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      Importance of serial changes in biomarkers in idiopathic pulmonary fibrosis

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          Abstract

          The clinical significance of serial changes in serum biomarkers in patients with idiopathic pulmonary fibrosis (IPF) remains to be established. This retrospective study was conducted to clarify the associations of serial changes in serum Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) with changes in physiological indices and overall mortality in IPF.

          The study subjects were 75 patients with IPF. The 6 month change in serum KL-6 was significantly correlated with changes in the percentage of the predicted forced vital capacity (FVC % pred) and the percentage of the predicted diffusing capacity of the lung for carbon monoxide (% D LCO), while the 6 month change in serum SP-D was correlated only with % D LCO. During the mean follow-up period of 647 days, 22 (29.3%) patients died. An increase in serum KL-6 over a 6 month period was a significant predictor of mortality even after adjustment for %FVC, % D LCO and serum KL-6 at the baseline (hazard ratio 1.10 per 100 U·mL −1, 95% CI 1.01–1.18, p=0.03), whereas the 6 month increase in serum SP-D was not significant.

          Serial measurements of serum KL-6 may provide additional prognostic information compared to that provided by physiological parameters in patients with IPF.

          Abstract

          Serial changes in serum KL-6 are associated with changes in physiological variables and can predict survival in IPF http://ow.ly/hCkb30eauLg

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          A multidimensional index and staging system for idiopathic pulmonary fibrosis.

          Brett Ley, Christopher J Ryerson, Eric Vittinghoff … (2012)
          Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an overall poor prognosis. A simple-to-use staging system for IPF may improve prognostication, help guide management, and facilitate research. To develop a multidimensional prognostic staging system for IPF by using commonly measured clinical and physiologic variables. A clinical prediction model was developed and validated by using retrospective data from 3 large, geographically distinct cohorts. Interstitial lung disease referral centers in California, Minnesota, and Italy. 228 patients with IPF at the University of California, San Francisco (derivation cohort), and 330 patients at the Mayo Clinic and Morgagni-Pierantoni Hospital (validation cohort). The primary outcome was mortality, treating transplantation as a competing risk. Model discrimination was assessed by the c-index, and calibration was assessed by comparing predicted and observed cumulative mortality at 1, 2, and 3 years. Four variables were included in the final model: gender (G), age (A), and 2 lung physiology variables (P) (FVC and Dlco). A model using continuous predictors (GAP calculator) and a simple point-scoring system (GAP index) performed similarly in derivation (c-index of 70.8 and 69.3, respectively) and validation (c-index of 69.1 and 68.7, respectively). Three stages (stages I, II, and III) were identified based on the GAP index with 1-year mortality of 6%, 16%, and 39%, respectively. The GAP models performed similarly in pooled follow-up visits (c-index ≥71.9). Patients were drawn from academic centers and analyzed retrospectively. The GAP models use commonly measured clinical and physiologic variables to predict mortality in patients with IPF.
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            Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials

            Paul Noble, Carlo Albera, Williamson Bradford … (2016)
            Pirfenidone is an antifibrotic agent that has been evaluated in three multinational phase 3 trials in patients with idiopathic pulmonary fibrosis (IPF). We analysed pooled data from the multinational trials to obtain the most precise estimates of the magnitude of treatment effect on measures of disease progression. All patients randomised to pirfenidone 2403 mg·day−1 or placebo in the CAPACITY or ASCEND studies were included in the analysis. Pooled analyses of outcomes at 1 year were based on the pre-specified end-points and analytic methods described in the ASCEND study protocol. A total of 1247 patients were included in the analysis. At 1 year, pirfenidone reduced the proportion of patients with a ≥10% decline in per cent predicted forced vital capacity or death by 43.8% (95% CI 29.3–55.4%) and increased the proportion of patients with no decline by 59.3% (95% CI 29.0–96.8%). A treatment benefit was also observed for progression-free survival, 6-min walk distance and dyspnoea. Gastrointestinal and skin-related adverse events were more common in the pirfenidone group, but rarely led to discontinuation. Analysis of data from three phase 3 trials demonstrated that treatment with pirfenidone for 1 year resulted in clinically meaningful reductions in disease progression in patients with IPF.
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              Utility of KL-6/MUC1 in the clinical management of interstitial lung diseases.

