Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 9-07: Bronchiectasis + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ Chronic productive cough with dyspnea and wheezing Radiographic findings of dilated, thickened airways and scattered, irregular opacities +++ General Considerations ++ A congenital or acquired disorder of large bronchi characterized by abnormal dilation and destruction of bronchial walls May be localized or diffuse May be caused by recurrent inflammation or infection Cystic fibrosis causes 50% of all cases Causes Cystic fibrosis Infection Tuberculosis Fungal Abscess Pneumonia Abnormal lung defense mechanisms Hypogammaglobulinemia Common variable immunodeficiency Selective IgA, IgM, and IgG subclass deficiency Acquired immunodeficiency from cytotoxic drugs, AIDS, lymphoma, leukemia, plasma cell myeloma, chronic kidney disease, chronic liver disease Alpha-1-antiprotease deficiency with cigarette smoking Mucociliary clearance disorders (eg, immotile cilia syndrome) Rheumatic disease (eg, rheumatoid arthritis) Localized airway obstruction + Clinical Findings Download Section PDF Listen +++ +++ Symptoms and Signs ++ Chronic cough with production of copious, purulent sputum Recurrent pneumonia Hemoptysis Pleuritic chest pain Dyspnea and wheezing Weight loss and anemia common Persistent basilar crackles commonly found on examination Clubbing Infrequent in mild cases Present in severe disease Obstructive pulmonary dysfunction with hypoxemia seen in moderate or severe disease Haemophilus influenzae is the most common organism recovered from noncystic fibrosis patients with bronchiectasis Pseudomonas aeruginosa, Streptococcus pneumoniae, and Staphylococcus aureus are commonly identified; nontuberculous mycobacteria are seen less commonly +++ Differential Diagnosis ++ Chronic obstructive pulmonary disease Asthma Bronchiolitis Allergic bronchopulmonary mycosis + Diagnosis Download Section PDF Listen +++ +++ Laboratory Tests ++ Sputum smear and culture for bacterial, mycobacterial, and fungal organisms Quantitative pilocarpine iontophoresis sweat test for sodium and chloride levels Quantitative immunoglobulins Alpha-1-antiprotease level Excluding patients with humoral immunodeficiencies, most patients have panhypergammaglobulinemia, reflecting an immune response to chronic airway infection +++ Imaging Studies ++ Chest radiographs Show dilated, thickened bronchi that may appear as "tram-tracks" or as ring-like markings Scattered irregular opacities, atelectasis, and focal consolidation may be present High-resolution CT scanning is the diagnostic test of choice + Treatment Download Section PDF Listen +++ +++ Medications ++ Antibiotics should be used in acute exacerbations Empiric therapy for 10–14 days with Amoxicillin or amoxicillin clavulanate (500 mg every 8 hours orally) Ampicillin or tetracycline (250–500 mg four times daily orally) Trimethoprim-sulfamethoxazole (160/800 mg every 12 hours orally) Ciprofloxacin (500–750 mg twice daily orally) Sputum smears and cultures should guide therapy where possible Preventive or suppressive antibiotics are frequently given to patients with increased purulent sputum, although this practice is not guided by clinical trial data Long-term macrolide therapy (azithromycin 500 mg three times a week orally for 6 months or 250 mg/day for 12 months) has been found to decrease the frequency of exacerbations compared to placebo High-dose (3 g/day) amoxicillin orally or alternating cycles of amoxicillin or amoxicillin clavulanate, ampicillin or tetracycline, trimethoprim-sulfamethoxazole, or ciprofloxacin orally for 2–4 weeks are also used, although this practice is not supported by clinical trial data In patients with underlying cystic fibrosis, inhaled aerosolized aminoglycosides reduce colonization by Pseudomonas species, improve FEV1 and reduce hospitalizations In noncystic fibrosis bronchiectasis, inhaled aztreonam is of no benefit, while adding inhaled tobramycin to oral ciprofloxacin for acute exacerbations due to Pseudomonas decreases microbial sputum burden but without apparent clinical benefit Inhaled bronchodilators are commonly used as maintenance therapy and in acute exacerbations +++ Surgery ++ Resection is reserved for the few patients with localized bronchiectasis and adequate pulmonary function who do not respond to conservative treatment Surgical treatment may be necessary to stop bleeding in some cases of massive hemoptysis +++ Therapeutic Procedures ++ Daily chest physiotherapy with postural drainage and chest percussion Bronchoscopy may be needed to evaluate hemoptysis, remove retained secretions, and rule out obstructing lesions Pulmonary angiography with embolization may be required to control massive hemoptysis + Outcome Download Section PDF Listen +++ +++ Follow-Up ++ Monitor serial pulmonary function tests and sputum cultures If patient is unresponsive to treatment, screen for nontuberculous mycobacteria +++ Complications ++ Hemoptysis Hypoxemia Cor pulmonale Amyloidosis Secondary visceral abscesses at distal sites +++ Prevention ++ Influenza vaccine Pneumococcal vaccine Regular chest physiotherapy +++ Prognosis ++ Depends on cause and severity +++ When to Refer ++ Most cases should be referred to a pulmonary, allergy, clinical immunology, or infectious disease specialist to assist in the evaluation and treatment +++ When to Admit ++ Hypoxemia Moderate to severe acute airflow obstruction Severe infection + References Download Section PDF Listen +++ + +Gruffydd-Jones K et al. Primary care implications of the British Thoracic Society Guidelines for bronchiectasis in adults 2019. NPJ Prim Care Respir Med. 2019 Jun 27;29(1):24. [PubMed: 31249313] + +King CS et al. Critical care of the adult patient with cystic fibrosis. Chest. 2019 Jan;155(1):202–14. [PubMed: 30077689] + +Lesan A et al. Short review on the diagnosis and treatment of bronchiectasis. Med Pharm Rep. 2019 Apr;92(2):111–6. [PubMed: 31086836] + +McShane PJ et al. Bronchiectasis. Chest. 2019 Apr;155(4):825–33. [PubMed: 30403962] + +Singh A et al. Bronchiectasis revisited: imaging-based pattern approach to diagnosis. Curr Probl Diagn Radiol. 2019 Jan;48(1):53–60. [PubMed: 29530453]