Topic Editor: Robert Giles, MBBS, BPharm

Review Date: 9/8/2012

Definition

Bronchiectasis is the permanent abnormal dilation of bronchi with destruction of the muscular and elastic elements of the bronchial wall. It is most often caused by infectious insults in a predisposed individual

Description

• Bronchiectasis is a chronic and progressive condition characterized by airway obstruction, dyspnea, and impaired clearance of respiratory secretions. It is accompanied by a chronic debilitating cough with mucopurulent secretions and sometimes hemoptysis

• Severe cases may progress to respiratory failure
• Bronchiectasis is mainly a focal process affecting a lobe, segment, or sub-segment of the lung. In some cases, diffuse involvement of one or both lungs can occur
• Bronchiectasis can be classified as
  • Cystic fibrosis (CF) bronchiectasis: Primarily caused by cystic fibrosis
  • Non-cystic fibrosis (non-CF) bronchiectasis: Mainly caused by recurrent respiratory infections, immune conditions, genetic disorders and idiopathic causes
• There is evidence of an increasing global prvalence of bronchiectasis, particularly in developing countries
• Pathophysiology: Caused by a vicious cycle of transmural bacterial infection, inflammation and obstruction, followed by increased bacterial colonization which is promoted by damaged airways and impaired cilia

Epidemiology

Incidence/prevalence

• The incidence of bronchiectasis has significantly declined in developed nations due to the emergence of vaccines and antibiotics, but it remains a major concern in the developing world
• The estimated prevalence of bronchiectasis is 4 per 100,000 persons in individuals aged 18 to 34 years, and 272 per 100,000 among those aged 75 years. Based on this data an estimated 110,000 people may be affected in the United States

Age

• Bronchiectasis affects individuals of all ages, though its prevalence increases with age

Gender

• Bronchiectasis more commonly affects females than males
• Females typically have more severe disease than males
• The most common patient with bronchiectasis is an older female

Race

• No racial predilection (other than associated socioeconomic conditions) has been noted

Risk factors

• Alpha 1- antitrypsin deficiency
• Chronic rhinosinusitis
• Congenital bronchial conditions
• Cystic fibrosis
• Airway obstruction (eg foreign body aspiration, cancers, mucoid impaction, postoperative)
• History of severe childhood respiratory infections (bacterial, fungal, viral)
• Immunodeficiency states
• Nontuberculous mycobacterial infection
• Primary ciliary dyskinesia
• Recurrent pneumonia
• Systemic diseases ( eg connective tissue disorders, inflammatory bowel disease, rheumatoid arthritis)
• Post transplantation

Etiology

Bronchiectasis primarily occurs as a result of recurrent pulmonary infection in combination with impaired drainage, airway obstruction and/or a defect in host defense. This leads to chronic inflammation and progressive bronchial damage. Predisposing factors which promote recurrent infections are classified as follows:

• Idiopathic causes (5-50%), particularly in non-CF bronchiectasis
• Genetic factors
  • Alpha-1-antitrypsin deficiency
  • Ciliary dyskinesia
  • Cystic fibrosis
  • Kartagener's syndrome (situs inversus, primary ciliary dyskinesia, sinusitis/bronchiectasis)
• Post-infectious causes
  • History of childhood viral respiratory infections (measles, influenza, pertussis, respiratory syncytial virus)
  • History of bronchopulmonary aspergillosis
  • History of mycobacterial infection or severe bacterial pneumonia
  • Swyer-James-Macleod syndrome
• Aspiration or inhalation injury
  • Gastric aspiration
  • Toxic gas inhalation
• Autoimmune diseases
  • Rheumatoid arthritis
  • Sjogren's syndrome
  • Systemic lupus erythematosus
• Bronchial obstruction
  • Bronchial stenosis
  • Broncholith
  • Foreign body aspiration
  • Lung neoplasms
  • Lymphadenopathy
• Immunodeficiency conditions
  • HIV infection
  • Immunoglobulin deficiency
• Inflammatory bowel conditions
  • Crohn's disease
  • Ulcerative colitis
• Others
  • Ehlers-Danlos syndrome
  • Marfan syndrome
  • Mounier-Kuhn syndrome
  • Post transplantation
  • Pulmonary sequestration
  • Williams-Campbell syndrome
  • Yellow nail syndrome
  • Young's syndrome

