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 and nontuberculous mycobacterial

    • 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 (α₁-antiprotease) deficiency

  • Primary ciliary dyskinesia

  • Rheumatic diseases

    • Rheumatoid arthritis

    • Sjögren syndrome

  • Allergic bronchopulmonary aspergillosis (ABPA)

  • 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 bases 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

• Patients with P aeruginosa infection experience an accelerated course, with more frequent exacerbations and more rapid decline in lung function

Differential Diagnosis

• Chronic obstructive pulmonary disease

• Asthma

• Bronchiolitis

• Allergic bronchopulmonary aspergillosis

Diagnosis

Laboratory Tests

• Complete blood count with differential

• Immunoglobulin quantification

• Testing for cystic fibrosis with sweat chloride level

• Sputum cultures, including for nontuberculous mycobacteria

• Additional testing, if appropriate, may include

  • Aspergillus antibodies

  • α₁-Antitrypsin (α₁-antiprotease) level

  • Ciliary testing

  • Autoimmune serologies

  • Swallow assessment

Imaging Studies

• High-resolution CT is the diagnostic study of choice

• Radiographic abnormalities include

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

  • Scattered irregular opacities

  • Atelectasis

  • Focal consolidation

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, inhaled antibiotics have been shown to reduce exacerbations

Therapeutic Procedures

• Daily chest physiotherapy with postural drainage and chest percussion

• Bronchoscopy ...