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Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena.

ABSTRACT: BACKGROUND:Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and has a median survival after diagnosis of 2-5?years. Pirfenidone is the first approved antifibrotic drug for the treatment of IPF. Here we report the functional progress, side effects and survival data of a population of patients with IPF, diagnosed at our centre and treated with pirfenidone. METHODS:We enrolled 91 patients with IPF (71 males) treated with pirfenidone. Clinical, survival and functional details were collected retrospectively at start of therapy and after 12, 24, 36 and 48?months of treatment. Lung function tests at least 12?months before starting therapy were available for 40 patients and were entered in the database, as well as side effects. RESULTS:During the observation period (922?±?529?days), 27 patients died, 5 patients underwent lung transplant and 10 patients interrupted therapy due to adverse events or IPF progression. The median survival was 1606?days. There was a significant reduction in disease progression rate, as measured by trend of forced vital capacity, after 1 year of therapy with respect to before treatment (p?=?0.0085). Forced vital capacity reduction rate was progressively higher in the subsequent years of treatment. Treatment-related side effects were reported in 25 patients and were predominantly mild. Overall, four patients discontinued therapy due to severe photosensitivity. CONCLUSIONS:Our findings confirm the efficacy of pirfenidone in reducing functional progression of IPF and its excellent safety profile in a real-life setting. This study, designed on a long-term follow up, contributes to the growing evidence on safety, tolerability and efficacy of pirfenidone in IPF. The reviews of this paper are available via the supplemental material section.

SUBMITTER: Vietri L

PROVIDER: S-EPMC7029533 | biostudies-literature | 2020 Jan-Dec

REPOSITORIES: biostudies-literature

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Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena.

Vietri Lucia L Cameli Paolo P Perruzza Marco M Cekorja Behar B Bergantini Laura L d'Alessandro Miriana M Refini Rosa Metella RM Pieroni Maria M Fossi Antonella A Bennett David D Spalletti Marco M Mazzei Maria Antonietta MA Sestini Piersante P Rottoli Paola P Bargagli Elena E

Therapeutic advances in respiratory disease 20200101

<h4>Background</h4>Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and has a median survival after diagnosis of 2-5 years. Pirfenidone is the first approved antifibrotic drug for the treatment of IPF. Here we report the functional progress, side effects and survival data of a population of patients with IPF, diagnosed at our centre and treated with pirfenidone.<h4>Methods</h4>We enrolled 91 patients with IPF (71 males) treated with pirfenidone. Clinical, ...[more]

PMID: 32066332

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Project description:BackgroundPirfenidone is the first antifibrotic drug approved for the treatment of idiopathic pulmonary fibrosis (IPF) and it is used in the treatment of other interstitial pneumonias, such as unclassifiable interstitial lung disease (ILD) and connective tissue-related ILD. This study examined the efficacy of pirfenidone in patients with IPF and fibrotic idiopathic non-specific interstitial pneumonia (f-iNSIP).MethodsIn a retrospective real-life study, 67 IPF and 24 f-iNSIP patients were enrolled and classified into a pirfenidone group and a non-antifibrotic group. The level of forced vital capacity (FVC) and diffusing capacity of lung for carbon monoxide (DLco) at baseline, 6, 12 and 24 months were recorded. The level of KL-6 in serum was detected by chemiluminescence enzyme immunoassay (CLEIA). The prognosis and safety outcomes were collected from patients.ResultsIn IPF patients, pirfenidone decreased the mean change of FVC and DLco, and decreased the proportion of patients with a ⩾10% decline in FVC or a ⩾15% decline in DLco compared with the non-antifibrotic group. There was no difference in the mean change of FVC and DLco between smokers and non-smokers who received pirfenidone treatment. There was an improvement in progression-free survival, defined as the time to the first occurrence of acute exacerbation or death related to pulmonary fibrosis. Moreover, the ratio of patients who experienced acute exacerbation and death related to pulmonary fibrosis was lower in the pirfenidone group. There was no change in lung function and prognosis between the pirfenidone and non-antifibrotic groups in f-iNSIP patients. The KL-6 level slightly decreased after 1 year of pirfenidone treatment but not significantly. Gastrointestinal and skin-related adverse events were most common, and four patients ceased treatment due to the side effects.ConclusionsPirfenidone safely reduced disease progression by improving the lung function and progression-free survival in IPF patients, with acceptable side effects. The efficacy of pirfenidone on f-iNSIP was not significant, suggesting the need for further studies.The reviews of this paper are available via the supplemental material section.

Dataset Information

Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena.

Publications

Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena.

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