Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial

Prasse, Antje; Schönfeld, Nicolas; Behr, Jürgen; Kreuter, Michael; Johow, Johannes; Rabe, Klaus F.; Bonella, Francesco; Bonnet, R.; Grohé, Christian; Held, Matthias; Wilkens, Heinrike; Hammerl, Peter; Koschel, Dirk; Blaas, Stefan; Wirtz, Hubert; Ficker, Joachim H.; Neumeister, Wolfgang; Schönfeld, N; Claussen, Martin; Kneidinger, Nikolaus; Frankenberger, Marion; Kahn, Nicolas; Tello, Silke; Freise, Julia; Welte, Tobias; Neuser, Petra; Günther, Andreas; Schade‐Brittinger, Carmen; Aminossadati, Behnaz; Nasemann, Christian; Yahiaoui, Soraya; Backofen, Christoph Dupuy; Hahmann, Maik; Wittenberg, Michael; Drakopanagiotakis, Fotios; Beck, Daniel von der; Ghofrani, Stefanie; Heinemann, S; Krauss, Ekaterina; Rethorn, Hella; Koch, Andrea; Leuschner, Gabriela; Matthes, Sandhya; Neurohr, Claus; Veit, Tobias; Milger, Katrin; Herth, Felix J.F.; Benstz, Julia; Hummler, Simone; Bahmer, Thomas; Biller, Heike; Apel, Rosa-Marie; Costabel, Ulrich; Börner, Eva; Wessendorf, Thomas; Arnrich, M.; Ilie, Lacramioara; Wald, Alexandra; Seyfarth, Hans-Jürgen; Reinhardt, Christian; Cinar, Atif; Vogler, Markus; Huhn, Siegfried Matthias; Richter, Jaqueline; Neff, U; Blum, Torsten; Vesenbeckh, Silvan; Boch, Christian; Semper, Hannes; Wilke, Andreas; Pfeifer, Michael; Schweda, Annette; Krill, Angelika; Lensch, Christian; Joa, F; Schröder, Barbara; Plaßmeier, Annabel; Baron, Stefan; Froehling, Klaus Peter; Waschki, Benjamin

doi:10.1016/s2213-2600(20)30554-3

Cited by 320 publications

(253 citation statements)

References 16 publications

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“…According to the RELIEF study, inpatients with fibrotic ILDs other than IPF (such as connective tissue disease-associated ILDs, fibrotic non-specific interstitial pneumonia, chronic hypersensitivity pneumonitis and pneumoconiosis) attended in 17 centers specialized in ILD in Germany deteriorated despite conventional therapy. However, with the addition of pirfenidone to the existing treatment there was an attenuation of disease progression, as measured by decline in FVC [ 34 ]. Nevertheless, more studies for assessing the efficacy and safety of pirfenidone in this kind of patients are needed.…”

Section: Pirfenidonementioning

confidence: 99%

Pharmacological Interactions of Nintedanib and Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis in Times of COVID-19 Pandemic

et al. 2021

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The discovery of antifibrotic agents have resulted in advances in the therapeutic management of idiopathic pulmonary fibrosis (IPF). Currently, nintedanib and pirfenidone have become the basis of IPF therapy based on the results of large randomized clinical trials showing their safety and efficacy in reducing disease advancement. However, the goal of completely halting disease progress has not been reached yet. Administering nintedanib with add-on pirfenidone is supposed to enhance the therapeutic benefit by simultaneously acting on two different pathogenic pathways. All this becomes more important in the context of the ongoing global pandemic of coronavirus disease 2019 (COVID-19) because of the fibrotic consequences following SARS-CoV-2 infection in some patients. However, little information is available about their drug–drug interaction, which is important mainly in polymedicated patients. The aim of this review is to describe the current management of progressive fibrosing interstitial lung diseases (PF-ILDs) in general and of IPF in particular, focusing on the pharmacokinetic drug-drug interactions between these two drugs and their relationship with other medications in patients with IPF.

