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      Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis

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          Abstract

          Background

          The assessment of treatment response in idiopathic pulmonary fibrosis (IPF) is complicated by the variable clinical course. We examined the variability in the rate of disease progression and evaluated the effect of continued treatment with pirfenidone in patients who experienced meaningful progression during treatment.

          Methods

          The source population included patients enrolled in the ASCEND and CAPACITY trials (N=1247). Pearson's correlation coefficients were used to characterise the relationship between changes in FVC during consecutive 6-month intervals in the placebo population. Outcomes following a ≥10% decline in FVC were evaluated by comparing the proportion of patients in the pirfenidone and placebo groups who experienced a ≥10% decline in FVC or death during the subsequent 6 months.

          Results

          A weak negative correlation was observed between FVC changes during consecutive intervals in the placebo population (coefficient, −0.146, p<0.001), indicating substantial variability. Thirty-four (5.5%) and 68 (10.9%) patients in the pirfenidone and placebo groups, respectively, experienced a ≥10% decline in FVC by month 6. During the subsequent 6 months, fewer patients in the pirfenidone group compared with placebo experienced a ≥10% decline in FVC or death (5.9% vs 27.9%; relative difference, 78.9%). There was one (2.9%) death in the pirfenidone group and 14 (20.6%) deaths in the placebo group (relative difference, 85.7%).

          Conclusions

          Longitudinal FVC data from patients with IPF showed substantial intrasubject variability, underscoring the inability to reliably assess therapeutic response using serial FVC trends. In patients who progressed during treatment, continued treatment with pirfenidone resulted in a lower risk of subsequent FVC decline or death.

          Trial registration numbers

          NCT01366209, NCT00287729, NCT00287716.

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          The clinical course of patients with idiopathic pulmonary fibrosis.

          Fernando J Martínez, Sharon Safrin, Derek Weycker … (2005)
          Prospective data defining the clinical course in idiopathic pulmonary fibrosis (IPF) are sparse. To analyze the clinical course of patients with mild to moderate IPF. Analysis of data from the placebo group of a randomized, controlled trial evaluating interferon-gamma1b. Academic and community medical centers. 168 patients in the placebo group of a trial evaluating interferon-gamma1b. Measures of physiology and dyspnea assessed at 12-week intervals; hospitalizations; and the pace of deterioration and cause of death over a median period of 76 weeks. Physiologic variables changed minimally during the study. However, 23% of patients required hospitalization for a respiratory disorder and 21% died. Idiopathic pulmonary fibrosis was the primary cause of death in 89% of patients who died, and an apparent acute clinical deterioration preceded death in 47% of these patients. The instrument used to define the pace of deterioration and cause of death was applied retrospectively. Recognition of the common occurrence of acute fatal deterioration in patients with mild to moderate IPF has important implications for monitoring patients and supports early referral for lung transplantation.
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            Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis.

            Roland M du Bois, Derek Weycker, Carlo Albera … (2011)
            Several predictors of mortality in patients with idiopathic pulmonary fibrosis have been described; however, there is a need for a practical and accurate method of quantifying the prognosis of individual patients. Develop a practical mortality risk scoring system for patients with idiopathic pulmonary fibrosis. We used a Cox proportional hazards model and data from two clinical trials (n = 1,099) to identify independent predictors of 1-year mortality among patients with idiopathic pulmonary fibrosis. From the comprehensive model, an abbreviated clinical model comprised of only those predictors that are readily and reliably ascertained by clinicians was derived. Beta coefficients for each predictor were then used to develop a practical mortality risk scoring system. Independent predictors of mortality included age, respiratory hospitalization, percent predicted FVC, 24-week change in FVC, percent predicted carbon monoxide diffusing capacity, 24-week change in percent predicted carbon monoxide diffusing capacity, and 24-week change in health-related quality of life. An abbreviated clinical model comprising only four predictors (age, respiratory hospitalization, percent predicted FVC, and 24-wk change in FVC), and the corresponding risk scoring system produced estimates of 1-year mortality risk consistent with observed data (9.9% vs. 9.7%; C statistic = 0.75; 95% confidence interval, 0.71–0.79). The prognosis for patients with idiopathic pulmonary fibrosis may be accurately determined using four readily ascertainable predictors. Our simplified scoring system may be a valuable tool for determining prognosis and guiding clinical management. Additional research is needed to validate the applicability and accuracy of the scoring system.
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              Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia.