              Nobuhisa Ishikawa, Noboru Hattori, Akihito Yokoyama … (2012)
              Interstitial lung diseases (ILDs) are a diverse group of pulmonary disorders characterized by various patterns of inflammation and fibrosis in the interstitium of the lung. Because injury and/or regeneration of type II pneumocytes are prominent histological features of ILDs, substances derived from type II pneumocytes have been the focus of research investigating potential biomarkers for ILD. One important biomarker for ILD is the high-molecular-weight glycoprotein, Krebs von den Lungen-6 (KL-6). KL-6 is now classified as a human MUC1 mucin protein, and regenerating type II pneumocytes are the primary cellular source of KL-6/MUC1 in the affected lungs of patients with ILD. KL-6/MUC1 is detectable in the serum of patients with ILD, and extensive investigations performed primarily in Japan have revealed that serum KL-6/MUC1 is elevated in 70-100% of patients with various ILDs, including idiopathic interstitial pneumonias, collagen vascular disease-associated interstitial pneumonia, hypersensitivity pneumonia, radiation pneumonitis, drug-induced ILDs, acute respiratory distress syndrome, pulmonary sarcoidosis, and pulmonary alveolar proteinosis. The results from these various studies have supported the utility of KL-6/MUC1 as a serum biomarker for detecting these various ILDs. Moreover, KL-6/MUC1 serum levels have been demonstrated to be useful for evaluating disease activity and predicting the clinical outcomes of various ILD types. Based on these observations, we believe that KL-6/MUC1 is currently one of the best and most reliable serum biomarkers available for ILD management. Copyright © 2012 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.
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                Author and article information

                Journal
                Journal ID (nlm-ta): ERJ Open Res
                Journal ID (iso-abbrev): ERJ Open Res
                Journal ID (publisher-id): ERJOR
                Journal ID (hwp): erjor
                Title: ERJ Open Research
                Publisher: European Respiratory Society
                ISSN (Electronic): 2312-0541
                Publication date Collection: July 2017
                Publication date (Electronic): 30 August 2017
                Volume: 3
                Issue: 3
                Electronic Location Identifier: 00019-2016
                Affiliations
                [1 ]Dept of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
                [2 ]Dept of Respiratory Care and Sleep Control Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
                [3 ]Dept of Health Informatics, School of Public Health, Kyoto University, Kyoto, Japan
                [4 ]Dept of Diagnostic Imaging and Nuclear Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
                [5 ]Kyoto Central Clinic, Clinical Research Center, Kyoto, Japan
                Author notes
                Kiminobu Tanizawa, Dept of Respiratory Care and Sleep Control Medicine, Graduate School of Medicine, Kyoto University, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto 606-8507, Japan. E-mail: tanizawa@ 123456kuhp.kyoto-u.ac.jp
                Article
                Publisher ID: 00019-2016
                DOI: 10.1183/23120541.00019-2016
                PMC ID: 5576222
                SO-VID: c43ba405-489b-41ef-bfeb-92a00d63f934
                Copyright © Copyright ©ERS 2017
                License:

                This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

                History
                Date received : 10 February 2016
                Date accepted : 01 July 2017
                Funding
                Funded by: Japan Society for the Promotion of Science http://doi.org/10.13039/501100001691
                Award ID: KAKENHI Grant-in-Aid for Young Scientists (B)
                Funded by: Japan’s Ministry of Health, Labor, and Welfare
                Award ID: the Research Program of Intractable Disease
                Award ID: the Diffuse Lung Diseases Research Group
                Award ID: the Respiratory Failure Study Group
                Funded by: Japan Intractable Diseases Research Foundation
                Categories
                Subject: Original Articles
                Subject: 13

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