Pirfenidone for acute exacerbation of idiopathic pulmonary fibrosis: A retrospective study

Furuya, Kenta; Sakamoto, Susumu; Shimizu, Hiroshige; Sekiya, Muneyuki; Kinoshita, Arisa; Isshiki, Takuma; Sugino, Keishi; Matsumoto, Keiko; Homma, Sakae

doi:10.1016/j.rmed.2017.03.026

Cited by 36 publications

(23 citation statements)

References 23 publications

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“…Under this technique, weights were the inverse of (1 − propensity score) for patients receiving rhTM (rhTM arm) and the inverse of the propensity score for patients not receiving rhTM (control arm). The propensity score was estimated using multivariable logistic regression analysis including the seven most clinically relevant variables known to be possible confounders: IPF/non‐IPF, disease severity (mild/severe), prednisolone administration and anti‐fibrotic therapy using pirfenidone and/or nintedanib in the stable state, HRCT pattern (diffuse/non‐diffuse) , PaO 2 /FiO 2 ratio (≤200/>200) and the level of Krebs von den Lungen (KL)‐6 at AE. Adjusted hazard ratios (HR) with 95% CI were calculated.…”

Section: Methodsmentioning

confidence: 99%

Recombinant thrombomodulin for acute exacerbation in idiopathic interstitial pneumonias

et al. 2019

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Background and objective: Acute exacerbation (AE) in idiopathic pulmonary fibrosis (IPF) or other idiopathic interstitial pneumonias (IIP) is a poor prognostic event despite conventional therapy with corticosteroids and/or immunosuppressants. We aimed to evaluate the efficacy and safety of recombinant human soluble thrombomodulin (rhTM) for AE-IIP. Methods: For this prospective single-arm open-label multicentre cohort study, we retrospectively registered 61 cases of AE-IIP treated with conventional therapy between 2011 and 2013 (control arm), and prospectively enrolled 39 cases of AE-IIP treated with conventional therapy and rhTM (380 U/kg/day for 6 days) between 2014 and 2016 (rhTM arm). To reduce potential confounding in treatment comparisons, an adjusted mortality analysis for 90-day survival was conducted with weighted Cox proportional hazards regression models using inverse probability of treatment weighting. Weights were derived from propensity scores estimated using a multivariable logistic regression analysis including potential confounders. Results: The 90-day survival rates of AE-IIP patients treated with/without rhTM were 66.7% (26/39) and 47.5% (29/61), respectively. After adjusting for imbalances, rhTM therapy was significantly associated with reduced mortality (adjusted hazard ratio (HR): 0.453; 95% CI: 0.237-0.864; P = 0.0163). The frequencies of adverse events with/without rhTM were 17.9% (7/39) and 19.7% (12/61), which were similar in both arms (P = 1.0). Two bleeding-related adverse events occurred in the rhTM arm. Conclusion: Safety and efficacy were observed for rhTM treatment of AE-IIP. A future randomized controlled trial is required to draw final conclusions.Clinical trial registration: UMIN000014969 at www.umin. ac.jp

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Section: Methodsmentioning

confidence: 99%

Recombinant thrombomodulin for acute exacerbation in idiopathic interstitial pneumonias

et al. 2019

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“…Previous retrospective studies have described the potential benefit of immunosuppressive therapies for AE‐IPF, including cyclosporine A, tacrolimus and rituximab, plasma exchange and intravenous immunoglobulin . Meanwhile, the survival benefit of adding non‐immunosuppressive therapies, including polymyxin‐B‐immobilized fibre column haemoperfusion, recombinant thrombomodulin, azithromycin and pirfenidone has also been reported, although high‐dose CS was the mainstay therapy in almost all these studies. Interestingly, a recent retrospective study by Papiris et al has shown that, by applying the non‐steroid approach, including supportive care and appropriate antimicrobials, half of the patients with AE‐IPF survived .…”

Section: Discussionmentioning

confidence: 99%

Efficacy of corticosteroid and intravenous cyclophosphamide in acute exacerbation of idiopathic pulmonary fibrosis: A propensity score‐matched analysis

et al. 2019

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Background and objective: Acute exacerbation (AE) is a leading cause of death in patients with idiopathic pulmonary fibrosis (IPF). Although optimal treatment for AE-IPF remains unclear, high-dose corticosteroids (CS) with/without immunosuppressants, including intravenous cyclophosphamide (IVCY), are often used as empirical therapy. However, the survival benefit of adding IVCY to CS therapy is unknown. We investigated the efficacy of this therapy in patients with AE-IPF. Methods: Overall, 102 consecutive patients with IPF with a first idiopathic AE were included. Post-AE survival rates and treatment safety were retrospectively assessed. Efficacy of CS + IVCY therapy for the first AE was compared with that of CS monotherapy using a propensity score-matched analysis. Results: The post-AE 90-day survival rate of the entire cohort was 64.7%. On the basis of the propensity scores, 26 matched patient pairs were made. Characteristics of matched patients with AE-IPF treated with CS (matched CS group) and those with CS + IVCY (matched CS + IVCY group) were well balanced. No significant between-group differences were observed in post-AE 90-day survival rates (84.6% vs 76.9%; P = 0.70), cumulative survival rates (P = 0.57 by log-rank test) or incidence of adverse events ≥ CTCAE (Common Terminology Criteria for Adverse Events) v5.0 grade 3 (61.5% vs 65.4%; P = 1.00). Conclusion: The propensity score-matched analysis demonstrated that compared with CS monotherapy, CS + IVCY therapy did not significantly improve post-AE survival in patients with AE-IPF. Further studies are warranted to assess the efficacy of CS + IVCY therapy for AE-IPF.A propensity score-matched analysis demonstrated that in patients with idiopathic pulmonary fibrosis with a first episode of idiopathic acute exacerbation, compared with corticosteroids (CS) monotherapy, CS plus intravenous cyclophosphamide (IVCY) therapy did not significantly improve their survival. This suggests that routine use of IVCY with CS is not recommended.

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“…Furuya et al found that among 47 cases of AE-IPF (all treated with high dose corticosteroid and some with NAC, CTX/CsA or rhTM), the 3 monthsurvival rate was significantly better in 20 patients receiving pirfenidone than in 27 patients not receiving pirfenidone (55% vs. 34%, P ¼ 0.042). Among the patients receiving rhTM (N ¼ 22), pirfenidone did not make significant difference in the outcome (80% vs. 42%, P ¼ 0.067) (Furuya et al, 2017). In univariate analysis, nonuse of rhTM was a potential risk factor for death (hazard ratio [HR], 3.717; P ¼ 0.035).…”

Section: Antifibrotic Drugsmentioning

confidence: 98%

Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Kim

2022

Encyclopedia of Respiratory Medicine

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Pirfenidone for acute exacerbation of idiopathic pulmonary fibrosis: A retrospective study

Abstract: A regimen of pirfenidone combined with corticosteroids and rhTM may improve survival in patients with AE-IPF.

Cited by 36 publications

References 23 publications

Recombinant thrombomodulin for acute exacerbation in idiopathic interstitial pneumonias

Recombinant thrombomodulin for acute exacerbation in idiopathic interstitial pneumonias

Efficacy of corticosteroid and intravenous cyclophosphamide in acute exacerbation of idiopathic pulmonary fibrosis: A propensity score‐matched analysis

Acute Exacerbation of Idiopathic Pulmonary Fibrosis

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