Pirfenidone for the treatment of idiopathic pulmonary fibrosis.
Idiopathic pulmonary fibrosis (IPF) is an under-recognised, rare, progressive disease of the lungs with unknown aetiology and high mortality. The currently advocated pathogenic mechanism is represented by progressive multifocal fibrosis. It is diagnosed based on clinical, radiographic, physiological and histopathological criteria. Existing therapeutic guidelines recommend anti-inflammatory and immunosuppressive combinations, despite proven limited efficacy. There is no therapy approved specifically for IPF, but several antifibrotic agents are currently under development for this indication. Pirfenidone is an antifibrotic agent potentially effective for IPF therapy, and preclinical and available clinical data support its use in IPF. Future clinical studies are expected to provide more consistent information on survival benefit, lung function and health-related quality of life.
Antoniu
Sabina A
SA
Clinic of Pulmonary Disease, 30 Dr I Cihac Street, 700115, Iasi, Romania. sabinaantoniu@excite.com
D000284:Administration, Oral; D000638:Amiodarone / Q000633:toxicity; D000818:Animals; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D001761:Bleomycin / Q000633:toxicity; D002460:Cell Line / Q000187:drug effects; D017322:Clinical Trials, Phase II as Topic; D055513:Connective Tissue Growth Factor; D006224:Cricetinae; D003520:Cyclophosphamide / Q000633:toxicity; D004353:Drug Evaluation, Preclinical; D015507:Drugs, Investigational / Q000627:therapeutic use*; D005347:Fibroblasts / Q000187:drug effects; D022861:Hermanski-Pudlak Syndrome / Q000150:complications; D006801:Humans; D017874:Immediate-Early Proteins / Q000037:antagonists & inhibitors; D036341:Intercellular Signaling Peptides and Proteins; D016040:Lung Transplantation; D051379:Mice; D009965:Orphan Drug Production; D010787:Photosensitivity Disorders / Q000139:chemically induced; D011658:Pulmonary Fibrosis / Q000139:chemically induced / Q000188:drug therapy*; D011728:Pyridones / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D016032:Randomized Controlled Trials as Topic; D012595:Scleroderma, Systemic / Q000150:complications; D016212:Transforming Growth Factor beta / Q000037:antagonists & inhibitors; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
D000894:Anti-Inflammatory Agents, Non-Steroidal; C526317:CCN2 protein, human; C526318:CCN2 protein, mouse; D015507:Drugs, Investigational; D017874:Immediate-Early Proteins; D036341:Intercellular Signaling Peptides and Proteins; D011728:Pyridones; D016212:Transforming Growth Factor beta; D014409:Tumor Necrosis Factor-alpha; D001761:Bleomycin; D055513:Connective Tissue Growth Factor; D003520:Cyclophosphamide; C093844:pirfenidone; D000638:Amiodarone