Bronchiectasis
MALCOLM K. BENSON
Bronchiectasis is a morphological disorder which represents endorgan damage resulting from a number of causes. Inflammation, usually dur to infection combined with localized obstruction, results in destruction and dilatation of one or more bronchi. As a consequence of this anatomical disruption there is a failure of normal mucociliary clearance, pooling of secretions, and recurrent bronchial sepsis. Cough and excessive sputum production are the major symptoms; these may be accompanied by breathlessness and respiratory failure if disease is widespread.
PATHOGENESIS
Knowledge of the underlying cause is important: correction of causative factors may be as important in overall management as the treatment of established disease. The main conditions associated with the development of brnchiectasis are listed in Table 1 543. Damage following childhood pneumonia was for many years regarded as an important cause. Post-infective damage now seems less common, probably because of the benefits of immunization and prompt treatment with antibiotics and physiotherapy. A small proportion of patients with bronchiectasis have a classical immune deficit, and identification of this group is important since immune replacement can prevent further infections. Mechanical obstruction due to an intraluminal foreign body or tumour will result in repeated infections and progressive damage. Allergic bronchopulmonary aspergillosis should be suspected if there is an antecedent history of atopy or asthma accompanied by the expectoration of viscid plugs of sputum. The role of antifungal drugs is yet to be clearly established in this condition, but the treatment with steroids during exacerbations may reduce the inflammatory response and consequent scarring.
TREATMENT OF ESTABLISHED DISEASE
The mainstay of medical treatment is a combination of physiotherapy and antibiotics. Postural drainage has long been established as a useful way of clearing accumulated mucus. The patient is taught the most appropriate position which will enable gravity to assist in expectoration. This can be assisted by percussion techniques and the use of a forced expiratory ‘huffing’ manoeuvre. Ideally physiotherapy should be undertaken regularly once or twice daily, although compliance is often poor.
Antibiotics
There is little doubt that antibiotics are beneficial in acute infective exacerbations, both in reducing the volume and purulence of sputum and in treating the constitutional symptoms. There is debate as to the most appropriate regimen for patients who have chronic sepsis and progressive damage. Many patients have relatively infrequent infective exacerbations and require short courses of antibiotics for 1 or 2 weeks during these episodes. Patients whose condition relapses once antibiotics have been stopped and those who are chronically infected require treatment on a more regular basis. Two options exist. One approach is to use continuous antibiotics, which are changed every few weeks to reduce the development of resistant organisms. An alternative is to give ‘pulsed’ courses of intravenous antibiotics for short periods, repeated every 2 or 3 months. This approach has been successfully used in some patients with cystic fibrosis. In practice, treatment tailored to suit an individual patient is preferable to strict adherence to a fixed regimen.
The antibiotic chosen should have a sufficiently wide spectrum to cover the organisms which most commonly colonize the bronchial tree. If the initial choice fails, further treatment should be based on results of sputum culture and sensitivity testing. Colonization with Pseudomonas is common, and its eradication virtually impossible. Treatment usually requires parenteral administration of antibiotics: the decision to treat should be taken on clinical grounds rather than on the basis of a microbiological isolate. Preliminary studies have reported favourable results with nebulized antibiotics although few are as yet licensed for use by this route.
Management of complications
Haemoptysis is a frequent symptom in patients with bronchiectasis, although bleeding is usually not life-threatening and tends to be self-limiting. If major haemoptysis occurs the source of the bleeding should be identified by a bronchoscopy or arteriography. If embolization of the appropriate vessel is technically not feasible, the bleeding segment of lung may have to be resected.
RESPIRATORY FAILURE
Patients with generalized disease may develop airway obstruction and respiratory failure. The degree of reversibility following treatment with inhaled bronchodilators is likely to be small, but even a limited improvement in airway function can be beneficial. It may also aid mucociliary clearance. Steroids and mucolytics have been used, but no consensus has been reached as to their benefit.
FURTHER READING
Currie DC, et al. Double blind randomized study of prolonged higher dose oral amoxycillin in purulent bronchiectasis. Q J Med 1990; 76: 799–816.
Ellis DA, Thornley PE, Wightman AJ, Walker M, Chalmers J, Crofton JW. Present outlook in bronchiectasis: clinical and social study and review of factors influencing prognosis. Thorax 1981; 36: 659–64.
Stockley RA. Bronchiectasis—new therapeutic approaches based on pathogenesis. Clin Chest med 1987; 8: 481–94.