> Table of Contents > Bronchiectasis
Bronchiectasis
Sumera R. Ahmad, MD
Scott E. Kopec, MD
image BASICS
DESCRIPTION
  • Bronchiectasis is an irreversible dilatation of ≥ 1 airways accompanied by recurrent transmural bronchial infection/inflammation and chronic mucopurulent sputum production.
  • Generally classified into cystic fibrosis (CF) and noncystic fibrosis (non-CF) bronchiectasis
EPIDEMIOLOGY
  • Predominant age: most commonly presents in 6th decade of life
  • Predominant sex: female > male
Incidence
Incidence has decreased in the United States for two reasons:
  • Widespread childhood vaccination against pertussis
  • Effective treatment of childhood respiratory infections with antibiotics
Prevalence
  • Prevalence in adult U.S. population estimated to be > 110,000 affected individuals.
  • Internationally, prevalence increases with age from 4.2/100,000 persons aged 18 to 34 years to 271.8/100,000 among those aged 75 years and older (1).
ETIOLOGY AND PATHOPHYSIOLOGY
  • CF bronchiectasis: bronchiectasis due to CF
  • Non-CF bronchiectasis
    • Most cases are idiopathic.
    • Most commonly associated with non-CF bronchiectasis is childhood infection.
  • Vicious circle hypothesis: Transmural infection, generally by bacterial organisms, causes inflammation and obstruction of airways. Damaged airways and dysfunctional cilia foster bacterial colonization, which leads to further inflammation and obstruction.
RISK FACTORS
  • Nontuberculous mycobacterial infection is both a cause and a complication of non-CF bronchiectasis.
  • Severe respiratory infection in childhood (measles, adenovirus, influenza, pertussis, or bronchiolitis)
  • Systemic diseases (e.g., rheumatoid arthritis and inflammatory bowel disease)
  • Chronic rhinosinusitis
  • Recurrent pneumonia
  • Aspirated foreign body
  • Immunodeficiency
  • Congenital abnormalities
GENERAL PREVENTION
  • Routine immunizations against pertussis, measles, Haemophilus influenza type B, influenza, and pneumococcal pneumonia
  • Genetic counseling if congenital condition is etiology
  • Smoking cessation
COMMONLY ASSOCIATED CONDITIONS
  • Mucociliary clearance defects
    • Primary ciliary dyskinesia
    • Young syndrome (secondary ciliary dyskinesia)
    • Kartagener syndrome
  • Other congenital conditions
    • α1-antitrypsin deficiency
    • Marfan syndrome
    • Cartilage deficiency (Williams-Campbell syndrome)
  • Chronic obstructive pulmonary disease
  • Pulmonary fibrosis, causing traction bronchiectasis
  • Postinfectious conditions
    • Bacteria (H. influenzae and Pseudomonas aeruginosa)
    • Mycobacterial infections (tuberculosis [TB] and Mycobacterium avium complex [MAC])
    • Whooping cough
    • Aspergillus species
    • Viral (HIV, adenovirus, measles, influenza virus)
  • Immunodeficient conditions
    • Primary: hypogammaglobulinemia
    • Secondary: allergic bronchopulmonary aspergillosis (ABPA), posttransplantation
    • Sequelae of toxic inhalation or aspiration (e.g., chlorine, luminal foreign body)
  • Rheumatic/chronic inflammatory conditions
    • Rheumatoid arthritis
    • Sjögren syndrome
    • Systemic lupus erythematosus
    • Inflammatory bowel disease
  • Miscellaneous
    • Yellow nail syndrome
image DIAGNOSIS
  • Typical symptoms include chronic productive cough, wheezing, and dyspnea.
  • Symptoms are often accompanied by repeated respiratory infections (2).
  • Once diagnosed, investigate etiology.
