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SPECIAL ARTICLE • J. bras. pneumol. 45 (04) • 2019 • https://doi.org/10.1590/1806-3713/e20190122 copy

Brazilian consensus on non-cystic fibrosis bronchiectasis

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

Bronchiectasis is a condition that has been increasingly diagnosed by chest HRCT. In the literature, bronchiectasis is divided into bronchiectasis secondary to cystic fibrosis and bronchiectasis not associated with cystic fibrosis, which is termed non-cystic fibrosis bronchiectasis. Many causes can lead to the development of bronchiectasis, and patients usually have chronic airway symptoms, recurrent infections, and CT abnormalities consistent with the condition. The first international guideline on the diagnosis and treatment of non-cystic fibrosis bronchiectasis was published in 2010. In Brazil, this is the first review document aimed at systematizing the knowledge that has been accumulated on the subject to date. Because there is insufficient evidence on which to base recommendations for various treatment topics, here the decision was made to prepare an expert consensus document. The Brazilian Thoracic Association Committee on Respiratory Infections summoned 10 pulmonologists with expertise in bronchiectasis in Brazil to conduct a critical assessment of the available scientific evidence and international guidelines, as well as to identify aspects that are relevant to the understanding of the heterogeneity of bronchiectasis and to its diagnostic and therapeutic management. Five broad topics were established (pathophysiology, diagnosis, monitoring of stable patients, treatment of stable patients, and management of exacerbations). After this subdivision, the topics were distributed among the authors, who conducted a nonsystematic review of the literature, giving priority to major publications in the specific areas, including original articles, review articles, and systematic reviews. The authors reviewed and commented on all topics, producing a single final document that was approved by consensus.

Keywords:
Bronchiectasis; Tomography, X-ray; Radiography, thoracic

Variables	Result	Score
Exacerbation	No	Zero
	Yes	2
FEV₁ ,% predicted	≥ 50%	Zero
	< 50%	2
Age	< 70 years	Zero
	≥ 70 years	2
Chronic colonization with P. aeruginosa	No	Zero
	Yes	1
Extent of CT findings: number of affected lobes	1-2 lobes	Zero
	> 2 lobes	1
Dyspnea, mMRC scale	0-II	Zero
	III-IV	1
		TOTAL: 0-9 points
Severity: 0-3 points: mild; 4-6 points: moderate; and 7-9 points: severe.

Condition or cause	Specific therapeutic measures
Allergic bronchopulmonary aspergillosis	Systemic corticosteroids, antifungal agents
Ciliary dyskinesia	Auditory monitoring, cardiac evaluation (malformations), guidance regarding difficulty in conceiving, mucociliary clearance techniques
Associated diseases (asthma, COPD, collagen diseases, inflammatory bowel disease, etc.)	Treatment of the underlying disease
Alpha-1 antitrypsin deficiency	Avoid tobacco exposure; consider replacement therapy in specific situations
Cystic fibrosis	DNase; consider CFTR modulators (if available and in the appropriate situation)
Immunodeficiencies	Periodic immunoglobulin replacement (i.v. or s.c.)
Nontuberculous mycobacterial infection	Treatment according to species and in accordance with national and international guidelines
Bronchial obstruction	Bronchoscopic clearance or surgical treatment
Gastroesophageal reflux disease	Inhibitor of acid gastric secretion; consider surgery

Treatment regimen	Dose	Frequency
Oral antibiotic + inhaled antibiotic
Oral: Ciprofloxacin +	500-750 mg^a	12/12 h for 14-21 days
Inhaled: Gentamicin or Nebulized tobramycin or Colistimethate**	80 mg 300 mg 1,000,000 IU	12/12 h for 3 months 12/12 h for 3 months 12/12 h for 3 months
Intravenous antibiotic (antipseudomonal beta-lactam + aminoglycoside) + inhaled antibiotic
Intravenous: Ceftazidime or Cefepime or Piperacillin + tazobactam or Meropenem +	2 g 2 g 4.5 g 2 g	8/8 h (14 days) 8/8 h (14 days) 6/6 h or 8/8 h (14 days) 8/8 h (14 days)
Intravenous: Amikacin or Gentamicin or Tobramycin +	20-30 mg/kg/day (max 1.5 g/day) 3-5 mg/kg/day (max 160 mg/day) 10 mg/kg/day (max 660 mg/day)	24/24 h (14 days) 24/24 h (14 days) 24/24 h (14 days)
Inhaled: Gentamicin or Nebulized tobramycin or Colistimethate^b	80 mg 300 mg 1,000,000 IU	12/12 h (3 months) 12/12 h (3 months) 12/12 h (3 months)

Antibiotic and formulation	Dose	Frequency
Nebulized colistimethate	1,000,000 IU	12/12 h continuously
Gentamicin^b	80 mg	12/12 h continuously (or in alternating cycles of 28 days)
Dry powder tobramycin	112 mg	12/12 h in alternating cycles of 28 days
Nebulized tobramycin	300 mg	12/12 h in alternating cycles of 28 days

