respir J 2018; 51:1701926.
Anuradha KWDA, Gunathilaka PKG, Wickramasinghe VP. Effectiveness of hypertonic saline nebulization in airway clearance in children with non-cystic fibrosis bronchiectasis: A randomized control trial. Pediatr Pulmonol 2021; 56:509.
Hart A, Sugumar K, Milan SJ, et al. Inhaled hyperosmolar agents for bronchiectasis. Cochrane Database Syst Rev 2014; :CD002996.
Bilton D, Tino G, Barker AF, et al. Inhaled mannitol for non-cystic fibrosis bronchiectasis: a randomised, controlled trial. Thorax 2014; 69:1073.
Wilkinson M, Sugumar K, Milan SJ, et al. Mucolytics for bronchiectasis. Cochrane Database Syst Rev 2014; 2014:CD001289.
Kapur N, Petsky HL, Bell S, et al. Inhaled corticosteroids for bronchiectasis. Cochrane Database Syst Rev 2018; 5:CD000996.
Lasserson T, Holt K, Greenstone M. Oral steroids for bronchiectasis (stable and acute exacerbations). Cochrane Database Syst Rev 2001; 2001:CD002162.
Chang CC, Singleton RJ, Morris PS, Chang AB. Pneumococcal vaccines for children and adults with bronchiectasis. Cochrane Database Syst Rev 2009; 2009:CD006316.

Adapted from: Goyal V, Grimwood K, Marchant J, et al. Pediatric bronchiectasis: No longer an orphan disease. Pediatr Pulmonol 2016; 51:450.

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Medical treatments for bronchiectasis in children