Bronchiectasis

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
- Ī±1-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
- Sj ¶gren syndrome
- Systemic lupus erythematosus
- Inflammatory bowel disease
Miscellaneous
- Yellow nail syndrome

DIAGNOSIS

Typical symptoms include chronic productive cough, wheezing, and dyspnea.
Symptoms are often accompanied by repeated respiratory infections (2).
Once diagnosed, investigate etiology.

HISTORY

Any predisposing factors (congenital, infectious, and/or exposure-related)
Immunization history

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 Ī±1-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

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 controlling 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.

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 Ī¼g inhaled BID
- Potential synergistic effect of long-acting β2-agonists with inhaled corticosteroids, allowing for lower steroid dose (12):
  - Budesonide 160 Ī¼g/Formoterol 4.5 Ī¼g 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 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.

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.

COMPLICATIONS

Hemoptysis
Recurrent pulmonary infections
Pulmonary hypertension
Cor pulmonale
Lung abscess

REFERENCES

11 Pappalettera M, Aliberti S, Castellotti P, et al. Bronchiectasis: an update. Clin Respir J. 2009;3(3):126-134.22 ten Hacken NH, Wijkstra PJ, Kerstjens HA. Treatment of bronchiectasis in adults. BMJ. 2007;335(7629):1089-1093.33 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):1329-1334.44 Kellett F, Robert NM. Nebulised 7% hypertonic saline improves lung function and quality of life in bronchiectasis. Respir Med. 2011;105(12):1831-1835.55 Wurzel D, Marchant JM, Yerkovich ST, et al. Short courses of antibiotics for children and adults with bronchiectasis. Cochrane Database Syst Rev. 2011;(6):CD008695.66 Evans DJ, Bara AI, Greenstone M. Prolonged antibiotics for purulent bronchiectasis in children and adults. Cochrane Database Syst Rev. 2007;(2):CD001392.77 Pasteur MC, Bilton D, Hill AT. British Thoracic Society guideline for non-CF bronchiectasis. Thorax. 2010;65(Suppl 1):i1-i58.88 Lobue PA. Inhaled tobramycin: not just for cystic fibrosis anymore? Chest. 2005;127(4):1098-1101.99 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):660-667.1010 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):1251-1259.1111 Kapur N, Bell S, Kolbe J, et al. Inhaled steroids for bronchiectasis. Cochrane Database Syst Rev. 2009;(1):CD000996.1212 Mart nez-Garc a M , Soler-Catalu ±a JJ, Catal ”n-Serra P, et al. Clinical efficacy and safety of budesonide-formoterol in non-cystic fibrosis bronchiectasis. Chest. 2012;141(2):461-468.1313 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
Q33.4 Congenital bronchiectasis

ICD9

494.0 Bronchiectasis without acute exacerbation
494.1 Bronchiectasis with acute exacerbation
748.61 Congenital bronchiectasis

SNOMED

12295008 Bronchiectasis (disorder)
445378003 Acute exacerbation of bronchiectasis
233627004 Congenital cystic bronchiectasis
233629001 Idiopathic bronchiectasis (disorder)

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.

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