Бронхоэктатическая болезнь

Causes

• Congenital: cystic fibrosis (CF); Young's syndrome; primary ciliary dyskinesia; Kartagener's syndrome (OHCS).

• Post-infection: measles; pertussis; bronchiolitis; pneumonia;

• TB;

• HIV.

• Other: bronchial obstruction (tumour, foreign body); allergic bronchopulmonary aspergillosis; hypogammaglobulinaemia; rheumatoid arthritis; ulcerative colitis; idiopathic.

Clinical

• Sx: persistent cough; copious purulent sputum; intermittent haemoptysis.

• Signs: finger clubbing; coarse inspiratory crepitations; wheeze (asthma, COPD, ABPA).

• Complications: pneumonia, pleural effusion; pneumothorax; haemoptysis; cerebral abscess; amyloidosis.

Tests Sputum culture.

• HRCT chest: to assess extent and distribution of disease.

• Spirometry - ± obstructive pattern; ✓reversibility.

• Bronchoscopy to locate site of haemoptysis, exclude obstruction and obtain samples for culture.

• Other tests: serum immunoglobulins; CF sweat test; Aspergillus precipitins or skin-prick test.

Management

• Postural drainage should be performed twice daily. Chest physiotherapy may aid sputum expectoration and mucous drainage.

• Antibiotics should be prescribed according to bacterial sensitivities. Patients known to culture Pseudomonas will require either oral ciprofloxacin or IV antibiotics.

  • If ≥3 exacerbations a year consider long-term antibiotics.

• Bronchodilators (eg nebulized salbutamol) in pts /w asthma, COPD, CF, ABPA.

• Corticosteroids (eg prednisolone) for ABPA.

• Surgery may be indicated in localized disease or to control severe haemoptysis.