Antibiotic and anti-inflammatory therapies for cystic fibrosis

JF Chmiel, MW Konstan… - Cold Spring …, 2013 - perspectivesinmedicine.cshlp.org
JF Chmiel, MW Konstan, JS Elborn
Cold Spring Harbor Perspectives in Medicine, 2013perspectivesinmedicine.cshlp.org
Cystic fibrosis (CF) lung disease is characterized by chronic bacterial infection and an
unremitting inflammatory response, which are responsible for most of CF morbidity and
mortality. The median expected survival has increased from< 6 mo in 1940 to> 38 yr now.
This dramatic improvement, although not great enough, is due to the development of
therapies directed at secondary disease pathologies, especially antibiotics. The importance
of developing treatments directed against the vigorous inflammatory response was realized …
Cystic fibrosis (CF) lung disease is characterized by chronic bacterial infection and an unremitting inflammatory response, which are responsible for most of CF morbidity and mortality. The median expected survival has increased from <6 mo in 1940 to >38 yr now. This dramatic improvement, although not great enough, is due to the development of therapies directed at secondary disease pathologies, especially antibiotics. The importance of developing treatments directed against the vigorous inflammatory response was realized in the 1990s. New therapies directed toward the basic defect are now visible on the horizon. However, the impact of these drugs on downstream pathological consequences is unknown. It is likely that antibiotics and anti-inflammatory drugs will remain an important part of the maintenance regimen for CF in the foreseeable future. Current and future antibiotic and anti-inflammatory therapies for CF are reviewed.
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