Bronchiectasis

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

• Other causes

  • Infection

    • Tuberculosis

    • Fungal

    • Abscess

    • Pneumonia

  • Immunodeficiencies

    • Congenital or acquired hypogammaglobulinemia

    • Common variable immunodeficiency

    • Selective IgA, IgM, and IgG subclass deficiencies

    • AIDS

    • Lymphoma

    • Plasma cell myeloma

    • Leukemia

  • α₁-Antitrypsin deficiency

  • Primary ciliary dyskinesia

  • Rheumatic diseases

    • Rheumatoid arthritis

    • Sjögren syndrome

  • Localized airway obstruction

    • Foreign body

    • Tumor

    • Mucoid impaction

Clinical Findings

Symptoms and Signs

• Chronic cough with production of copious, purulent sputum

• Hemoptysis

• Pleuritic chest pain

• Dyspnea

• Weight loss

• Crackles at the lung cases and wheezing

• 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

• Immunoglobulin quantification

• Testing for cystic fibrosis

• Sputum culture, including for nontuberculous mycobacteria

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

• Treatment of acute exacerbations consists of

  • Antibiotics

  • Inhaled bronchodilators

• Handheld flutter valve devices may be as effective as chest physiotherapy in clearing secretions

• Antibiotic therapy should be guided by sputum smears and prior cultures

• If a specific bacterial pathogen cannot be isolated, then empiric oral antibiotic therapy for 10–14 days is appropriate

• Common regimens include

  • Amoxicillin or amoxicillin-clavulanate

  • Ampicillin

  • A second- or third-generation cephalosporin

  • Doxycycline

  • A fluoroquinolone

• For recurrent exacerbations,

  • Preventive macrolide therapy for 6–12 months has been found to decrease the frequency of exacerbations

  • Alternatively, a trial of inhaled antibiotics may be used

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

Outcome

Follow-Up

• Monitor serial pulmonary function tests and sputum cultures

• If patient is unresponsive to treatment, screen ...