              Yangjin Jegal, Dong Soon Kim, Tae Sun Shim … (2005)
              The histopathologic pattern provides the most important prognostic marker for idiopathic interstitial pneumonia; however, studies have suggested that short-term changes in lung function may be more important. We investigated the prognostic factors for fibrotic interstitial pneumonia. The clinical features and follow-up course of 179 patients (131 with idiopathic pulmonary fibrosis and 48 with nonspecific interstitial pneumonia; 41 fibrotic types and 7 cellular) were analyzed retrospectively. The lung function indices improved or stabilized in most patients with fibrotic nonspecific interstitial pneumonia in contrast to the deterioration or stable condition of most patients with idiopathic pulmonary fibrosis. The 5-year survival of patients with fibrotic nonspecific interstitial pneumonia (76.2%) was better than for those with idiopathic pulmonary fibrosis (43.8%) (p = 0.007). Multivariate analysis at the time of presentation revealed that pathologic pattern, age, and diffusion capacity had important prognostic implications. However, after 6 months of follow-up, changes in FVC, initial diffusion capacity, and sex were the only independent prognostic factors, with no additional prognostic information conferred by the histologic diagnosis. Our data confirmed the importance of physiological parameters including short-term change in FVC. However, at the time of diagnosis, histopathology was important for the prediction of prognosis and future change in lung function.
              Article 0 comments Cited 71 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Thorax
                Journal ID (iso-abbrev): Thorax
                Journal ID (hwp): thoraxjnl
                Journal ID (publisher-id): thorax
                Title: Thorax
                Publisher: BMJ Publishing Group (BMA House, Tavistock Square, London, WC1H 9JR )
                ISSN (Print): 0040-6376
                ISSN (Electronic): 1468-3296
                Publication date (Print): May 2016
                Publication date (Electronic): 11 March 2016
                Volume: 71
                Issue: 5
                Pages: 429-435
                Affiliations
                [1 ]Inova Fairfax Hospital, Heart and Lung Transplant Center , Falls Church, Virginia, USA
                [2 ]Department of Clinical and Biological Sciences, University of Turin , Turin, Italy
                [3 ]InterMune Inc. , Brisbane, California, USA
                [4 ]Department of Pneumology/Allergy, Ruhrlandklinik , Essen, Germany
                [5 ]Imperial College , London, UK
                [6 ]Alfred Hospital and Monash University , Melbourne, Australia
                [7 ]University of Miami Miller School of Medicine , Miami, Florida, USA
                [8 ]University of California , San Francisco, California, USA
                [9 ]Vanderbilt University Medical Center , Nashville, Tennessee, USA
                [10 ]Columbia University Medical Center , New York, New York, USA
                [11 ]Paulista School of Medicine, Federal University of São Paulo , São Paulo, Brazil
                [12 ]Interstitial Lung Disease Program, National Jewish Health , Denver, Colorado, USA
                [13 ]Assistance Publique-Hôpitaux de Paris, Avicenne University Hospital , Bobigny, France
                [14 ]Cedars Sinai Medical Center , Los Angeles, California, USA
                [15 ]Royal Brompton Hospital , London, UK
                Author notes
                [Correspondence to ] Dr Steven D Nathan, Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, 3300 Gallows Road, Falls Church, VA 22042. USA; Steven.Nathan@ 123456inova.org
                Author information
                http://orcid.org/0000-0002-5167-7754
                Article
                Publisher ID: thoraxjnl-2015-207011
                DOI: 10.1136/thoraxjnl-2015-207011
                PMC ID: 4862066
                PubMed ID: 26968970
                SO-VID: 057fb168-ae9b-4236-9050-a9b265000077
                Copyright © Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
                License:

                This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

                History
                Date received : 4 March 2015
                Date revision received : 12 January 2016
                Date accepted : 13 February 2016
                Related
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                Subject: 1506
                Subject: Interstitial Lung Disease
                Series title: Original article
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                ScienceOpen disciplines: Surgery
                Keywords: idiopathic pulmonary fibrosis
                Data availability:
                ScienceOpen disciplines: Surgery
                Keywords: idiopathic pulmonary fibrosis

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