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Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?

U. Costabel
European Respiratory Review 2011 20: 201-207; DOI: 10.1183/09059180.00002011
U. Costabel
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  • For correspondence: ulrich.costabel@ruhrlandklinik.uk-essen.de
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  • Figure 1.
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    Figure 1.

    Effects of N-acetylcysteine (▒) and placebo (░) on vital capacity (VC) and diffusing capacity of the lung for carbon monoxide (DL,CO) depending on baseline composite physiological index (BL-CPI) being lower or higher than 50 points. #: p = 0.0031; ¶: p = 0.0015; +: p = 0.067. Reproduced from [19] with permission from BioMed Central.

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    Figure 2.

    Effects of pirfenidone on vital capacity (VC) at week 52 in a Japanese phase III trial. The last observation carried forward method was used for drop-outs in each group. Data are presented as mean±se. Placebo group: n = 103; high-dose group: n = 104; low-dose group: n = 54. #: p<0.1, comparison of adjusted means based on ANCOVA (negative and positive of the changes represent deterioration and improvement from baseline, respectively). Reproduced from [31].

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    Figure 3.

    Change in forced vital capacity (FVC) % predicted (% pred) in study 004 and study 006 comparing pirfenidone or placebo in patients with idiopathic pulmonary fibrosis [29, 35]. ▪: study 004 pirfenidone 2,403 mg·day−1; •: study 006 pirfenidone 2,403 mg·day−1; ▴: study 006 placebo; ▾: study 004 placebo.

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    Figure 4.

    Temporal analysis of change in forced vital capacity per cent predicted with pirfenidone in patients with idiopathic pulmonary fibrosis [34].

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    Figure 5.

    Exploratory pooled analysis (studies 004 and 006 combined) of change in forced vital capacity (FVC) with pirfenidone 2,403 mg·day−1 (□: n = 345) or placebo (▪: n = 347) in patients with idiopathic pulmonary fibrosis [29, 35]. #: p = 0.005, rank ANCOVA (pirfenidone 2,403 mg·day−1 versus placebo at week 72).

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    Figure 6.

    Exploratory pooled analysis (studies 004 and 006 combined) of a) categorical forced vital capacity change at week 72 and b) 6-min walk test distance decrement >50 m (p = 0.03 Cochran–-Haenszel row mean score test based on five categories) [28, 31]. ▒: pirfenidone 2,403 mg·day−1 (n = 345); ░: placebo (n = 347). *: p<0.05; #: p = 0.003; ¶: p≤0.001.

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    Figure 7.

    Forest plot of pirfenidone versus placebo in improving progression-free survival in patients with idiopathic pulmonary fibrosis. Reproduced with modification from [36].

Tables

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  • Table 1. Selected recent clinical trials in idiopathic pulmonary fibrosis
    TrialDrug (putative MoA)Patients nEnd-pointOutcome
    IFIGENIANAC (anti-oxidant; plus prednisone and AZT)155ΔVC, ΔDL,COPositive
    NCT0063869Etanercept (TNF antagonist)88ΔFVC, ΔDL,CO, PA-a,O2Negative
    INSPIREIFN-γ-1b (antifibrotic)826SurvivalNegative
    BUILD-1Bosentan (endothelin inhibitor)1586MWTNegative
    BUILD-3Bosentan (endothelin inhibitor)600Progression-free survivalNegative
    NCT00131274Imatinib (tyrosin kinase inhibitor)119Disease progression or deathNegative
    STEP-IPFSildenafil (PD5 inhibitor)1806MWTNegative
    Shionogi phase IIIPirfenidone (antifibrotic)267ΔVCPositive
    PIPF 004 (CAPACITY 2)Pirfenidone (antifibrotic)435ΔFVC % predPositive
    PIPF 006 (CAPACITY 1)Pirfenidone (antifibrotic)344ΔFVC % predNegative
    • MoA: mechanism of action; NAC: N-acetylcysteine; AZT: azathioprine; TNF: tumour necrosis factor; IFN: interferon; PD5: phosphodiesterase-5; VC: vital capacity; DL,CO: diffusing capacity of the lung for carbon monoxide; FVC: forced vital capacity; PA–a,O2: alveolar–arterial oxygen tension difference; 6MWT: 6-min walk test; % pred: % predicted.

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Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?
U. Costabel
European Respiratory Review Sep 2011, 20 (121) 201-207; DOI: 10.1183/09059180.00002011

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Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?
U. Costabel
European Respiratory Review Sep 2011, 20 (121) 201-207; DOI: 10.1183/09059180.00002011
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  • Article
    • Abstract
    • PHARMACOLOGICAL THERAPIES FOR IPF: CURRENT STATUS
    • TRIPLE THERAPY WITH PREDNISONE, AZATHIOPRINE AND N-ACETYLCYSTEINE
    • PIRFENIDONE
    • JAPANESE PHASE II AND III TRIALS
    • STUDIES 004 AND 006: EUROPEAN AND NORTH AMERICAN PHASE III TRIALS
    • EXPLORATORY POOLED ANALYSIS OF STUDIES 004 AND 006
    • SAFETY AND TOLERABILITY OF PIRFENIDONE IN STUDIES 004 AND 006
    • META-ANALYSIS OF PHASE III STUDIES
    • PROGNOSTIC FACTORS FOR IPF AND ASSESSMENT OF NEW TREATMENT OPTIONS
    • SUMMARY
    • Acknowledgments
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