PHYSICAL EXAM
Symptoms are commonly present for many years and include the following:
  • Chronic cough (90%)
  • Sputum: may be copious and purulent (90%)
  • Rhinosinusitis (60-70%)
  • Fatigue: may be a dominant symptom (70%)
  • Dyspnea (75%)
  • Chest pain: may be pleuritic (20-30%)
  • Hemoptysis (20-30%)
  • Wheezing (20%)
  • Bibasilar crackles (60%)
  • Rhonchi (44%)
  • Digital clubbing (3%)
DIFFERENTIAL DIAGNOSIS
  • CF
  • Chronic obstructive pulmonary disease
  • Asthma
  • Chronic bronchitis
  • Pulmonary TB
  • ABPA
DIAGNOSTIC TESTS & INTERPRETATION
  • Spirometry
    • Moderate airflow obstruction and hyperresponsive airways
    • Forced expiratory volume in the 1st second of expiration (FEV1): <80% predicted and FEV1/FVC <0.7
  • Special tests
    • Ciliary biopsy by electron microscopy
  • Sputum culture
    • H. influenzae, nontypeable form (42%)
    • P. aeruginosa (18%)
    • Cultures may also be positive for Streptococcus pneumoniae, Moraxella catarrhalis, MAC, and Aspergillus.
    • Of all isolates, 30-40% will show no growth.
  • Special tests
    • Sweat test for CF
    • Purified protein derivative (PPD) test for TB
    • Skin test for Aspergillus
    • HIV
    • Serum immunoglobulins to test for humoral immunodeficiency
    • Protein electrophoresis to test for α1-antitrypsin deficiency
    • Barium swallow to look for abnormalities of deglutition, achalasia, esophageal hypomotility
    • pH probe to characterize reflux
    • Screening tests for rheumatologic diseases
  • Chest radiograph
    • Nonspecific; increased lung markings or may appear normal
  • Chest computed tomography (CT)
    • Noncontrast high-resolution chest CT is the most important diagnostic tool.
    • Bronchi are dilated and do not taper, resulting in “tram track sign”; parallel opacities seen on scan.
    • Varicose constrictions and balloon cysts may be seen.
    • For focal bronchiectasis, rule out endobronchial obstruction.
    • For exclusively upper lobe bronchiectasis, consider CF and ABPA.
Diagnostic Procedures/Other
  • Bronchoscopy may be used to obtain cultures and evacuate sputum.
  • Bronchoscopy for hemoptysis
  • Bronchoscopy may be useful to rule out airway-obstructing lesions with focal bronchiectasis.
Test Interpretation
Bronchoscopy findings include the following:
  • Dilatation of airways and purulent secretions
  • Thickened bronchial walls with necrosis of bronchial mucosa
  • Peribronchial scarring
image TREATMENT
  • Treat underlying conditions.
  • Recognize an acute exacerbation with 4 out of 9 criteria (3)
    • Change in sputum production
    • Increased dyspnea
    • Increased cough
    • Fever
    • Increased wheezing
    • Malaise, fatigue, lethargy
    • Reduced pulmonary function
    • Radiographic changes
    • Changes in chest sounds
  • Non-CF bronchiectasis: determine cause of exacerbations; promote good bronchopulmonary hygiene via daily airway clearance.
  • Consider surgical resection of damaged lung for focal disease that is refractory to medical management.
  • Medical management: Reduce morbidity by control-ling symptoms and preventing disease progression.
  • Patients with non-CF bronchiectasis may not respond to CF treatment regimens in the same way as patients with CF do.
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GENERAL MEASURES
  • Maintain hydration (nebulized hypertonic saline, 3% or 7% may be used to help increase mucus clearance) (4)[A].
  • Noninvasive positive-pressure ventilation
MEDICATION
  • Insufficient evidence exists to support efficacy of short-course antibiotics in adults and children with bronchiectasis (5)[A].
  • Frequent exacerbations may be treated with prolonged and aerosolized antibiotics (2)[A].
  • Role of mucolytics, antiinflammatory agents, and bronchodilators is still unclear (2)[A].
