Clinical Pathology · MU Sofia

62. Bronchiectasis – congenital and acquired. Bronchiectatic disease. Complications.

Covers bronchiectasis as permanent bronchial dilation from chronic necrotising infection or obstruction, noting its usual bilateral lower-lobe distribution and gross appearance. Lists causes grouped as obstructive, congenital (including cystic fibrosis, immunodeficiency and Kartagener syndrome, with its ciliary defect explained) and necrotising pneumonia. Then describes the pathogenesis, clinical features, severe complications such as cor pulmonale, and a brief treatment list.

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Topic 62 of 138 in the MU Sofia syllabus for Clinical Pathology (Medicine, Year 4). The free AceMed note covers it in 1 section, about 336 words (2 min of reading), with 15 flashcards.

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  1. Bronchiectasis - congenital and acquired. Bronchiectatic disease. Complications

Bronchiectasis - congenital and acquired. Bronchiectatic disease. Complications

Bronchiectasis is the permanent dilation of bronchi and bronchioles caused by destruction of muscle and elastic tissue due to chronic necrotising infections. It can really be considered a complication to persistent infections or obstructions. It usually affects the lower lobes bilaterally, although when caused by a tumour or aspiration of an FB may affect a single lobe. Airways may be dilated up to 4x, and gross examination shows large cavities.

Gross: bronchiectasis. Bronchi are dilated into large cylindrical and saccular cavities that reach close to the pleura, with fibrous thickening around them.

Image: “Bronchiectasis” by Yale Rosen, CC BY-SA 2.0, via Wikimedia Commons.

Common causes are:

  • Bronchial obstruction (due to tumors, FBs or impaction of mucus)
  • Bronchiectasis is localised to obstructive lung segment
  • Congenital conditions (e.g. cystic fibrosis, immunodeficiency states, kartagener syndrome)
  • Due to the excess mucous (CF), constant infections (ID states) or structural abnormality of mucociliary transport system (Kartagener syndrome)
  • Necrotising, or suppurative pneumonia (particularly with virulent organisms such as S. aureus or Klebsiella spp.)

Example: Kartagener syndrome is a form of primary ciliary dyskinesia: defective dynein arms leave cilia immotile, giving the triad bronchiectasis + chronic sinusitis + situs inversus; immotile sperm also cause male infertility.

All of these have the same PG. Blockage/chronic infection leads to inflammatory damage of bronchial wall, with accumulation of exudate and further distended airways, causing irreversible distension.

Clinical manifestation is that of severe, persistent cough with mucopurulent sputum which may contain blood. Symptoms are often episodic, and brought on by upper respiratory tract infections. In the most severe cases, hypoxemia, hypercapnia, pulmonary hypertension and cor pulmonale can then commence. Less frequent complications include metastatic brain abscesses and reactive amyloidosis.

GP NOTEBOOK SAYS

Treatment:

  • Physiotherapy which includes airway clearance techniques and exercise (e.g postural drainage)
  • Simple airway clearance techniques like active cycle of breathing and positive expiratory devices in non-cystic fibrosis cases
  • Nebulisation of normal saline or beta 2 agonists
  • Inhaled steroids only with coexisting asthma or COPD; AB in infective cases
  • Surgical lung resection may also be considered

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