Follow-up |
Functional aspects |
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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). |
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Microbiologic aspects |
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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. |
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Systemic markers |
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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. |
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Severity and prognostic scores |
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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. |
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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.
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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. |
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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. |
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Bronchodilators |
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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. |
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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. |
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Physical exercise and pulmonary rehabilitation |
Refer bronchiectasis patients with exertional limitation for regular exercise and participation in pulmonary rehabilitation programs, if available. |
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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. |
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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. |
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Vaccines |
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Bronchiectasis patients should receive influenza vaccine annually and should receive PCV13 and PPSV23 in the sequence recommended by the SBIm and the SBPT. |
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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. |
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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. |
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Surgical treatment |
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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 |
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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. |