First Line
  • Antibiotics
    • Potentially useful in acute exacerbations
    • Chronic therapy decreases sputum volume and purulence, but it does not diminish the frequency of exacerbations (6)[A].
    • Patients may require twice the usual dose and longer treatment for 14 days (7)[C].
    • Sputum culture and sensitivity should direct therapy; antibiotic selection is complicated by a wide range of pathogens and resistant organisms.
    • Should be administered IV in cases of severe infection
      • Augmentin: 500 mg PO q8-12h. Pediatric: base dosing on amoxicillin content
      • Trimethoprim (TMP)/sulfamethoxazole (SMX) : 160 mg TMP/800 mg SMX PO q12h. Pediatric: ≥2 months, 8 mg/kg TMP and 40 mg/kg SMX PO/24 hours, administered in two divided doses q12h
      • Doxycycline and cefaclor given PO are also effective.
      • Nebulized aminoglycosides (tobramycin): 300 mg by aerosol BID (8)[B]
      • Ciprofloxacin: 750 mg PO q12h for adults for susceptible strain of Pseudomonas
      • Macrolides: appear to have immunomodulatory benefits
  • Chronic use of azithromycin as an oral macrolide for 6 to 12 months in non-CF bronchiectasis has been shown to reduce exacerbations (9,10). Needs caution with respect to cardiovascular deaths, where it is a QTc-prolonging medication (9).
  • Bronchodilators
    • Chronic use of β2-agonists (e.g., albuterol) reverses airflow obstruction.
  • Inhaled corticosteroids
    • Insufficient evidence exists to recommend use of inhaled steroids with stable bronchiectasis (11)[A].
    • A therapeutic trial of inhaled steroids may be justified in adults with difficult-to-control symptoms (11)[A].
    • Decrease sputum and tend to improve lung function
      • Fluticasone propionate: 110 to 220 µg inhaled BID
    • Potential synergistic effect of long-acting β2-agonists with inhaled corticosteroids, allowing for lower steroid dose (12):
      • Budesonide 160 µg/Formoterol 4.5 µg 2 puffs inhaled BID
Second Line
Other broad-spectrum antimicrobials, including those with antipseudomonal coverage
ADDITIONAL THERAPIES
Sputum clearance techniques, including physiotherapy (percussion and postural drainage) and pulmonary rehabilitation (improves exercise tolerance)
SURGERY/OTHER PROCEDURES
  • Surgery if area of bronchiectasis is localized and symptoms remain intolerable despite medical therapy or if disease is life-threatening (2)[A]
  • Surgery effectively improves symptoms in 80% of these cases.
INPATIENT CONSIDERATIONS
Admission Criteria/Initial Stabilization
Bronchiectasis can present as a life-threatening massive hemoptysis. In this situation, in addition to airway protection and resuscitation, bronchial artery embolization or surgical intervention is necessary to control bleeding.
image ONGOING CARE
Long-term outpatient treatment recommendations for bronchiectasis in children (13)[B]:
  • Children with CF and non-CF-related bronchiectasis should be treated by comprehensive interdisciplinary chronic disease management programs.
  • Pathogen-directed aerosolized tobramycin treatment should be used long-term on a regular basis to improve the course of CF-related bronchiectasis.
  • PO macrolide antibiotic use long term (up to 6 months) improves lung function among children with CF-related bronchiectasis.
  • Long-term antibiotic use (PO or aerosolized) in children with non-CF-related bronchiectasis has not been studied enough to warrant routine use.
  • Hypertonic saline administered by inhalation used long-term (48 weeks) improves lung function and is safer when used with pretreatment bronchodilator therapy among children who have CF.
  • Nebulized dornase improves multiple pulmonary outcomes of children who have CF and is indicated for long-term use.
  • Risks for long-term oral corticosteroid use outweigh pulmonary benefits in the treatment of CF-related bronchiectasis.
  • High-dose ibuprofen therapy reduces the rate of decline among children with mild CF-related bronchiectasis and is indicated for long-term use.
