Skip to Main Content

Key Features

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, multiple myeloma, chronic kidney disease, chronic liver disease

      • α1-Antiprotease deficiency with cigarette smoking

      • Mucociliary clearance disorders (eg, immotile cilia syndrome)

      • Rheumatic disease (eg, rheumatoid arthritis)

    • Localized airway obstruction

Clinical Findings

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 non-cystic fibrosis patients with bronchiectasis

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

Differential Diagnosis

  • Chronic obstructive pulmonary disease

  • Asthma

  • Bronchiolitis

  • Allergic bronchopulmonary mycosis

Diagnosis

Laboratory Tests

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

  • Quantitative pilocarpine iontophoresis sweat test for sodium and chloride levels

  • Quantitative immunoglobulins

  • α1-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

Treatment

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/d for 12 months) has been found to decrease the frequency of exacerbations compared to placebo

    • High-dose (3 g/d) amoxicillin orally or alternating cycles of amoxicillin or amoxicillin clavulanate, ampicillin or tetracycline, trimethoprim-sulfamethoxazole, or ciprofloxacin orally for 2–4 weeks ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.