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Management of idiopathic pulmonary fibrosis: selected case reports

Michael Kreuter, Peter Kardos, Victor Hoffstein
European Respiratory Review 2014 23: 239-248; DOI: 10.1183/09059180.00002014
Michael Kreuter
1Dept of Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany. 2Group Practice and Respiratory, Allergology and Sleep Unit, Maingau Hospital, Frankfurt, Germany. 3Division of Respirology, St. Michael’s Hospital, Toronto, Canada
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  • For correspondence: kreuter@uni-heidelberg.de
Peter Kardos
1Dept of Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany. 2Group Practice and Respiratory, Allergology and Sleep Unit, Maingau Hospital, Frankfurt, Germany. 3Division of Respirology, St. Michael’s Hospital, Toronto, Canada
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Victor Hoffstein
1Dept of Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany. 2Group Practice and Respiratory, Allergology and Sleep Unit, Maingau Hospital, Frankfurt, Germany. 3Division of Respirology, St. Michael’s Hospital, Toronto, Canada
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Figures

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  • Figure 1.
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    Figure 1.

    Chest high-resolution computed tomography scans. Multi-slice computed tomography demonstrates a) apical emphysema and b) honeycombing and reticulation with subpleural, basal predominance. Extensive ground-glass opacities can be seen in both panels. Image courtesy of Claus P. Heussel (Thoraxklinik, University of Heidelberg, Heidelberg, Germany).

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    Figure 2.

    a) Low magnification histopathological biopsy showing typical features of usual interstitial pneumonia pattern with a heterogeneous appearance and areas of fibrosis with scarring and honeycomb change. Areas with less affected parenchyma also show emphysematous changes. b) Higher magnification of the histopathological biopsy reveals diffuse pulmonary involvement of numerous macrophage accumulations within most of the distal airspaces, consistent with a desquamative interstitial pneumonia pattern. Image courtesy of Philipp A. Schnabel (Institute of Pathology, University of Heidelberg, Heidelberg, Germany).

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    Figure 3.

    Forced vital capacity (FVC) over the course of the disease, some disease stabilisation can be seen following initiation of pirfenidone therapy. However, there was a significant decline in FVC due to neurological disease. After treatment for this disease the patient’s FVC increased again.

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    Figure 4.

    Chest high-resolution computed tomography scans performed on a) August 16, 2011 and b) September 4, 2012.

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    Figure 5.

    Initial high-resolution computed tomography scans performed in November 2011 showing mild reticulation and sub-pleural honeycombing. Representative sections of the lungs from a) the upper to f) the lower zones.

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    Figure 6.

    Progression in the patient’s pulmonary function tests from 2010 to 2013. a) Forced vital capacity (FVC); b) total lung capacity (TLC); c) diffusing capacity of the lung for carbon monoxide (DLCO); d) 6-min walking distance (6MWD); and e) oxygen saturation (arrow indicates patient was receiving supplemental oxygen). Start: oxygen saturation at the beginning of the 6MWD; end: oxygen saturation at the end of the 6MWD.

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    Figure 7.

    Patient computed tomography scans performed in a) 2010, b) 2012 and c) 2013. Representative sections of the lungs from the upper region to the lower region (left to right).

Tables

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  • Table 1. Pulmonary function tests
    ParameterAbsolute% Predicted
    FVC L2.581
    FEV1 L2.182
    FEV1/FVC %84106
    RV L2.2124
    TLC L4.794
    DLCO-SB36
    DLCO–VA54
    • FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; RV: residual volume; TLC: total lung capacity; DLCO: diffusing capacity of the lung for carbon monoxide; SB: single breath; VA: alveolar volume.

  • Table 2. Pulmonary function tests performed between 2000 and 2012#
    Lung functionDate of test
    Feb 1, 2000May 29, 2006Oct 31, 2011April 21, 2012Sept 15, 2012Oct 27, 2012
    VC % predicted10010180888888
    FVC % predicted10110380949194
    FEV1 % predicted959872939192
    FEV1/VC % predicted717165767467
    DLCO % predicted10076324251.2
    • VC: vital capacity; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; DLCO: diffusing capacity of the lung for carbon monoxide. #: the full dose of pirfenidone was started in November 2011.

  • Table 3. Initial pulmonary function tests performed in November 2011
    PredictedPatient
    NormalRangeMeasured% Predicted
    Lung volumes
        TLCpleth L6.44>5.074.61#72
        VC L4.13>3.302.97#72
        RV L2.30<2.991.6471
        RV/TLC35.8<46.935.6100
        FRC L2.2
    Spirometry
        FVC L4.13>3.302.86#69
        FEV1 L3.19>2.512.30#72
        FEV1/FVC %77.1>67.980.4
        FEF25–75 L·s−12.8>1.52.175
        FEFmax L·s−17.8>4.411.0142
        FIFmax L·s−13.7
        FEF50/FIF500.7
        MVV L·min−1124>9187#71
    Diffusing capacity
        DLCO-SB L·min−1·mmHg−125.3>17.315.4#61
        DLCO adjusted for Hb16.0 g·dL−114.8#59
        VA L6.23>5.073.82#61
    Oximetry
        Oxygen saturation %96≥9396
    Heart rate
        Pulse beats·min−170
    • TLCpleth: total lung capacity (TLC) measured by plethysmography; VC: vital capacity; RV: residual volume; FRC: functional residual capacity; FVC: functional vital capacity; FEV1: forced expiratory volume in 1 s; FEF25–75: forced expiratory flow at 25–75% of FVC; FEFmax: maximal forced expiratory flow; FIFmax: maximum forced inspiratory flow; FEF50: forced expiratory flow at 50% of FVC; FIF50: forced inspiratory flow at 50% of FVC; MVV: maximum voluntary ventilation; DLCO: diffusing capacity of the lung for carbon monoxide; SB: single breath; Hb: haemoglobin; VA: alveolar volume. #: outside normal limits.

Additional Files

  • Figures
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  • Disclosures

    Files in this Data Supplement:

    • P. Kardos
    • M. Kreuter
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Management of idiopathic pulmonary fibrosis: selected case reports
Michael Kreuter, Peter Kardos, Victor Hoffstein
European Respiratory Review Jun 2014, 23 (132) 239-248; DOI: 10.1183/09059180.00002014

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Management of idiopathic pulmonary fibrosis: selected case reports
Michael Kreuter, Peter Kardos, Victor Hoffstein
European Respiratory Review Jun 2014, 23 (132) 239-248; DOI: 10.1183/09059180.00002014
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