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Bronchiectasis

Title               

Ñ    Bronchiectasis

Definition

A condition in which there is:

Ñ    Fixed dilatation of the bronchi

Pathogenesis

Bronchiectasis can result from lesions in the following:

Lumen

Obstruction by:

Ñ    Mucous plug

Ñ    Tumour

Ñ    Inhaled foreign body

Mural Factors

Infections

Especially childhood infections:

Ñ    Measles, whooping cough

Other infections:

Ñ    Tuberculosis, pneumococcal pneumonia, adenovirus and AIDS

Mucociliary Clearance Defects

Ñ    Cystic fibrosis

Ñ    Immotile cilia

Ñ    Kartagener’s syndrome

Miscellaneous

Ñ    Marfan’s syndrome

Ñ    Yellow nail syndrome

External Factors

Ñ    Fibrosis of the lungs

Ñ    Compression by a lymph node

Systemic Factors

HS

Immune deficiency states:

Ñ    Hypogammaglobulinaemia

Ñ    Complement deficiency

Ñ    Phagocyte defects

Ñ    Chronic granulomatous disease

RS

Hyperimmune states:

Ñ    Allergic bronchopulmonary aspergillosis

Ñ    Following lung transplantation

GIT

Ñ    Inflammatory bowel disease

Ñ    Gastric aspiration

LMS

Ñ    Rheumatoid arthritis

Pathological Process

Ñ    Damage to the bronchial wall occurs in the setting of:

An infectious insult

Impairment of drainage

Airway obstruction

Defect in host defence

Ñ    Neutrophil proteases, inflammatory cytokines, nitric oxide, oxygen free radicals cause tissue damage

Ñ    Muscular and elastic components of the bronchial wall are damaged

Ñ    In addition, peribronchial alveolar tissue may be damaged, resulting in diffuse peribronchial fibrosis

Ñ    The result is abnormal bronchial dilatation with bronchial wall destruction and transmural inflammation.

Ñ    Functionally, this severely impairs clearance of secretions from the bronchial tree

Ñ    Impaired clearance of secretions causes colonisation and infection with pathogenic organisms

Ñ    This results in further bronchial damage and a cycle of bronchial damage, bronchial dilation, impaired clearance of secretions, recurrent infection and more bronchial damage

Clinical Features

History

Ñ    Symptoms may date from childhood

E&M

Ñ    Fever

RS

Ñ    Cough, sputum production

Ñ    Chest pain

Ñ    Breathlessness

Ñ    Haemoptysis

Ñ    Halitosis

Examination

E&M

Ñ    Fever

IS

Ñ    Cyanosis

Ñ    Clubbing

RS

Ñ    Crepitations (crackles) over affected area

Usually:

Ñ    Bilateral or unilateral coarse basal crepitations

Sometimes

Ñ    Widespread crepitations

Ñ    Rhonchi

Complications

IS

Ñ    Cutaneous vasculitis

CVS

Ñ    Cor pulmonale

RS

Ñ    Severe haemoptysis

Ñ    Respiratory failure

KUS

Ñ    Amyloidosis

LMS

Ñ    Seronegative arthropathy

Investigations

Fluids

Sputum

Major pathogens include:

Ñ    Staphylococcus aureus

Ñ    Pseudomonas aeroginosa

Ñ    Haemophilus influenzae

Ñ    Anaerobes

Blood

Immunology

Ñ    Serum immunoglobulins:

10% of adults have immune deficiency

Imaging

Chest X-ray

Ñ    Normal

Ñ    Dilated bronchi with thickened bronchial walls

Ñ    Multiple cysts containing fluid

High Resolution CT scan (HRCT)

Ñ    Bronchial dilatation and wall thickening

Bronchography

Ñ    Rarely performed

Sinus X-rays

Ñ    30% have rhinosinusitis

Physiological studies

Mucociliary clearance

Ñ    1 mm cube of saccharin placed on the inferior turbinate

Ñ    Time taken to taste should be less than 30 minutes

Management

Control

Drugs

Ñ    Antibiotics:

Eradicate bronchopulmonary infections to try and halt progression of disease

Ñ    Bronchodilators:

If there is demonstrable airflow obstruction

Ñ    Steroids:

Inhaled or oral can decrease the rate of progression

Surgery

Ñ    Localised resection:

Rarely used

Staffing

Ñ    Physiotherapy:

Teach postural drainage

Support

Biological Agents

Ñ    Lung transplant

Ñ    Heart lung transplant

Prognosis

Ñ    With antibiotics prognosis has improved

Ñ    Most will develop respiratory failure and cor pulmonale

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