History

• Presence of predisposing risk factors (congenital, infectious, and/or exposure-related)
• Chronic cough (98% of patients)
• Voluminous and purulent sputum
• Dyspnea (73% of patients), especially on exertion
• Fatigue, particularly during acute exacerbations
• Hemoptysis (56-92% of patients)
• Intermittent pleuritic chest pain (19-46% of patients )
• Recurrent episodic fever (>50% of patients)
• Urinary incontinence in women

Physical findings on examination

• Bibasilar crackles
• Clubbing of digits (rare)
• Cyanosis and plethora (rare)
• Inspiratory squeaks
• Rhinosinusitis
• Rhonchi
• Weight loss
• Wheeze

• Allergic bronchopulmonary aspergillosis
• Alpha 1-Antitrypsin deficiency
• Aspiration pneumonia
• Asthma
• Bacterial pneumonia
• Chronic bronchitis
• Chronic obstructive pulmonary disease (COPD or "Emphysema")
• Chronic Sinusitis
• Cystic fibrosis
• Gastroesophageal reflux
• Pulmonary tuberculosis

General treatment items

• Antibiotic treatment
  • Antibiotics are the primary treatment for recurrent lung infections associated with bronchiectasis. The most common causes are H. influenza, P. aeruginosa, and more rarely S. pneumoniae
  • Antibiotics may be administered via oral, parenteral or inhalation routes, based on the severity of the condition and causative organism
  • Sputum culture should be obtained and empirical antibiotics commenced while awaiting results. Previous sputum results may help guide therapy
  • Oral broad-spectrum antibiotics are generally preferred for mild to moderate acute exacerbations, guided by sputum results
  • Empiric treatment commonly will be one of the following:
    • Amoxicillin-clavulanate
    • Azithromycin
    • Ciprofloxacin
    • Doxycycline
    • Gemifloxacin
    • Levofloxacin
    • Moxifloxacin
    • Tetracycline
    • Trimethoprim-sulfamethoxazole
  • Once a specific organism is identified, modifying therapy to the most narrow spectrum agent possible is reasonable
  • Treatment duration typically ranges from 7–14 days
  • Moderate to severe cases require parenteral administration of antibiotics such as gentamicin, tobramycin, third-generation cephalosporins, or advanced fluoroquinolones such as ciprofloxacin, levofloxacin, gemifloxacin or moxifloxacin
  • Chronic suppressive therapy is useful in patients with frequent exacerbations. Various regimes have been developed including daily oral/ inhaled doses or short courses of oral / intravenous antibiotics
  • Aerosolized antibiotics may be useful in Pseudomonas aeruginosa infections, with minimal systemic effects. Inhaled tobramycin has been effectively used for treatment of CF or non-CF bronchiectasis. Other inhaled antibiotics such as gentamicin and colistin are currently under investigation
  • Mycobacterium avium complex (MAC) infection should be treated with a specific combination regimen of clarithromycin or azithromycin along with other anti-tubercular agents for a prolonged duration (18–24 months)
• Anti-inflammatory therapy
  • Anti-inflammatory agents may alter the inflammatory response generated by microorganisms associated with bronchiectasis and subsequently limit tissue damage
  • The role of inhaled or oral corticosteroids, leukotriene receptor antagonists, and non-steroidal anti-inflammatory drugs (NSAIDs) is unclear with studies showing mixed results. Opinions regarding the use of these agents are currently mixed
  • Certain oral macrolides like azithromycin have demonstrated anti-inflammatory properties and are known to improve lung function in patients with P. aeruginosa colonization
• Bronchodilator therapy
  • Bronchodilators, including beta-agonists (albuterol) and anticholinergics (ipratropium) may be useful especially in patients with underlying asthma or chronic obstructive pulmonary disease. Randomized trial data to support their effectiveness is lacking
• Surgery
  • Patients with advanced or complicated disease, particularly those with focal disease, intractable hemoptysis, or poor response to antibiotics require surgical resection
  • Surgical interventions may also be useful for reducing acute infective episodes
  • Surgery may be considered to decrease sputum production, control severe hemoptysis, remove foreign bodies or neoplasm, or in the treatment of bronchopulmonary aspergillosis
• Lung transplantation
  • Severe bronchiectasis, especially cystic fibrosis-related, may necessitate single or double lung transplantation
  • Decline of FEV1 below 30% of the predicted value may prompt transplant consideration

