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.

[1]  Johny Verschakelen,et al.  High-dose acetylcysteine in idiopathic pulmonary fibrosis. , 2005, The New England journal of medicine.

[2]  Demosthenes Bouros,et al.  Current and future therapeutic approaches in idiopathic pulmonary fibrosis , 2005, European Respiratory Journal.

[3]  S. Kudoh,et al.  Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. , 2005, American journal of respiratory and critical care medicine.

[4]  F. Martinez,et al.  Idiopathic pulmonary fibrosis: emerging concepts on pharmacotherapy , 2004, Expert opinion on pharmacotherapy.

[5]  H. Oku,et al.  Pirfenidone attenuates expression of HSP47 in murine bleomycin-induced pulmonary fibrosis , 2004, European Respiratory Journal.

[6]  Douglas Curran-Everett,et al.  Combined corticosteroid and cyclophosphamide therapy does not alter survival in idiopathic pulmonary fibrosis. , 2004, Chest.

[7]  G. Raghu,et al.  A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. , 2004, The New England journal of medicine.

[8]  D. Hyde,et al.  Anti-Inflammatory Effect of Pirfenidone in the Bleomycin-Hamster Model of Lung Inflammation , 2000, Inflammation.

[9]  H. Misra,et al.  Pirfenidone inhibits NADPH-dependent microsomal lipid peroxidation and scavenges hydroxyl radicals , 2004, Molecular and Cellular Biochemistry.

[10]  H. Forman,et al.  Glutathione regulates transforming growth factor-beta-stimulated collagen production in fibroblasts. , 2004, American journal of physiology. Lung cellular and molecular physiology.

[11]  G. Izbicki,et al.  Role of interferon-γ in the evolution of murine bleomycin lung fibrosis , 2003 .

[12]  T. Massey,et al.  Differential effects of pirfenidone on acute pulmonary injury and ensuing fibrosis in the hamster model of amiodarone-induced pulmonary toxicity. , 2003, Toxicological sciences : an official journal of the Society of Toxicology.

[13]  S. Nagai,et al.  Open-label compassionate use one year-treatment with pirfenidone to patients with chronic pulmonary fibrosis. , 2002, Internal medicine.

[14]  F. Ridolfi,et al.  Effect of pirfenidone on rat hepatic stellate cell proliferation and collagen production. , 2002, Journal of hepatology.

[15]  J. Troendle,et al.  Effect of pirfenidone on the pulmonary fibrosis of Hermansky-Pudlak syndrome. , 2002, Molecular genetics and metabolism.

[16]  H. Oku,et al.  Pirfenidone suppresses tumor necrosis factor-alpha, enhances interleukin-10 and protects mice from endotoxic shock. , 2002, European journal of pharmacology.

[17]  H. Oku,et al.  A novel anti-fibrotic agent pirfenidone suppresses tumor necrosis factor-alpha at the translational level. , 2002, European journal of pharmacology.

[18]  G. Becker,et al.  Pirfenidone reduces in vitro rat renal fibroblast activation and mitogenesis. , 2001, Journal of nephrology.

[19]  J. Tooze,et al.  Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. , 2001, American journal of respiratory and critical care medicine.

[20]  W. Thurlbeck,et al.  Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality. , 2001, American journal of respiratory and critical care medicine.

[21]  J. Egan,et al.  Pulmonary function in idiopathic pulmonary fibrosis and referral for lung transplantation. , 2001, American journal of respiratory and critical care medicine.

[22]  R. Strawderman,et al.  Steroids in idiopathic pulmonary fibrosis: a prospective assessment of adverse reactions, response to therapy, and survival. , 2001, The American journal of medicine.

[23]  A. Pardo,et al.  Idiopathic Pulmonary Fibrosis: Prevailing and Evolving Hypotheses about Its Pathogenesis and Implications for Therapy , 2001, Annals of Internal Medicine.

[24]  F. Martinez,et al.  Cyclophosphamide in the treatment of idiopathic pulmonary fibrosis: a prospective study in patients who failed to respond to corticosteroids. , 2000, Chest.

[25]  S. Iyer,et al.  Effects of pirfenidone on transforming growth factor-beta gene expression at the transcriptional level in bleomycin hamster model of lung fibrosis. , 1999, The Journal of pharmacology and experimental therapeutics.

[26]  G. Raghu,et al.  Treatment of Idiopathic Pulmonary Fibrosis with a New Antifibrotic Agent , Pirfenidone Results of a Prospective , Open-label Phase II Study , 1999 .

[27]  S. Iyer,et al.  Effects of pirfenidone on procollagen gene expression at the transcriptional level in bleomycin hamster model of lung fibrosis. , 1999, The Journal of pharmacology and experimental therapeutics.

[28]  G. Gurujeyalakshmi,et al.  Pirfenidone inhibits PDGF isoforms in bleomycin hamster model of lung fibrosis at the translational level. , 1999, American journal of physiology. Lung cellular and molecular physiology.

[29]  J. Samet,et al.  Idiopathic pulmonary fibrosis: survival in population based and hospital based cohorts , 1998, Thorax.

[30]  M A Schork,et al.  Idiopathic pulmonary fibrosis: predicting response to therapy and survival. , 1998, American journal of respiratory and critical care medicine.

[31]  R. Novick,et al.  Effect of diagnosis on survival benefit of lung transplantation for end-stage lung disease , 1998, The Lancet.

[32]  D. Hyde,et al.  Lung fibrosis is ameliorated by pirfenidone fed in diet after the second dose in a three-dose bleomycin-hamster model. , 1998, Experimental lung research.

[33]  J. Kehrer,et al.  Pirfenidone diminishes cyclophosphamide-induced lung fibrosis in mice. , 1997, Toxicology letters.

[34]  D. Hyde,et al.  Dietary intake of pirfenidone ameliorates bleomycin-induced lung fibrosis in hamsters. , 1995, The Journal of laboratory and clinical medicine.

[35]  H. Magnussen,et al.  The effect of oral N-acetylcysteine on lung glutathione levels in idiopathic pulmonary fibrosis. , 1994, The European respiratory journal.

[36]  G. Raghu,et al.  Azathioprine combined with prednisone in the treatment of idiopathic pulmonary fibrosis: a prospective double-blind, randomized, placebo-controlled clinical trial. , 1991, The American review of respiratory disease.

[37]  R. McAnulty,et al.  Oral N-acetylcysteine reduces bleomycin-induced collagen deposition in the lungs of mice. , 1991, The European respiratory journal.

[38]  A. Cantin,et al.  Extracellular glutathione suppresses human lung fibroblast proliferation. , 1990, American journal of respiratory cell and molecular biology.