Emerging drugs for bronchiectasis.
BACKGROUND
The global burden of disease due to bronchiectasis is high, disproportionately impacting developing countries and disadvantaged populations. Bronchiectasis, the destruction and dilation of airways, is due to a variety of causes and is characterized by a self-perpetuating cycle of airway inflammation, infection and obstruction that results in substantial morbidity and mortality. Although many therapies have been tested that address each of these three components, as well as the diseases that both cause and result from bronchiectasis, there have been few randomized, placebo-controlled trials.


METHODS
In this review, current knowledge of the clinical features, pathophysiology and epidemiology of bronchiectasis among both adults and children is summarized. We discuss the quality and extent of evidence supporting current treatment strategies, focusing on therapies for which the strongest evidence of efficacy exists. We then identify key goals for future research on the causes and treatments of a variety of types of bronchiectasis.


CONCLUSIONS
Significant advances in the prevention and treatment of bronchiectasis will require substantially improved understanding of the pathogenesis of this orphan disease. A concerted, global effort to coordinate studies of both the pathophysiology and potential treatments of bronchiectasis, in its many forms, could lead to substantial improvements in outcomes.
Queensland Children's Medical Research Institute, Royal Children's Hospital, Queensland. annechang@ausdoctors.net
Anne B
AB
Chang
Robyn L
RL
Marsh
Heidi C
HC
Smith-Vaughan
Lucas R
LR
Hoffman
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000191:economics / Q000627:therapeutic use*; D000328:Adult; D000900:Anti-Bacterial Agents / Q000008:administration & dosage / Q000191:economics / Q000627:therapeutic use*; D001987:Bronchiectasis / Q000175:diagnosis / Q000188:drug therapy* / Q000191:economics / Q000453:epidemiology / Q000209:etiology / Q000382:microbiology; D001993:Bronchodilator Agents / Q000008:administration & dosage / Q000191:economics / Q000627:therapeutic use*; D002648:Child; D002986:Clinical Trials as Topic; D055808:Drug Discovery; D005100:Expectorants / Q000008:administration & dosage / Q000191:economics / Q000627:therapeutic use*; D006801:Humans; D040541:Marketing; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D000900:Anti-Bacterial Agents; D001993:Bronchodilator Agents; D005100:Expectorants