Antiinflammatory therapies for cystic fibrosis: past, present, and future.
Inflammation is a major component of the vicious cycle characterizing cystic fibrosis pulmonary disease. If untreated, this inflammatory process irreversibly damages the airways, leading to bronchiectasis and ultimately respiratory failure. Antiinflammatory drugs for cystic fibrosis lung disease appear to have beneficial effects on disease parameters. These agents include oral corticosteroids and ibuprofen, as well as azithromycin, which, in addition to its antimicrobial effects, also possesses antiinflammatory properties. Inhaled corticosteroids, colchicine, methotrexate, montelukast, pentoxifylline, nutritional supplements, and protease replacement have not had a significant impact on the disease. Therapy with oral corticosteroids, ibuprofen, and fish oil is limited by adverse effects. Azithromycin appears to be safe and effective, and is thus the most promising antiinflammatory therapy available for patients with cystic fibrosis. Pharmacologic therapy with antiinflammatory agents should be started early in the disease course, before extensive irreversible lung damage has occurred.
School of Pharmacy and Pharmaceutical Sciences, State University of New York at Buffalo, Buffalo, New York 14260, USA. prescott@buffalo.edu
William A
WA
Prescott
Cary E
CE
Johnson
D000085:Acetates / Q000627:therapeutic use; D000280:Administration, Inhalation; D000284:Administration, Oral; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D017963:Azithromycin / Q000627:therapeutic use; D002648:Child; D002986:Clinical Trials as Topic; D003521:Cyclopropanes; D003550:Cystic Fibrosis / Q000188:drug therapy*; D003850:Deoxyribonuclease I / Q000627:therapeutic use; D019587:Dietary Supplements; D006801:Humans; D008727:Methotrexate / Q000627:therapeutic use; D010431:Pentoxifylline / Q000627:therapeutic use; D011804:Quinolines / Q000627:therapeutic use; D013440:Sulfides
D000085:Acetates; D000893:Anti-Inflammatory Agents; D003521:Cyclopropanes; D011804:Quinolines; D013440:Sulfides; D017963:Azithromycin; C085693:DNASE1 protein, human; D003850:Deoxyribonuclease I; C093875:montelukast; D010431:Pentoxifylline; D008727:Methotrexate