Definition

 An abnormal permanent dilation of bronchi, accompanied by inflammation in their walls and in adjacent lung parenchyma.

Epidemiology

 • Incidence rates between 20 and 35 per 100 000 people years.

 • More common in underdeveloped countries due to higher incidence of severe childhood pulmonary infections.

 Aetiology

 • A structural condition resulting from a number of different causes.

 • In developed countries, bronchiectasis is usually related to obstruction to an area of the lung (e.g. tumour or foreign body) or in association with cystic fibrosis (CF). Many cases prove to be idiopathic.

 • In less developed countries, severe pulmonary infections are a major cause.

 Pathogenesis

 • thought to be the result of weakening in bronchial walls caused by recurrent inflammation.

 • Scarring in the adjacent lung parenchyma places traction on the weakened bronchi, causing them to permanently dilate.

 Presentation

 • Persistent productive cough and haemoptysis (which may be massive).

Macroscopy

 • Affected areas of the lung contain visibly dilated airways filled with mucopurulent material which extend right up to the pleural surface.

 • In obstructive cases, the cause may be seen proximally (e.g. a tumour).

Histopathology

 • Bronchial dilation with marked chronic inflammation in the wall, often with lymphoid aggregates and germinal centres.

 • Adjacent alveoli may show an acute and organizing pneumonia.

 Complications

 • Pulmonary hypertension and right ventricular failure (RVF).

 • Deposition of serum amyloid A protein in β- pleated sheets in multiple organs (AA amyloidosis).