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Bronchiectasis

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Bronchiectasis

BASICS

DESCRIPTION

Incidence has decreased in the United States for two reasons:

Symptoms are commonly present for many years and include the following:

Other broad-spectrum antimicrobials, including those with antipseudomonal coverage

Sputum clearance techniques, including physiotherapy (percussion and postural drainage) and pulmonary rehabilitation (improves exercise tolerance)

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.

http://www.lungusa.org/

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.

  • 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
  • Widespread childhood vaccination against pertussis
  • Effective treatment of childhood respiratory infections with antibiotics
  • 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 bronchiectasisMost 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.
  • Most cases are idiopathic.
  • Most commonly associated with non-CF bronchiectasis is childhood infection.

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 defectsPrimary ciliary dyskinesiaYoung syndrome (secondary ciliary dyskinesia)Kartagener syndrome
  • Other congenital conditionsĪ±1-antitrypsin deficiencyMarfan syndromeCartilage deficiency (Williams-Campbell syndrome)
  • Chronic obstructive pulmonary disease
  • Pulmonary fibrosis, causing traction bronchiectasis
  • Postinfectious conditionsBacteria (H. influenzae and Pseudomonas aeruginosa)Mycobacterial infections (tuberculosis [TB] and Mycobacterium avium complex [MAC])Whooping coughAspergillus speciesViral (HIV, adenovirus, measles, influenza virus)
  • Immunodeficient conditionsPrimary: hypogammaglobulinemiaSecondary: allergic bronchopulmonary aspergillosis (ABPA), posttransplantationSequelae of toxic inhalation or aspiration (e.g., chlorine, luminal foreign body)
  • Rheumatic/chronic inflammatory conditionsRheumatoid arthritisSj ¶gren syndromeSystemic lupus erythematosusInflammatory bowel disease
  • MiscellaneousYellow nail syndrome
  • Primary ciliary dyskinesia
  • Young syndrome (secondary ciliary dyskinesia)
  • Kartagener syndrome
  • Ī±1-antitrypsin deficiency
  • Marfan syndrome
  • Cartilage deficiency (Williams-Campbell syndrome)
  • 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)
  • Primary: hypogammaglobulinemia
  • Secondary: allergic bronchopulmonary aspergillosis (ABPA), posttransplantation
  • Sequelae of toxic inhalation or aspiration (e.g., chlorine, luminal foreign body)
  • Rheumatoid arthritis
  • Sj ¶gren syndrome
  • Systemic lupus erythematosus
  • Inflammatory bowel disease
  • 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

  • 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

  • SpirometryModerate airflow obstruction and hyperresponsive airwaysForced expiratory volume in the 1st second of expiration (FEV1): <80% predicted and FEV1/FVC <0.7
  • Special testsCiliary biopsy by electron microscopy
  • Sputum cultureH. 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 testsSweat test for CFPurified protein derivative (PPD) test for TBSkin test for AspergillusHIVSerum immunoglobulins to test for humoral immunodeficiencyProtein electrophoresis to test for Ī±1-antitrypsin deficiencyBarium swallow to look for abnormalities of deglutition, achalasia, esophageal hypomotilitypH probe to characterize refluxScreening tests for rheumatologic diseases
  • Chest radiographNonspecific; 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.
  • Moderate airflow obstruction and hyperresponsive airways
  • Forced expiratory volume in the 1st second of expiration (FEV1): <80% predicted and FEV1/FVC <0.7
  • Ciliary biopsy by electron microscopy
  • 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.
  • 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
  • Nonspecific; increased lung markings or may appear normal
  • 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.
  • 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.
  • 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 productionIncreased dyspneaIncreased coughFeverIncreased wheezingMalaise, fatigue, lethargyReduced pulmonary functionRadiographic changesChanges 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.
  • Change in sputum production
  • Increased dyspnea
  • Increased cough
  • Fever
  • Increased wheezing
  • Malaise, fatigue, lethargy
  • Reduced pulmonary function
  • Radiographic changes
  • Changes in chest sounds

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].
  • AntibioticsPotentially useful in acute exacerbationsChronic 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 infectionAugmentin: 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 q12hDoxycycline 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 PseudomonasMacrolides: 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).
  • BronchodilatorsChronic use of β2-agonists (e.g., albuterol) reverses airflow obstruction.
  • Inhaled corticosteroidsInsufficient 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 functionFluticasone propionate: 110 to 220 Ī¼g inhaled BIDPotential 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
  • 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 infectionAugmentin: 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 q12hDoxycycline 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 PseudomonasMacrolides: appear to have immunomodulatory benefits
  • 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 β2-agonists (e.g., albuterol) reverses airflow obstruction.
  • 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 functionFluticasone 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
  • Fluticasone propionate: 110 to 220 Ī¼g inhaled BID
  • Budesonide 160 Ī¼g/Formoterol 4.5 Ī¼g 2 puffs inhaled BID

ADDITIONAL THERAPIES

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

ONGOING CARE

  • 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

  • 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

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

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.