Recommendations
Follow-up	Functional aspects		Perform spirometry with bronchodilator use every 6 months, lung volume assessment (if available) annually, and the six-minute walk test (at the physician’s discretion).
	Microbiologic aspects		Collect samples from the lower respiratory tract (sputum, for example) at regular intervals of 3-4 months and during pulmonary exacerbations for aerobic culture, as well as annually for culture for fungi and mycobacteria. If the patient is being treated with chronic macrolide therapy, culture for mycobacteria should be performed every 6 months.
	Systemic markers		Although CRP appears to be an inflammation-related marker and is available for use in clinical practice, there is insufficient evidence to recommend its routine use to assess disease severity.
	Severity and prognostic scores		A severity score and a prognostic estimate should be calculated at the time patients are diagnosed with bronchiectasis. Periodic calculation of the score (annually, for example) aids in therapeutic management. To date, the FACED and the E-FACED scores are the ones that have been validated for use in Brazil.
Treatment of stable patients	Chronic airway infection	Primary infection	Immediately following the first identification of P. aeruginosa in the sputum of a patient, the patient should be treated with a systemic antipseudomonal antibiotic combined with an inhaled antibiotic. Follow-up sputum culture is recommended 2-4 weeks after treatment completion.
		Chronic bronchial infection	Bronchiectasis patients with chronic Pseudomonas aeruginosa infection and exacerbations may benefit from and should be treated with long-term inhaled antibiotics. The choice will depend on the availability of and access to medication.
	Chronic inflammation	Macrolides	Use macrolides as continuous therapy for at least 6-12 months for bronchiectasis patients with at least two exacerbations per year. Prefer azithromycin. The use of macrolides may be considered, although there is no evidence, for patients with fewer than two exacerbations per year but with a history of severe exacerbations or primary or secondary immunodeficiency, those whose exacerbations have a significant impact on their quality of life, and those with more severe bronchiectasis. Active nontuberculous mycobacterial infection should be ruled out.
		Inhaled corticosteroids	There is insufficient evidence to support the routine use of inhaled corticosteroids in adults with bronchiectasis. Inhaled corticosteroid therapy may be justified in some subgroups of adults if there is associated asthma or COPD.
	Bronchodilators		There is insufficient data to recommend the routine use of BDs in bronchiectasis patients without dyspnea. Long-acting BDs may be recommended if bronchiectasis is associated with asthma or COPD. Because of the potential risk of bronchospasm resulting from the use of inhaled mucoactive drugs and inhaled antibiotics, it is suggested that BDs be used prior to using these drugs.
	Airway clearance	Respiratory therapy	Respiratory therapy techniques for improving mucociliary clearance should be applied and taught to all bronchiectasis patients with chronic production of secretions and/or (CT scan) signs of mucus plugging.
		Physical exercise and pulmonary rehabilitation	Refer bronchiectasis patients with exertional limitation for regular exercise and participation in pulmonary rehabilitation programs, if available.
		Osmotic agents	The use of hypertonic saline (6-7%) should be considered in bronchiectasis patients with persistent secretions despite other measures. Hypertonic saline should be first administered under supervision to assess for adverse effects (bronchospasm), which can be prevented or minimized by prior administration of a short-acting bronchodilator.
		Mucolytics	There is insufficient evidence to recommend the routine use of mucolytics in bronchiectasis patients. The use of DNase is contraindicated for adult non-cystic fibrosis bronchiectasis patients.
	Vaccines		Bronchiectasis patients should receive influenza vaccine annually and should receive PCV13 and PPSV23 in the sequence recommended by the SBIm and the SBPT.
	Chronic respiratory failure	Home oxygen therapy and noninvasive ventilation	In patients with chronic hypoxemia despite optimal clinical treatment, long-term home oxygen therapy is indicated. In clinically stable patients with chronic hypercapnic respiratory failure, noninvasive mechanical ventilation by BiPAP should be used as an adjuvant to cardiopulmonary rehabilitation and respiratory therapy.
		Lung transplantation	Lung transplantation should be considered for patients with FEV₁ < 30% of predicted or for those with higher FEV₁ values but with rapid lung function decline. Some factors, if present, should alert to the possibility of early referral of the patient for lung transplantation evaluation. These factors include severe and frequent exacerbations, with ICU admissions; recurrent or treatment-refractory pneumothorax or hemoptysis; chronic respiratory failure; and hypercapnia or pulmonary hypertension.
	Surgical treatment		Surgical treatment should be reserved for individuals with localized bronchiectasis refractory to clinical treatment, and video-assisted thoracoscopy is the procedure of choice.
Treatment of exacerbations			Once an exacerbation is diagnosed, the severity of the exacerbation should be determined in order to decide between home care and hospitalization. Before initiating antibiotic therapy (based on previous culture results), another sputum sample should be collected for microbiological analysis, the results of which will be used if there is no response to treatment. Adjuvant measures (use of corticosteroids, bronchodilators, respiratory therapy, and/or hypertonic agents) should be instituted based on clinical judgment.

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E-mail: jbp@sbpt.org.br

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