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Section: Pirfenidonementioning

confidence: 99%

Pharmacological Interactions of Nintedanib and Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis in Times of COVID-19 Pandemic

et al. 2021

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“…Disease outcomes remained similar to placebo and there were more treatment-emergent adverse events seen in the two-week vs. four-week titration group [ 25 , 45 ]. The RELIEF trial was a multi-center, double-blinded prospective trial in Germany evaluating the use of pirfenidone in a group of 127 PF-ILD patients with underlying etiologies including CTD-ILD, cHP, iNSIP, and asbestos-related fibrotic lung disease [ 39 , 40 ]. These patients were noted to have progression of disease even with conventional therapy, and antifibrotics were being considered due to disease progression.…”

Section: Pirfenidonementioning

confidence: 99%

“…After 48 weeks, the pirfenidone group showed a significantly lower decline in FVC percent predicted compared to placebo. Although prematurely terminated due to slow recruitment, the results of this trial again suggest that in patients with PF-ILD with progressive disease despite conventional therapy, pirfenidone therapy can attenuate further decrement in FVC and disease progression [ 25 , 39 , 40 ].…”

Section: Pirfenidonementioning

confidence: 99%

Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

Shumar

Chandel

King

2021

JCM

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Progressive fibrosing interstitial lung disease (PF-ILD) describes a phenotypic subset of interstitial lung diseases characterized by progressive, intractable lung fibrosis. PF-ILD is separate from, but has radiographic, histopathologic, and clinical similarities to idiopathic pulmonary fibrosis. Two antifibrotic medications, nintedanib and pirfenidone, have been approved for use in patients with idiopathic pulmonary fibrosis. Recently completed randomized controlled trials have demonstrated the clinical efficacy of antifibrotic therapy in patients with PF-ILD. The validation of efficacy of antifibrotic therapy in PF-ILD has changed the treatment landscape for all of the fibrotic lung diseases, providing a new treatment pathway and opening the door for combined antifibrotic and immunosuppressant drug therapy to address both the fibrotic and inflammatory components of ILD characterized by mixed pathophysiologic pathways.

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“…Für Pirfenidon, einer weiteren antifibrotisch wirkenden Substanz, liegen nunmehr ebenfalls Wirksamkeitsdaten bei fibrosierenden Lungenerkrankungen außerhalb der IPF vor [41].…”

Section: Antifibrotikaunclassified

Lunge – Interstitielle Lungenerkrankungen in der Rheumatologie

Bastian

Krause

2021

Aktuelle Rheumatologie

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ZusammenfassungInterstitielle Lungenerkrankungen (ILD) gehören zu den häufigsten Organmanifestationen bei entzündlich-rheumatischen Systemerkrankungen. Sie können sich bei einer Vielzahl rheumatischer Erkrankungen manifestieren, insbesondere bei rheumatoider Arthritis, systemischer Sklerose und idiopathischen inflammatorischen Myopathien. ILD sind mit einer erhöhten Morbidität und Mortalität assoziiert. Eine sorgfältige Diagnostik unter Einbeziehung von Klinik, apparativer und Labordiagnostik ist zur Klassifikation und Risikostratifizierung der zugrundeliegenden Erkrankung und vorliegenden Lungenbeteiligung unabdingbar. Auch wenn weiterhin nicht alle ätiologischen und pathogenetischen Details bekannt sind, haben die letzten Jahre einige Fortschritte in Verständnis und Management der (CTD-) ILD ergeben.

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Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial

Cited by 320 publications

References 16 publications

Pharmacological Interactions of Nintedanib and Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis in Times of COVID-19 Pandemic

Pharmacological Interactions of Nintedanib and Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis in Times of COVID-19 Pandemic

Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

Lunge – Interstitielle Lungenerkrankungen in der Rheumatologie

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