  • Mucolytic agents, airway-hydrating treatments, antiinflammatory therapy, chest physical therapy (CPT), and bronchodilator therapy have not been studied sufficiently long term in children with non-CF-related bronchiectasis to merit their routine use.
FOLLOW-UP RECOMMENDATIONS
Regular exercise is recommended.
Patient Monitoring
  • Serial spirometry, every 2 to 5 years, to monitor the course of the disease
  • Chest CTs to monitor progression of disease may be indicated with some conditions such as bronchiectasis with MAC infections.
  • Routine microbiological sputum analysis
PATIENT EDUCATION
http://www.lungusa.org/
PROGNOSIS
  • Mortality rate (death due directly to bronchiectasis) is 13%.
  • Pseudomonas infection is associated with poorer prognosis.
REFERENCES
1. Pappalettera M, Aliberti S, Castellotti P, et al. Bronchiectasis: an update. Clin Respir J. 2009;3(3):126-134.
2. ten Hacken NH, Wijkstra PJ, Kerstjens HA. Treatment of bronchiectasis in adults. BMJ. 2007;335(7629):1089-1093.
3. O'Donnell AE, Barker AF, Ilowite JS, et al. Treatment of idiopathic bronchiectasis with aerosolized recombinant human DNase I. rhDNase Study Group. Chest. 1998;113(5):1329-1334.
4. Kellett F, Robert NM. Nebulised 7% hypertonic saline improves lung function and quality of life in bronchiectasis. Respir Med. 2011;105(12):1831-1835.
5. Wurzel D, Marchant JM, Yerkovich ST, et al. Short courses of antibiotics for children and adults with bronchiectasis. Cochrane Database Syst Rev. 2011;(6):CD008695.
6. Evans DJ, Bara AI, Greenstone M. Prolonged antibiotics for purulent bronchiectasis in children and adults. Cochrane Database Syst Rev. 2007;(2):CD001392.
7. Pasteur MC, Bilton D, Hill AT. British Thoracic Society guideline for non-CF bronchiectasis. Thorax. 2010;65(Suppl 1):i1-i58.
8. Lobue PA. Inhaled tobramycin: not just for cystic fibrosis anymore? Chest. 2005;127(4):1098-1101.
9. Wong C, Jayaram L, Karalus N, et al. Azithromycin for prevention of exacerbations in non-cystic fibrosis bronchiectasis (EMBRACE): a randomised, double-blind, placebo-controlled trial. Lancet. 2012;380(9842):660-667.
10. Altenburg J, de Graaff CS, Stienstra Y, et al. Effect of azithromycin maintenance treatment on infectious exacerbations among patient with non-cystic fibrosis bronchiectasis: the BAT randomized controlled trial. JAMA. 2013;309(12):1251-1259.
11. Kapur N, Bell S, Kolbe J, et al. Inhaled steroids for bronchiectasis. Cochrane Database Syst Rev. 2009;(1):CD000996.
12. Martínez-García MÁ, Soler-Cataluña JJ, Catalán-Serra P, et al. Clinical efficacy and safety of budesonide-formoterol in non-cystic fibrosis bronchiectasis. Chest. 2012;141(2):461-468.
13. Redding GJ. Bronchiectasis in children. Pediatr Clin North Am. 2009;56(1):157-171.
Codes
ICD10
  • J47.9 Bronchiectasis, uncomplicated
  • J47.1 Bronchiectasis with (acute) exacerbation
  • J47.0 Bronchiectasis with acute lower respiratory infection
Clinical Pearls
  • Symptoms of bronchiectasis include chronic productive cough, wheezing, and dyspnea, often accompanied by repeated respiratory infections.
  • A chest x-ray has poor sensitivity and specificity for the diagnosis; a noncontrast high-resolution chest CT is the most important diagnostic tool.
  • Current practice guidelines recommend treating acute exacerbations with a 14-day course of antibiotics. Frequent exacerbations may be treated with prolonged and aerosolized antibiotics.