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      Pirfenidone treatment of idiopathic pulmonary fibrosis

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          Abstract

          Idiopathic pulmonary fibrosis (IPF) is a discrete clinicopathologic entity defined by the presence of usual interstitial pneumonia on high-resolution CT scan and/or open lung biopsy and the absence of an alternate diagnosis or exposure explaining these findings. There are currently no FDA-approved therapies available to treat this disease, and the 5-year mortality is ∼80%. The pyridone derivative pirfenidone has been studied extensively as a possible therapeutic agent for use in this deadly disease. This review will present the unique clinical features and management issues encountered by physicians caring for IPF patients, including the poor response to conventional therapy. The biochemistry and preclinical efficacy of pirfenidone will be discussed along with a comprehensive review of the clinical efficacy, safety, and side effects and patient-centered foci such as quality of life and tolerability. It is hoped that this information will lend insight into the complex issues surrounding the use of pirfenidone in IPF and lead to further investigation of this agent as a possible therapy in this devastating disease.

          Most cited references15

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          Pulmonary function testing in idiopathic interstitial pneumonias.

          David Flaherty, Alma E. Martínez (2006)
          Diffuse parenchymal lung diseases are a group of disorders that involve the space between the epithelial and endothelial basement membranes and are generally segregated into four major categories. These include the idiopathic interstitial pneumonias, which are further categorized into seven clinical/radiologic/pathologic subsets. These disorders generally share a common pattern of physiologic abnormality characterized by a restrictive ventilatory defect and reduced diffusing capacity (DLCO). Pulmonary function testing is often used and recommended in their assessment and management. The potential clinical application of physiologic testing includes to aid in diagnosis, although its value in differential diagnosis is limited. Pulmonary function testing also aids in establishing disease severity and in defining prognosis. In nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis, severely decreased DLCO has proven valuable in this regard. Similarly, exertional desaturation to less than 88% at baseline testing and a decrease in FVC (greater than 10%) over the course of short-term follow-up identify patients at particular risk of mortality. Finally, physiologic testing, especially spirometry and DLCO, have demonstrated value in monitoring response to therapy and identifying disease progression.
          Article 0 comments Cited 35 times – based on 0 reviews     Review now
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            Pirfenidone prevents collagen accumulation in the remnant kidney in rats with partial nephrectomy.

            S Margolin, S Hata, S Yamauchi … (1997)
            Pirfenidone (PFD) is a new compound that prevents and even reverses the extracellular matrix accumulation in several organs as shown by experimental and clinical studies. In the present study, we examined the effect of PFD (500 mg/kg daily in the food) on the progression of chronic renal failure (CRF) in the 5/6 nephrectomy rat model. Proteinuria progressively increased in rats with renal ablation (C) at 12 weeks. Urinary protein excretion in PFD-treated rats (P) was numerically lower than C, but the difference did not reach statistical significance. In contrast, in the chronic phase, PFD improved renal function and reduced collagen accumulation detected by hydroxyproline content (OH-Pro) in the cortex of the remnant kidney. Although creatinine clearance decreased with time in C, the values in P were significantly better at 10 and 12 weeks. The OH-Pro in C at 12 weeks was significantly higher than that of no-ablation, sham-operated rats, whereas OH-Pro in CRF was lower in (P). Expression of mRNA for type IV and I collagen in the cortex also increased in C, but it was inhibited in (P). To study the role that TGF-beta plays in the regulatory process following CRF, we examined the expression of TGF-beta mRNA in this model. Levels of cortical TGF-beta mRNA in C were significantly elevated at 12 weeks. The increase was suppressed by PFD. These results demonstrate that PFD attenuates the development of CRF by preventing collagen accumulation in this model, and suggest that PFD can be clinically useful for treating CRF.
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              Potential functional and survival benefit of double over single lung transplantation for selected patients with idiopathic pulmonary fibrosis.

              Lorenz Frey, Werner Leuschner, Rainer Baumgartner … (2010)
              Idiopathic pulmonary fibrosis (IPF) is a frequent indication for lung transplantation (LTX) with pulmonary hypertension (PH) negatively affecting outcome. The optimal procedure type remains a debated topic. The aim of this study was to evaluate the impact of pretransplant PH in IPF patients. Single LTX (SLTX, n = 46) was the standard procedure type. Double LTX (DLTX, n = 30) was only performed in cases of relevant PH or additional suppurative lung disease. There was no significant difference for pretransplant clinical parameters. Preoperative mean pulmonary arterial pressure was significantly higher in DLTX recipients (22.7 +/- 0.8 mmHg vs. 35.9 +/- 1.8 mmHg, P < 0.001). After transplantation, 6-min-walk distance and BEST-FEV(1) were significantly higher for DLTX patients (6-MWD: 410 +/- 25 m vs. 498 +/- 23 m, P = 0.02; BEST-FEV(1): 71.2 +/- 3.0 (% pred) vs. 86.2 +/- 4.2 (% pred), P = 0.004). Double LTX recipients demonstrated a significantly better 1-year-, overall- and Bronchiolitis obliterans Syndrome (BOS)-free survival (P < 0.05). Cox regression analysis confirmed SLTX to be a significant predictor for death and BOS. Single LTX offers acceptable survival rates for IPF patients. Double LTX provides a significant benefit in selected recipients. Our data warrant further trials of SLTX versus DLTX stratifying for potential confounders including PH.
              Article 0 comments Cited 10 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Ther Clin Risk Manag
                Journal ID (publisher-id): Therapeutics and Clinical Risk Management
                Title: Therapeutics and Clinical Risk Management
                Publisher: Dove Medical Press
                ISSN (Print): 1176-6336
                ISSN (Electronic): 1178-203X
                Publication date Collection: 2011
                Publication date (Print): 2011
                Publication date (Electronic): 08 February 2011
                Volume: 7
                Pages: 39-47
                Affiliations
                [1 ]Department of Medicine, Central South University, Changsha, Hunan, China;
                [2 ]Department of Medicine, Yale University School of Medicine, New Haven, CT, USA;
                [3 ]Department of Biology, Texas A&M University, College Station, TX, USA
                Author notes
                Correspondence: Richard H Gomer, Department of Biology, Texas A&M University, ILSB MS 3474, College Station, TX 77843-3474, USA, Tel +1 979 458 5745, Fax +1 979 845 2891, Email rgomer@ 123456tamu.edu
                Article
                Publisher ID: tcrm-7-039
                DOI: 10.2147/TCRM.S12209
                PMC ID: 3039013
                PubMed ID: 21339942
                SO-VID: 84536c3f-8a81-44fa-aa20-1148fec4fef5
                Copyright © © 2011 Gan et al, publisher and licensee Dove Medical Press Ltd.
                License:

                This is an Open Access article which permits unrestricted noncommercial use, provided the original work is properly cited.

                History
                Date received : 8 February 2011
                Categories
                Subject: Review

                ScienceOpen disciplines: Medicine
                Keywords: pirfenidone,fibrosis,clinical trials
                Data availability:
                ScienceOpen disciplines: Medicine
                Keywords: pirfenidone, fibrosis, clinical trials

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