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Pulmonary Function Tests in Idiopathic Pulmonary Fibrosis

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Idiopathic Pulmonary Fibrosis

Part of the book series: Respiratory Medicine ((RM))

Abstract

Pulmonary function tests are typically impaired in IPF, although patients in early disease stage can show lung volumes in normal range. The decreased lung compliance caused by tissue stiffness leads to a restrictive ventilatory defect, while ventilation and perfusion abnormalities are associated with a reduction of lung diffusion for carbon monoxide (DLco). The consequence of these volume and diffusion alterations is a progressive gas exchange impairment, which translates into increasing shortness of breath as a classical IPF symptom. Based on its well-established correlation with mortality, forced vital capacity (FVC) is the best biomarker we have to stratify IPF patients according to lung function. However, the concomitant presence of emphysema and the consequent hyperinflation of the lung makes FVC interpretation challenging. Pulmonary hypertension, another frequent complication of IPF, can cause a marked reduction of DLco even when lung volumes are still well preserved. Decline in FVC over time is currently used as a primary endpoint in clinical trials in IPF alone or in combination with other biomarkers. Daily home spirometry is a promising approach and may allow the separation of rapid from slow progressors and, likely, identify those patients prone to develop acute exacerbations.

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Author information

Authors and Affiliations

  1. Interstitial and Rare Lung Disease Unit, Department of Pulmonary Medicine, Ruhrlandklinik University Hospital, Essen, Germany

    Francesco Bonella

  2. Respiratory Unit, Ospedale San Paolo, Department of Health Science, Università degli Studi di Milano, Milan, Italy

    Fabiano di Marco

  3. Section of Respiratory Diseases, Department of Cardiac, Thoracic, and Vascular Sciences, University of Padua, Padua, Italy

    Paolo Spagnolo

Authors
  1. Francesco Bonella
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  2. Fabiano di Marco
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  3. Paolo Spagnolo
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Corresponding author

Correspondence to Francesco Bonella .

Editor information

Editors and Affiliations

  1. Department of Internal Medicine, Section of Allergy, Pulmonary and Critical Care Medicine, University of Wisconsin School of Medicine & Public Health, Madison, WI, USA

    Keith C. Meyer

  2. Advanced Lung Disease and Lung Transplant Program, Inova Fairfax Hospital, Falls Church, VA, USA

    Steven D. Nathan

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Bonella, F., di Marco, F., Spagnolo, P. (2019). Pulmonary Function Tests in Idiopathic Pulmonary Fibrosis. In: Meyer, K., Nathan, S. (eds) Idiopathic Pulmonary Fibrosis. Respiratory Medicine. Humana Press, Cham. https://doi.org/10.1007/978-3-319-99975-3_5

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  • DOI: https://doi.org/10.1007/978-3-319-99975-3_5

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  • Publisher Name: Humana Press, Cham

  • Print ISBN: 978-3-319-99974-6

  • Online ISBN: 978-3-319-99975-3

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