[Bronchiectasis--an orphan disease? Diagnosis and treatment in the 21st century]

Bronchiectasis has been termed an "orphan disease" since its decline in the developed world but it is still common in developing countries and in lower socioeconomic classes. Recurrent respiratory infections, under treatment with antibiotics and poor immunization rates are the main causes for the relatively high prevalence in these populations. Today the disease is not often adequately diagnosed nor treated. Airways dilatation, poor clearance of secretions and recurrent infections are the basis of the disease that is characterized by chronic productive purulent cough and recurrent exacerbations. The disease can be localized or diffuse, primary or secondary to other systemic disease. High resolution CT is the examination of choice for diagnosis. Lack of sufficient randomized control trials makes it difficult to establish guidelines. Nevertheless, partial information favors the use of prolonged antibiotic treatment, mucolytic agents and inhaled corticosteroids. Treatment should be tailored to the individual patient guided by the clinical picture while bronchodilators, bronchopulmonary hygiene and physical therapy are optional. Surgical resection is recommended for symptomatic localized bronchiectatic disease while lung transplantation should be reserved, as a last resort, for end-stage bilateral disease.