Medication indicated with specific doses

Antibiotics

• Amoxicillin-Clavulanate
  • Adult Dosing
  • Pediatric Dosing
• Azithromycin [Oral]
  • Adult Dosing
  • Pediatric Dosing
• Azithromycin [IV]
  • Adult Dosing
  • Pediatric Dosing
• Ciprofloxacin (oral)
  • Adult Dosing
  • Pediatric Dosing
• Ciprofloxacin (IV)
  • Adult Dosing
  • Pediatric Dosing
• Clarithromycin
  • Adult Dosing
  • Peiatric dosing
• Doxycycline (oral)
  • Adult Dosing
  • Pediatric Dosing
• Gemifloxacin (oral)
  • Adult Dosing
  • Pediatric Dosing
• Gentamicin [IM/IV]
  • Adult Dosing
  • Pediatric Dosing
• Levofloxacin (oral)
  • Adult Dosing
  • Pediatric Dosing
• Levofloxacin (IV)
  • Adult Dosing
  • Pediatric Dosing
• Moxifloxacin [IV]
  • Adult Dosing
  • Pediatric Dosing
• Moxifloxacin [Oral]
  • Adult Dosing
  • Pediatric Dosing
• Sulfamethoxazole/trimethoprim [Oral]
  • Adult Dosing
  • Pediatric Dosing
• Tetracycline [Oral]
  • Adult Dosing
  • Pediatric Dosing
• Tobramycin [inhaled]
  • Adult dosing
  • Pediatric Dosing
• Tobramycin [IM/IV]
  • Adult Dosing
  • Pediatric Dosing

Inhaled corticosteroids

• Beclomethasone [Inhaled]
  • Adult Dosing
• Fluticasone [Inhaled]
  • Adult Dosing

Bronchodialators

• Albuterol [Inhaled]
  • Adult Dosing
• Ipratropium [Inhaled]
  • Adult Dosing

Dietary or Activity restrictions

• A healthy, well-balanced and regular diet is recommended for maintaining a body mass index (BMI) >18.5
• Patients should be advised to adhere to a regular exercise program
• Patients should be encouraged to perform airway clearance therapy on a daily basis

Monitoring

• Subsequent monitoring may be performed using chest CT or CXR
• Regular microbiological sputum analysis is recommended for assessment of changes in antibiotic sensitivities of organisms
• Patients should undergo spirometric evaluation regularly for assessment of disease progression or exacerbations

Complications

• Amyloidosis (rare)
• Bacterial superinfection
• Chronic bronchial infection
• Cor pulmonale
• Empyema
• Lung abscess
• Metastatic abscesses (rare)
• Pneumothorax
• Recurrent pneumonia and degenerating lung function
• Respiratory failure
• Severe hemoptysis

Prevention

• Early diagnosis and prompt treatment of underlying etiology may retard the development of bronchiectasis
• Cessation of smoking and avoidance of passive smoke is recommended
• High-risk patients should receive vaccination against Haemophilus influenzae and Streptococcus pneumoniae

Prognosis

• Prognosis of bronchiectasis is difficult to estimate, due to its coexistence with other respiratory conditions
• Bronchiectasis had a reported mortality rate of 13% in 1981. The current mortality rate is difficult to estimate due to lack of studies
• Predictors of mortality include hypoxemia, hypercapnia, intensity of dyspnea, and radiological level of condition
• Chronic P. aeruginosa colonization, severe exacerbations, and systemic inflammation are indicators of poor prognosis
• A higher BMI and vaccination are associated with improved survival

Associated conditions

• Cystic fibrosis

Pregnancy/pediatric affects on the condition

• Bronchiectasis is relatively unusual during pregnancy; its presence may aggravate the patient's condition
• Bronchiectasis during pregnancy may be associated with intra-uterine growth retardation of the fetus, primarily due to maternal hypoxia

ICD-9-CM

• 494 Bronchiectasis

ICD-10-CM

• J47 Bronchiectasis

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