Bronchiectasis

Essentials of Diagnosis

• Chronic productive cough with dyspnea and wheezing

• Radiographic findings of dilated, thickened airways and scattered, irregular opacities

General Considerations

• A congenital or acquired disorder of large bronchi characterized by abnormal dilation and destruction of bronchial walls

• May be localized or diffuse

• May be caused by recurrent inflammation or infection

• Cystic fibrosis causes 50% of all cases

• Causes

  • Cystic fibrosis

  • Infection

    • Tuberculosis

    • Fungal

    • Abscess

    • Pneumonia

  • Abnormal lung defense mechanisms

    • Hypogammaglobulinemia

    • Common variable immunodeficiency

    • Selective IgA, IgM, and IgG subclass deficiency

    • Acquired immunodeficiency from cytotoxic drugs, AIDS, lymphoma, leukemia, plasma cell myeloma, chronic kidney disease, chronic liver disease

    • Alpha-1-antiprotease deficiency with cigarette smoking

    • Mucociliary clearance disorders (eg, immotile cilia syndrome)

    • Rheumatic disease (eg, rheumatoid arthritis)

  • Localized airway obstruction

Symptoms and Signs

• Chronic cough with production of copious, purulent sputum

• Recurrent pneumonia

• Hemoptysis

• Pleuritic chest pain

• Dyspnea and wheezing

• Weight loss and anemia common

• Persistent basilar crackles commonly found on examination

• Clubbing

  • Infrequent in mild cases

  • Present in severe disease

• Obstructive pulmonary dysfunction with hypoxemia seen in moderate or severe disease

• Haemophilus influenzae is the most common organism recovered from noncystic fibrosis patients with bronchiectasis

• Pseudomonas aeruginosa, Streptococcus pneumoniae, and Staphylococcus aureus are commonly identified; nontuberculous mycobacteria are seen less commonly

Differential Diagnosis

• Chronic obstructive pulmonary disease

• Asthma

• Bronchiolitis

• Allergic bronchopulmonary mycosis

Laboratory Tests

• Sputum smear and culture for bacterial, mycobacterial, and fungal organisms

• Quantitative pilocarpine iontophoresis sweat test for sodium and chloride levels

• Quantitative immunoglobulins

• Alpha-1-antiprotease level

• Excluding patients with humoral immunodeficiencies, most patients have panhypergammaglobulinemia, reflecting an immune response to chronic airway infection

Imaging Studies

• Chest radiographs

  • Show dilated, thickened bronchi that may appear as "tram-tracks" or as ring-like markings

  • Scattered irregular opacities, atelectasis, and focal consolidation may be present

• High-resolution CT scanning is the diagnostic test of choice

Medications

• Antibiotics should be used in acute exacerbations

• Empiric therapy for 10–14 days with

  • Amoxicillin or amoxicillin clavulanate (500 mg every 8 hours orally)

  • Ampicillin or tetracycline (250–500 mg four times daily orally)

  • Trimethoprim-sulfamethoxazole (160/800 mg every 12 hours orally)

  • Ciprofloxacin (500–750 mg twice daily orally)

• Sputum smears and cultures should guide therapy where possible

• Preventive or suppressive antibiotics are frequently given to patients with increased purulent sputum, although this practice is not guided by clinical trial data

  • Long-term macrolide therapy (azithromycin 500 mg three times a week orally for 6 months or 250 mg/day for 12 months) has been found to decrease the frequency of exacerbations compared to placebo

  • High-dose (3 g/day) amoxicillin orally or alternating cycles of amoxicillin or amoxicillin clavulanate, ampicillin or tetracycline, trimethoprim-sulfamethoxazole, or ciprofloxacin orally for 2–4 weeks are also used, although this practice is not supported by clinical trial data

• In patients with underlying cystic fibrosis, inhaled aerosolized aminoglycosides reduce colonization by Pseudomonas species, improve FEV₁ and reduce hospitalizations

• In noncystic fibrosis bronchiectasis, inhaled aztreonam is of no benefit, while adding inhaled tobramycin to oral ciprofloxacin for acute exacerbations due to Pseudomonas decreases microbial sputum burden but without apparent clinical benefit

• Inhaled bronchodilators are commonly used as maintenance therapy and in acute exacerbations

Surgery

• Resection is reserved for the few patients with localized bronchiectasis and adequate pulmonary function who do not respond to conservative treatment

• Surgical treatment may be necessary to stop bleeding in some cases of massive hemoptysis

Therapeutic Procedures

• Daily chest physiotherapy with postural drainage and chest percussion

• Bronchoscopy may be needed to evaluate hemoptysis, remove retained secretions, and rule out obstructing lesions

• Pulmonary angiography with embolization may be required to control massive hemoptysis

Follow-Up

• Monitor serial pulmonary function tests and sputum cultures

• If patient is unresponsive to treatment, screen for nontuberculous mycobacteria

Complications

• Hemoptysis

• Hypoxemia

• Cor pulmonale

• Amyloidosis

• Secondary visceral abscesses at distal sites

Prevention

• Influenza vaccine

• Pneumococcal vaccine

• Regular chest physiotherapy

Prognosis

• Depends on cause and severity

When to Refer

• Most cases should be referred to a pulmonary, allergy, clinical immunology, or infectious disease specialist to assist in the evaluation and treatment

When to Admit

• Hypoxemia

• Moderate to severe acute airflow obstruction

• Severe infection