Non-cystic fibrosis bronchiectasis: its diagnosis and management

Abstract

An increase in the frequency of diagnosing non-cystic fibrosis bronchiectasis in children is due to heightened awareness of the disease and the wider availability of high-resolution computed tomography. The most common underlying conditions leading to bronchiectasis include infections, immunodeficiency, aspiration and primary ciliary dyskinesia. Treatment centres on airway clearance with aggressive antibiotic regimens and physiotherapy; more specific approaches are available for some of the underlying conditions. A high index of suspicion that a child may have underlying bronchiectasis must be maintained in the presence of prolonged or recurrent "wet/productive" cough. The classic definition of bronchiectasis is of irreversible bronchial dilatation; however, at the milder end of the spectrum it appears that radiographic changes may be reversible. Untreated, in its severest form bronchiectasis can progress to end stage pulmonary failure in adult life. In this article, we review its pathogenesis and diagnosis and the evidence base for available treatments.

Bronchiectasis: why the diagnosis shouldn't be missed in primary care

“…[1][2][3] Part of the reason for this is the impression that the condition is uncommonly seen and is therefore of decreasing importance -particularly in the developed world -mainly as a consequence of childhood vaccination and the effective use of antibiotics for the management of respiratory tract infections. However, since the advent of high resolution CT (HRCT) scanning of the chest, the diagnosis of bronchiectasis is being made with increasing frequency throughout the world and the condition has become recognised as an important and common cause of respiratory disease.…”

“…9 Although symptoms are very non-specific, the diagnosis should always be suspected in an individual presenting with cough productive of sputum, and/or unexplained haemoptysis. [1][2][3][4] The diagnosis should also be considered in children with "asthma" that responds poorly to treatment, and similarly in adults with "chronic obstructive pulmonary disease (COPD)". 2,4,5,9 The plain chest X-ray -which is recommended for all patients -is not sufficiently sensitive for adequate diagnosis of the condition, and the use of bronchography has been replaced by HRCT scanning of the chest, which is currently considered the gold standard for bronchiectasis diagnosis.…”

“…[1][2][3][4] The diagnosis should also be considered in children with "asthma" that responds poorly to treatment, and similarly in adults with "chronic obstructive pulmonary disease (COPD)". 2,4,5,9 The plain chest X-ray -which is recommended for all patients -is not sufficiently sensitive for adequate diagnosis of the condition, and the use of bronchography has been replaced by HRCT scanning of the chest, which is currently considered the gold standard for bronchiectasis diagnosis. 1,2 It is the more widespread use of HRCT scanning that has largely contributed to the recognition of the importance of bronchiectasis.…”

“…However, since the advent of high resolution CT (HRCT) scanning of the chest, the diagnosis of bronchiectasis is being made with increasing frequency throughout the world and the condition has become recognised as an important and common cause of respiratory disease. [2][3][4][5] Bronchiectasis is important to recognise since it may cause severe and recurrent pulmonary infections, with associated permanent and progressive loss of lung function leading to significant morbidity and even premature mortality. 1 For example, a recent mortality study conducted in England and Wales reported the number of deaths from bronchiectasis to be increasing by 3% per annum in the older age groups, and this despite likely underreporting -thus necessitating increased clinical provision for these patients to reverse this trend.…”

“…Whilst there are a myriad of treatment options potentially available for the management of patients with bronchiectasis, few have been appropriately studied in randomised controlled trials; many treatment recommendations are based either on guidelines for the management of cystic fibrosis, or extrapolation from the management of other respiratory conditions, or even purely expert opinion. 2,14,15 The general aims of treatment are to limit the cycle of infection and inflammation in order to halt the progression of airway damage, to reduce symptoms and the number of exacerbations, and to improve quality of life. 1,7 The natural history of bronchiectasis and its associated mortality can be improved with expert management, with increasing evidence that early and intensive treatment can improve lung function in children and prevent deterioration, reduce respiratory exacerbations, and improve the overall long-term prognosis.…”