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Pharmacological treatment of idiopathic pulmonary fibrosis: from the past to the future

Katerina M. Antoniou, George A. Margaritopoulos, Nikos M. Siafakas
European Respiratory Review 2013 22: 281-291; DOI: 10.1183/09059180.00002113
Katerina M. Antoniou
Interstitial Lung Disease Unit, University Hospital of Heraklion, Medical School, University of Crete, Heraklion, Greece.
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  • For correspondence: kantoniou@med.uoc.gr
George A. Margaritopoulos
Interstitial Lung Disease Unit, University Hospital of Heraklion, Medical School, University of Crete, Heraklion, Greece.
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Nikos M. Siafakas
Interstitial Lung Disease Unit, University Hospital of Heraklion, Medical School, University of Crete, Heraklion, Greece.
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Tables

  • Table 1. Completed clinical trials in idiopathic pulmonary fibrosis
    DrugMechanism of Action
    IFIGENIA [20]NACAntioxidant
    PANTHER-IPF [21]Prednisone
    Azathioprine
    NAC
    Antioxidant
    Immunosuppression
    Taniguchi [22]PirfenidoneAntifibrotic
    CAPACITY 1 [23]PirfenidoneAntifibrotic
    CAPACITY 2 [23]PirfenidoneAntifibrotic
    ACE-IPF [24]WarfarinAnticoagulant
    TOMORROW [25]BIBF 1120Tyrosine-kinase inhibitor
    Daniels [26]Imatinib mesylateTyrosine-kinase inhibitor
    STEP-IPF [27]SildenafilPhosphodiesterase-5 inhibitor
    BUILD-1 [28]BosentanEndothelin-receptor antagonist
    BUILD-3 [29]BosentanEndothelin-receptor antagonist
    ARTEMIS-IPF [30]AmbrisentanEndothelin-receptor antagonist
    MUSIC-IPF [31]MacitentanEndothelin-receptor antagonist
    Raghu [32]IFN-γImmunomodulation
    INSPIRE [33]IFN-γImmunomodulation
    • NAC: N-acetyl-cysteine; IFN: interferon.

  • Table 2. Ongoing clinical trials in idiopathic pulmonary fibrosis
    Trial#DrugMechanism of action
    ASCEND (NCT01366209)PirfenidoneAntifibrotic
    NCT01335477BIBF 1120Tyrosine-kinase inhibitor
    NCT01335464BIBF 1120Tyrosine-kinase inhibitor
    NCT01266135QAX576Anti-IL-13 monoclonal antibody
    NCT00786201CNTO 888Anti-CCL-2 monoclonal antibody
    NCT01262001FG-3019Anti-CTGF antibody
    NCT01362231AB0024Anti-LOXL2 monoclonal antibody
    NCT01385644MSCsEpithelial tissue repair
    • MSC: mesenchymal stem cell; IL: interleukin; CCL: chemokine (C–C motif) ligand; CTGF: connective tissue growth factor; LOXL: lysyl oxidase-like. #: identifier numbers are provided for http://clinicaltrials.gov/

  • Table 3. Pharmacological treatments in idiopathic pulmonary fibrosis (IPF)
    Strong no recommendation
        Corticosteroid monotherapy
        Colchicine
        Cyclosporin A
        A combination of corticosteroids and immunomodulatory therapy
        Interferon-γ-1b
        Bosentan
        Etanercept
    Weak yes recommendation
        Corticosteroids in patients with acute exacerbation
        Treatment of asymptomatic gastro-oesophageal reflux
    Weak no recommendation
        Combination of prednisone, azathioprine and NAC: needs to be updated
        Monotherapy with NAC: further update pending
        Pirfenidone: needs to be updated
        Anticoagulants: needs to be updated
        PH associated with IPF: needs to be updated
    • NAC: N-acetyl-cysteine; PH: pulmonary hypertension.

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Pharmacological treatment of idiopathic pulmonary fibrosis: from the past to the future
Katerina M. Antoniou, George A. Margaritopoulos, Nikos M. Siafakas
European Respiratory Review Sep 2013, 22 (129) 281-291; DOI: 10.1183/09059180.00002113

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Pharmacological treatment of idiopathic pulmonary fibrosis: from the past to the future
Katerina M. Antoniou, George A. Margaritopoulos, Nikos M. Siafakas
European Respiratory Review Sep 2013, 22 (129) 281-291; DOI: 10.1183/09059180.00002113
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  • Article
    • Abstract
    • Introduction
    • Completed clinical trials
    • Ongoing clinical trials
    • Treatment of IPF: from 2000 to 2011 and beyond
    • Design of clinical trials
    • Conclusion
    • Footnotes
    • References
  • Figures & Data
  • Info & Metrics
  • PDF

Subjects

  • Interstitial and orphan lung disease
  • Pulmonary pharmacology and therapeutics
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More in this TOC Section

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