Bronchiectasis I

Bronchiectasis I

OVERVIEW:

Chronic irreversible, abnormal dilatation of the bronchi, usually accompanied by infection and productive cough

System(s) affected: Pulmonary
Genetics: Associated with many conditions including some that are congenital or hereditary
Incidence/Prevalence in USA:
• No reliable figures available
• Less common than it once was, probably due to more effective treatment of childhood respiratory infections
Predominant age: Begins most often in early childhood, but symptoms may not appear until later in life
Predominant sex: Male = Female

SIGNS AND SYMPTOMS:

• Cough
• Sputum - copious and purulent
• Hemoptysis
• Wheezing
• Coarse or moist crackles
• Cyanosis
• Digital clubbing
• Dyspnea
• Barrel chest
• Emaciation
• Fatigue
• Fever
• Recurrent pneumonia
• Tachycardia
• Tachypnea

CAUSES:

• Alpha-1-antitrypsin deficiency
• Allergic bronchopulmonary aspergillosis
• Bronchial obstruction
• Cystic fibrosis
• Dyskinetic cilia syndromes
• Hypogammaglobulinemia
• Inhaling noxious chemicals
• Kartagener's syndrome (situs inversus, sinusitis, immotile spermatozoa, bronchiectasis)
• Necrotizing pulmonary infections
• Pulmonary abscess
• Severe lung infection in childhood (measles, adenovirus, influenza, pertussis, or bronchiolitis)
• Tuberculosis
• Congenital immunodeficiency syndromes
• Chronic aspiration
• Rheumatic diseases
• Transplant graft rejection

RISK FACTORS:

• Repeated bouts of pneumonia
• Any chronic respiratory illness
• Retained foreign body
• Immunodeficiency

DIAGNOSIS

DIFFERENTIAL DIAGNOSIS:

• Chronic bronchitis
• Chronic obstructive pulmonary disease
• Cystic fibrosis
• Pulmonary tuberculosis
• Allergic bronchopulmonary aspergillosis

LABORATORY:

• Positive sputum culture (yields H. influenzae, Streptococcus pneumoniae, staphylococcal, klebsiella, pseudomonas, or anaerobes)
• Hypoxemia
• Leukocytosis, usually
• Serum immunoglobulins - check for hypogammaglobulinemia, IgE level helpful

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS:

• Bronchial dilation
• Inflamed bronchi
• Purulent bronchorrhea
• Necrosis of bronchial mucosa
• Peribronchial scarring

SPECIAL TESTS:

• Sweat test
• Skin test for aspergillus
• Bronchoscopy useful in locating bleeding site and to exclude adenoma or foreign body
• Ciliary biopsy with electron microscopy (EM)
• Pulmonary function tests show variable obstruction and restriction
• Sputum culture/sensitivity, AFB, fungus

IMAGING:

• Bronchography
• For definitive diagnosis, to help determine extent, and if surgery contemplated
• Bronchial dilation, truncated
• Chest x-ray
• Can be normal
• Coarse lung markings - honeycomb/tram tracks
• Air-fluid level
• Cystic lesions
• Atelectasis
• CT scan
• Shows dilation of airways with signet rings
• High resolution CT is best to establish diagnosis and extent of disease
• Spiral CT helpful for questionable findings

DIAGNOSTIC PROCEDURES:

• Fiberoptic bronchoscopy
• Recommended when disease is of recent onset or is unilateral
• May be combined with bronchography
• Obtain culture

TREATMENT

APPROPRIATE HEALTH CARE:

Outpatient except for possible surgery

GENERAL MEASURES:

• Airway clearance techniques
• Chest physical therapy
• Percussion
• Postural drainage
• Hydration
• Pulmonary rehabilitation to improve functional status
• Noninvasive positive pressure ventilation, nocturnal or chronic
• Bronchial artery embolization may be lifesaving for massive pulmonary hemorrhage
• Avoid cigarette smoking
• Bronchoscopy may be required for extraction of mucus or mycelial plugs, or if physiotherapy has failed

SURGICAL MEASURES:

Segmental pulmonary resection for localized disease or refractory hemoptysis

ACTIVITY:

As fully active as possible

DIET:

No restrictions

PATIENT EDUCATION:

Printed patient information available from: American Lung Association, 1740 Broadway, New York, NY 10019, (212)315-8700

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SEE ALSO (Enter the keywords below into our search box or click on the link):

Aspergillosis
Bronchiolitis obliterans & organizing pneumonia
Cystic fibrosis
Lung abscess

Medical Diagnosis » B » Bronchiectasis I