Bronchiectasis

Description of Medical Condition

Chronic irreversible, abnormal dilatation of the bronchi, usually accompanied by infection and productive cough System(s) affected: Pulmonary Genetics: Associated with many conditions including some that are congenital or hereditary Incidence/Prevalence in USA:

No reliable figures available
Less common than it once was, probably due to more effective treatment of childhood respiratory infections

Predominant age: Begins most often in early childhood, but symptoms may not appear until later in life Predominant sex: Male = Female

Medical Symptoms and Signs of Disease

Cough
Sputum — copious and purulent
Hemoptysis
Wheezing
Coarse or moist crackles
Cyanosis
Digital clubbing
Dyspnea
Barrel chest
Emaciation
Fatigue
Fever
Recurrent pneumonia
Tachycardia
Tachypnea

What Causes Disease?

Alpha-1 -antitrypsin deficiency
Allergic bronchopulmonaty aspergillosis
Bronchial obstruction
Cystic fibrosis
Dyskinetic cilia syndromes
Hypogammaglobulinemia
Inhaling noxious chemicals
Kartagener syndrome (situs inversus, sinusitis, immotile spermatozoa, bronchiectasis)
Necrotizing pulmonary infections
Pulmonary abscess
Severe lung infection in childhood (measles, adenovi-rus, influenza, pertussis, or bronchiolitis)
Tuberculosis
Congenital immunodeficiency syndromes
Chronic aspiration
Rheumatic diseases
Transplant graft rejection

Risk Factors

Repeated bouts of pneumonia
Any chronic respiratory illness
Retained foreign body
Immunodeficiency
Gastroesophageal reflux
Sinusitis

Diagnosis of Disease

Differential Diagnosis

Chronic bronchitis
Chronic obstructive pulmonary disease
Cystic fibrosis
Pulmonary tuberculosis
Allergic bronchopulmonary aspergillosis

Laboratory

Positive sputum culture (yields H. influenzae, Streptococcus pneumoniae, staphylococcal, klebsiella, pseudomonas, or anaerobes)
Hypoxemia
Leukocytosis, usually
Serum immunoglobulins — check for hypogammaglobulinemia, IgE level helpful

Drugs that may alter lab results: N/A Disorders that may alter lab results: N/A

Pathological Findings

Bronchial dilation
Inflamed bronchi
Purulent bronchorrhea
Necrosis of bronchial mucosa
Peribronchial scarring

Special Tests

Sweat test
Skin test for aspergillus
Bronchoscopy useful in locating bleeding site and to exclude adenoma or foreign body
Ciliary biopsy with electron microscopy (EM)
Pulmonary function tests show variable obstruction and restriction; include test for reversible component
Sputum culture/sensitivity, AFB, fungus

Imaging

CT scan
- Shows dilation and truncation of airways with signet rings prominent in lower lobes
- High resolution CT is best to establish diagnosis and extent of disease
- Spiral CT helpful for questionable findings
Bronchography has limited role
Chest x-ray
- Can be normal
- Coarse lung markings — honeycomb/tram tracks
- Air-fluid level
- Cystic lesions
- Atelectasis

Diagnostic Procedures

Fiberoptic bronchoscopy

Recommended when disease is of recent onset or is unilateral
May be combined with bronchography
Obtain culture

Treatment (Medical Therapy)

Appropriate Health Care

Outpatient except for possible surgery

General Measures

Airway clearance techniques
- Chest physical therapy
- Percussion (mechanical)
- Postural drainage
- Hyd ration
Onebulized saline
- Pulmonary rehabilitation with inspiratory muscle training
- Noninvasive positive pressure ventilation, nocturnal or chronic
Bronchoscopy may be required for extraction of mucus or mycelial plugs, or if physiotherapy has failed
Pulmonary rehabilitation to improve functional status
Bronchial artery embolization may be lifesaving for massive pulmonary hemorrhage
Avoid cigarette smoking
Treat acid reflux if needed

