Safety and Efficacy of Pirfenidone in Advanced Idiopathic Pulmonary Fibrosis: A Nationwide Post-Marketing Surveillance Study in Korean Patients

Chung, Man Pyo; Park, Moo Suk; Oh, In Jae; Lee, Heung Bum; Kim, Young Whan; Uh, Soo Taek; Kim, Yun Seong; Jegal, Yangjin; Song, Jin Woo

doi:10.1007/s12325-020-01328-8

Cited by 28 publications

(27 citation statements)

References 31 publications

(63 reference statements)

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“…[4,5] The use of agents to prevent or reduce fibrotic status, such A preliminary version of this report was made publicly available as a socalled preprint at a nonprofit repository (Authorea) with DOI number: https://doi.org/10.22541/au.158802198.88770371 as pirfenidone, may be therapeutically effective in preventing serious or fatal complications. Pirfenidone is the drug of choice in the treatment of idiopathic pulmonary fibrosis (IPF) [6] and with a pleiotropic mechanism of action reduces the fibrotic and inflammatory state of lung tissue. Its therapeutic efficacy could also benefit healed patients with consequences on lung tissue with a state of fibrosis.…”

Section: Introductionmentioning

confidence: 99%

The added value of pirfenidone to fight inflammation and fibrotic state induced by SARS-CoV-2

Ferrara

Granata

Pelliccia

et al. 2020

Eur J Clin Pharmacol

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Aim SARS-CoV-2 infection has been divided by scientific opinion into three phases: the first as asymptomatic or slightly symptomatic and the second and the third with greater severity, characterized by a hyperinflammatory and fibrotic state, responsible for lung lesions, in some cases fatal. The development of antiviral drugs directed against SARS-CoV-2 and effective vaccines is progressing; meanwhile, the best pharmacological objective is related to the management of all the complications caused by this viral infection, mainly controlling the inflammatory and fibrotic state and preventing the infection from moving into the most serious phases. Subject and method Describe the scientific rationale related to the use of an antifibrotic therapy with pirfenidone, as monotherapy and/or in combination with anti-inflammatory drugs to manage and control complications of SARS-CoV-2 infection. Results Based on the scientific literature and epidemiological results and considering the pathophysiological, biological, and molecular characteristics of SARS-CoV-2, an antifibrotic drug such as pirfenidone as monotherapy or in combination with antiinflammatory drugs can be (acting early, at the right doses and at the right time) therapeutically effective to avoid serious complications during viral infection. The same approach can also be effective as postinfection therapy in patients with residual pulmonary fibrotic damage. Management of inflammation and fibrotic status with a combination therapy of pirfenidone and IL-6 or IL-1 inhibitors could represent a pharmacological synergy with added value. Conclusion In this article, we consider the role of antifibrotic therapy with pirfenidone in patients with SARS-CoV-2 infection on going or in the stage of postinfection with pulmonary fibrotic consequences. The scientific rationale for its use is also described.

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Section: Introductionmentioning

confidence: 99%

The added value of pirfenidone to fight inflammation and fibrotic state induced by SARS-CoV-2

Ferrara

Granata

Pelliccia

et al. 2020

Eur J Clin Pharmacol

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“…Patients with this degree of physiologic impairment have been excluded from clinical trials of antifibrotic therapies. Therefore there is a dearth of information about the clinical course, functional impairment as well as the safety and efficacy of antifibrotic therapy in this population, although mining of registry data or recent real-world papers have focused on this issue, providing encouraging results in this neglected population [10][11][12][13]21].…”

Section: Discussionmentioning

confidence: 99%

“…Similarly, patients who initially present with milder impairment but progress to more advanced disease despite treatment with antifibrotic therapy are often not candidates for enrollment in addon treatment trials. Despite some recent data in this population [10][11][12][13], there remains a large void of information regarding the efficacy of antifibrotic therapy in patients with severe disease.…”

