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For further information, see CMDT Part 9-08: 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

  • Chronic infection with P aeruginosa is a marker for disease severity, frequent exacerbation, accelerated course, and 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 (IgG, IgM, IgA)

  • Testing for cystic fibrosis with sweat chloride level and/or mutation analysis of CFTR gene

  • Sputum cultures, including for bacteria, nontuberculous mycobacteria, and fungi

  • Additional testing, if appropriate, may include

    • Aspergillus antibodies

    • Total serum IgE

    • α1-Antitrypsin (α1-antiprotease) level, genotype, or both

    • 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 “end-on-ring” markings

    • Mucus plugs in the airways

    • Atelectasis

    • Focal consolidation

TREATMENT

Medications

  • Treatment of acute exacerbations consists of

    • Antibiotics

    • Airway clearance (postural drainage, chest percussion, hypertonic saline nebulization)

    • 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

    • Azithromycin

    • A fluoroquinolone

  • For recurrent exacerbations,

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

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