Surgical Measures

Segmental pulmonary resection for localized disease or refractory hemoptysis

Activity

As fully active as possible

Diet

No restrictions

Patient Education

Printed patient information available from: American Lung Association, 1740 Broadway, New York, NY 10019, (212)315-8700

Medications (Drugs, Medicines)

Drug(s) of Choice

Bronchodilators
- Use chronically to enhance sputum clearance; particularly beneficial with asthma or aspergillosis
- Beta-adrenergic agonists (e.g., albuterol) given by metered dose inhaler with use of a spacer (reservoir device)
Inhaled corticosteroids
- Use chronically to enhance sputum clearance
- Beclomethasone, fluticasone, etc., given by metered dose inhaler
Antibiotics. Use early for exacerbations. Continue minimum of 7-10 days. May use culture to guide choice. Chronic prophylaxis not recommended.
- Ciprofloxacin, levofloxacin
- Ampicillin: 250-500 mg orally q 6 hours (50 mg/kg/day in divided doses q 6-8 hours in children less than 20 kg) Trimethoprim-sulfamethoxazole: DSq12h
- Tetracycline: 250-500 mg orally q 6 hours
Onebulized aminoglycosides — tobramycin 300 mg by aerosol bid
Steroids
- Consider for patients with bronchopulmonary aspergillosis. IgE level guides steroid dosing.

Contraindications:

Tetracycline: not for use in pregnancy or children < 8 years.

Precautions:

Tetracycline: may cause photosensitivity; sunscreen recommended.

Significant possible interactions:

Tetracycline: avoid concurrent administration with antacids, dairy products, or iron.
Broad-spectrum antibiotics: may reduce the effectiveness of oral contraceptives; barrier method recommended.

Alternative Drugs

For chronic persistent infection, long-term high dose of amoxicillin 3 g every 12 hours may be useful. It does not provide relief for everyone and has more side effects.
Other broad-spectrum antimicrobials including anti-pseudomonals if required. Choice would depend on pathogen and susceptibility.
Inhaled corticosteroids if reversible obstruction present
Oxygen — if P02 < 60 mm Hg
Nicotine replacement, consider to aid smoking cessation

Patient Monitoring

Frequent followupfor progress of illness, prevention of infection, smoking cessation, and to check on physiotherapy
Pulmonary function and C-reactive protein may be monitored to assess inflammation and disease progression
At some point in followup, need to discuss with patient the possibility of mechanical ventilation and cardiopulmonary resuscitation in the future. The patient, family and provider should determine if this type of treatment is appropriate.

Prevention / Avoidance

Treat all pneumonias adequately
Immunizations for viral illnesses (e.g., influenza)
Immunization for pneumococcal pneumonia
Routine childhood immunizations (e.g., pertussis, measles, Hib)
Genetic counseling if inherited etiology

Possible Complications

Recurrent pulmonary infections
Pulmonary hypertension
Secondary amyloidosis
Cor pulmonale
Brain abscess
Massive hemoptysis
Atelectasis
Lung abscess

Expected Course / Prognosis

Chronic. Surgery may be curative if disease localized.
Average life expectancy — 55 years
10% of patients have a rapidly progressive course

Miscellaneous

Associated Conditions

Sinusitis
Cor pulmonale
Kartagener syndrome
Cystic fibrosis
Ulcerative colitis
Marian syndrome
Ehlers-Danlos syndrome
Yellow nail syndrome

Age-Related Factors

Pediatric: Cystic fibrosis and other congenital disorders Geriatric: Elderly more likely to need hospitalization for treatment

Pregnancy

N/A

International Classification of Diseases

494.0 Bronchiectasis without acute exacerbation 494.1 Bronchiectasis with acute exacerbation

Other Notes

Conditions that may lead to bronchiectasis include severe pneumonia (especially measles, pertussis, adenoviral infections in children), necrotizing infections due to Klebsiella, staphylococci, influenza virus, fungi, mycobacteria, mycoplasma, bronchial obstruction from any cause (foreign body, carcinoma, enlarged mediastinal lymph nodes.