Pirfenidone: A Novel Agent for the Treatment of Idiopathic Pulmonary Fibrosis

Potts, Jason; Yogaratnam, Dinesh

doi:10.1345/aph.1r337

Cited by 22 publications

(17 citation statements)

References 17 publications

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“…Pirfenidone is a novel antifibrotic agent developed for the treatment of IPF and has been approved for use in Europe (Esbriet ® ) since 2011. It is supposed that pirfenidone regulates the activity of TGF‐β and TNF‐α leading to the inhibition of fibroblast proliferation and collagen synthesis . Adverse effects of pirfenidone include gastrointestinal and skin‐related reactions .…”

Section: Introductionmentioning

confidence: 99%

Pirfenidone‐induced severe phototoxic reaction in a patient with idiopathic lung fibrosis

Παπακωνσταντίνου

Prasse

Schacht

et al. 2016

Acad Dermatol Venereol

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Section: Introductionmentioning

confidence: 99%

Pirfenidone‐induced severe phototoxic reaction in a patient with idiopathic lung fibrosis

Παπακωνσταντίνου

Prasse

Schacht

et al. 2016

Acad Dermatol Venereol

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“…Pirfenidone is the only pharmacological agent approved for the treatment of mild-to-moderate IPF [11]. Nowadays, there is no drug available for the treatment of patients with IPF in an advanced stage.…”

Section: Introductionmentioning

confidence: 99%

Pirfenidone for the treatment of idiopathic pulmonary fibrosis

Xaubet

Serrano-Mollar

Ancochea

2013

Expert Opinion on Pharmacotherapy

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Pirfenidone is the only anti-fibrotic drug approved for the treatment of IPF. Pirfenidone provides a meaningful clinical effect on reductions in the decrease in forced vital capacity (FVC), six-minute walk test (6MWT) distance and mortality, and it improves the progression-free survival in IPF patients with mild-to-moderate disease. Pirfenidone is well tolerated, with the most common side-effects being gastrointestinal discomfort and photosensitivity. Pirfenidone has a favorable benefit-risk profile and represents a suitable treatment option for patients with mild-to-moderate IPF.

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“…The only currently approved drug for lung fibrosis is pirfenidone [76,77,78,79]. It is an orally active, small molecule that inhibits the synthesis of profibrotic and inflammatory mediators.…”

Section: Clinical Trials In Idiopathic Pulmonary Fibrosismentioning

confidence: 99%

New Therapeutic Avenues for Treatment of Fibrosis: Can We Learn from Other Diseases?

Rogler

2014

Dig Dis

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Crohn's disease (CD) is characterized by the frequent occurrence of complications, such as fibrotic strictures and subsequently the need for CD-related surgery. Chronic or recurrent inflammation is generally regarded to be a necessary precondition for the initiation of intestinal fibrosis. In this view, fibrosis is a pathologically augmented healing response to inflammation-induced mucosal tissue destruction and injury. At present, there are no approved or effective medical therapies aimed specifically at fibrosis or stricture in IBD. Indirect benefits may occur from anti-inflammatory therapies, although there is no consensus on this. Therapy for fibrosis is complicated by the fact that a wound-healing response is essential in CD and ulcerative colitis. Several pharmaceutical companies are now working on the therapy of fibrosis in other diseases. Strategies interfering with TGF-β expression and activation are promising. Pirfenidone has been studied in several clinical trials. Further therapeutic options are second-generation and wide-spectrum tyrosine kinase inhibitors. These inhibit growth factor receptor signaling, thus reducing fibrosis in animal models and some patients with tumor-associated fibrosis. At present, the development of antifibrotic therapies takes place in other diseases such as lung and liver fibrosis. This is partially due to a lack of experimental models for gut fibrosis and the fact that reliable readouts (MRI, serum markers) in patients are lacking. It will be important to test the above-mentioned newly available treatment strategies in IBD to profit from progress in other fibrotic diseases.

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Pirfenidone: A Novel Agent for the Treatment of Idiopathic Pulmonary Fibrosis

Abstract: Although pirfenidone appears to be an effective treatment for IPF, additional clinical trials are needed to better delineate its risk-benefit profile.

Cited by 22 publications

References 17 publications

Pirfenidone‐induced severe phototoxic reaction in a patient with idiopathic lung fibrosis

Pirfenidone‐induced severe phototoxic reaction in a patient with idiopathic lung fibrosis

Pirfenidone for the treatment of idiopathic pulmonary fibrosis

New Therapeutic Avenues for Treatment of Fibrosis: Can We Learn from Other Diseases?

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