Promising pharmacologic innovations in treating pulmonary fibrosis.
Idiopathic pulmonary fibrosis is the most common form of the interstitial lung diseases and is characterized by chronic progressive pulmonary parenchymal fibrosis. Although the diagnosis and pathophysiology of this disease have been better characterized over the past few years, there remains no effective therapy for this disease. Therapies initially aimed at inflammation have proven ineffective, and newer strategies targeting aspects of aberrant wound repair involving alveolar epithelial cells or septal endothelial cells are now being investigated. Therapeutic strategies include the anti-fibrotic agents pirfenidone and interferon-gamma. Agents targeting specific cytokines, including connective tissue growth factor, transforming growth factor-beta and chemokines, are being evaluated. The restoration of oxidant balance and inhibition of leukotrienes represent other strategies. Additionally, the role of the coagulation/fibrinolytic systems and angiogenesis has also been examined.
Bhatt
Nitin
N
Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Ohio State University, 201 Heart and Lung Research Institute, 473 W 12th Avenue, Columbus, OH 43210, USA.
Baran
Christopher P
CP
Allen
James
J
Magro
Cynthia
C
Marsh
Clay B
CB
D020533:Angiogenesis Inhibitors / Q000494:pharmacology / Q000627:therapeutic use*; D000818:Animals; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000494:pharmacology / Q000627:therapeutic use*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D000975:Antioxidants / Q000494:pharmacology / Q000627:therapeutic use*; D055513:Connective Tissue Growth Factor; D006801:Humans; D017874:Immediate-Early Proteins / Q000276:immunology / Q000378:metabolism; D036341:Intercellular Signaling Peptides and Proteins / Q000276:immunology / Q000378:metabolism; D008168:Lung / Q000098:blood supply* / Q000187:drug effects; D008982:Molybdenum / Q000494:pharmacology / Q000627:therapeutic use; D009389:Neovascularization, Pathologic / Q000517:prevention & control*; D011658:Pulmonary Fibrosis / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D011728:Pyridones / Q000494:pharmacology / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic
D020533:Angiogenesis Inhibitors; D000894:Anti-Inflammatory Agents, Non-Steroidal; D000911:Antibodies, Monoclonal; D000975:Antioxidants; C526317:CCN2 protein, human; D017874:Immediate-Early Proteins; D036341:Intercellular Signaling Peptides and Proteins; D011728:Pyridones; D055513:Connective Tissue Growth Factor; D008982:Molybdenum; C020809:tetrathiomolybdate; C093844:pirfenidone