Antibiotic and Anti-Inflammatory Therapies for Cystic Fibrosis

  1. J. Stuart Elborn2
  1. 1Department of Pediatrics, Case Western Reserve University School of Medicine, Rainbow Babies and Children’s Hospital, Cleveland, Ohio 44106
  2. 2Medicine and Surgery, Queens University Belfast, Belfast City Hospital, Belfast BT9 7AB, Northern Ireland, United Kingdom
  1. Correspondence: michael.konstan{at}case.edu

Abstract

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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