Bronchiectasis: not an orphan disease in the East

Bronchiectasis is a common disease in the developing world. While the aetiology of bronchiectasis is diverse, many patients suffer from idiopathic disease. Although the pathogenesis of bronchiectasis is poorly understood, there are three distinct pathogenic elements, namely infection, inflammation and enzymatic actions. These interact to perpetuate airway destruction in many cases. There are four patient stereotypes: rapidly progressive, slowly progressive, indolent disease and haemoptysis-predominant. The diagnosis of bronchiectasis is best made with high resolution computed tomography, which should be followed by delineation of aetiology and evaluation of disease severity. Management of bronchiectasis is unsatisfactory and there are no disease-modifying drugs or treatment guidelines. Specific therapy to correct an underlying defect should be instituted whenever possible, although established disease often continues to deteriorate relentlessly. Treatment with prolonged, high-dose antibiotics is useful for exacerbations and probably also for some severely affected patients with frequent exacerbations who habour Pseudomonas aeruginosa in their airways. Commencement of long-term nebulised aminoglycoside, elective in-patient intravenous antibiotic therapy, long-term oral antibiotic or low-dose macrolide therapy requires special considerations. Inhaled corticosteroid therapy reduces chemokine expression in bronchiectasis in vivo, and may be useful for some patients. For severely affected patients, the use of non-invasive positive-pressure ventilation with supplementary oxygen sometimes helps. The lack of enthusiasm about bronchiectasis has already resulted in a lack of research in the treatment of this frustrating disease, and such research needs to be encouraged.