Inflammation and anti-inflammatory therapies for cystic fibrosis.
Cystic fibrosis lung disease is characterized by a self-propagating cycle of obstruction, infection, and inflammation. The inflammatory response, which accounts for the majority of the morbidity and mortality of the disease, begins early in life, becomes persistent, and is excessive relative to the bacterial burden. Therapies aimed at decreasing the inflammatory response represent a relatively new strategy for treatment. This article reviews the current state of the art of anti-inflammatory therapy in cystic fibrosis and introduces clinical trials that are underway.
Chmiel
James F
JF
Division of Pediatric Pulmonology, Department of Pediatrics, Case Western Reserve University School of Medicine, 11100 Euclid Avenue, Cleveland, OH 44106, USA.
Konstan
Michael W
MW
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D000975:Antioxidants / Q000627:therapeutic use; D003550:Cystic Fibrosis / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D016207:Cytokines / Q000627:therapeutic use; D018450:Disease Progression; D006801:Humans; D007052:Ibuprofen / Q000627:therapeutic use; D007249:Inflammation; D018836:Inflammation Mediators / Q000627:therapeutic use*; D011480:Protease Inhibitors / Q000627:therapeutic use; D015398:Signal Transduction
D000305:Adrenal Cortex Hormones; D000893:Anti-Inflammatory Agents; D000975:Antioxidants; D016207:Cytokines; D018836:Inflammation Mediators; D011480:Protease Inhibitors; D007052:Ibuprofen