Section: Introductionmentioning

confidence: 99%

Idiopathic pulmonary fibrosis patients with severe physiologic impairment: characteristics and outcomes

et al. 2021

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Research question There is no widely accepted grading system for IPF disease severity, although physiologic impairment based on pulmonary function testing is frequently employed. We sought to describe clinical and functional characteristics as well as outcomes of patients with severe physiologic impairment. Patients and methods IPF patients with severe physiologic impairment defined by FVC ≤ 50% and/or DLco ≤ 30% predicted evaluated in the Inova Advanced Lung Disease Program between 2011 and 2019 were included. Demographic, physiologic, functional treatment and outcome data were collated. Results There were 531 patients with IPF evaluated of whom 242 (46%) had severe physiologic impairment. Mean age was 72 ± 8 years; baseline FVC was 53 ± 17% and DLCO 28 ± 9% of predicted. The mean 6 min walks test (6MWT) distance was 304 ± 121 m with 59% of the patients requiring supplemental oxygen ($${\text{6MWT}}_{{{\text{O}}_{{2}} }}$$ 6MWT O 2 group). There was a poor correlation between the 6MWT distance and both FVC% and DLco%. Patients in the 6MWTRA group had a better transplant-free survival than the $${\text{6MWT}}_{{{\text{O}}_{{2}} }}$$ 6MWT O 2 group (p = 0.002). Patients managed before October 2014 and not receiving antifibrotic therapy had worse outcomes with reduced transplant-free survival compared with patients presenting after this date who did receive antifibrotic therapy (n = 113) (log rank p < 0.0001). Conclusion IPF patients often present with severe physiologic impairment which may be poorly correlated with their functional status. Assessment of IPF disease severity should not be based on physiologic impairment alone, but should also encompass functional status as well as need for supplemental oxygen. Antifibrotic therapy in patients with severe physiologic impairment is associated with improved outcomes.

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“…Studies have shown that bilateral interstitial pneumonia caused by COVID-19, so called because it attacks the tissue covering the lung alveoli, is associated with the presence of fibrotic tissue caused by excess collagen (fibrosis) in the lung crevice with associated hyperinflammation present. In this direction, the use of a pharmacological approach to reduce or prevent fibrotic status, with antifibrotic agents such as pirfenidone, used with demonstrated clinical efficacy in idiopathic pulmonary fibrosis [4] can be a valuable aid in the prevention of serious or fatal complications from COVID-19 in patients with ongoing infection, or in those already healed with residual fibrotic lung lesions [5].…”

mentioning

confidence: 99%

COVID-19 Patients with Pulmonary Fibrotic Tissue: Clinical Pharmacological Rational of Antifibrotic Therapy

Vitiello

Pelliccia

Ferrara

2020

SN Compr. Clin. Med.

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In December 2019, the first data emerged from Wuhan, China, of a serious acute respiratory disease caused by a new coronavirus, SARS-CoV-2 (COVID-19). In a short time, the health emergency became a global pandemic. To date, there are about 18.8 million infected people and about 700,000 deaths. There are currently no effective vaccines, and treatments are mostly experimental. The symptoms associated with COVID-19 are different, ranging from mild upper respiratory tract symptoms to severe acute respiratory distress syndrome (SARS). Data from previous coronavirus outbreaks such as SARS-CoV (2003 outbreak) and emerging epidemiological data from the current global COVID-19 pandemic suggest that there could be substantial tissue fibrotic consequences following SARS-CoV-2 infection, responsible for severe and in some cases fatal lung lesions. Some data show that even patients cured of viral infection have lung fibrotic tissue residues responsible for incorrect respiratory function even after healing. The role of antifibrotic drug therapy in patients with ongoing SARS-CoV-2 infection or in patients cured of residual pulmonary fibrosis is still to be defined and unclear; the scientific rationale for initiating, continuing, or discontinuing therapy is poorly defined. In this article, we describe the advantages of antifibrotic therapy in patients with ongoing SARS-CoV-2 viral infection to prevent the worsening and aggravation of the clinical situation, and the advantages it could have in the role of preventing pulmonary fibrosis after SARS-CoV-2 infection, and in accelerating the complete healing process.

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Safety and Efficacy of Pirfenidone in Advanced Idiopathic Pulmonary Fibrosis: A Nationwide Post-Marketing Surveillance Study in Korean Patients

Cited by 28 publications

References 31 publications

The added value of pirfenidone to fight inflammation and fibrotic state induced by SARS-CoV-2

The added value of pirfenidone to fight inflammation and fibrotic state induced by SARS-CoV-2

Idiopathic pulmonary fibrosis patients with severe physiologic impairment: characteristics and outcomes

COVID-19 Patients with Pulmonary Fibrotic Tissue: Clinical Pharmacological Rational of Antifibrotic Therapy

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