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Idiopathic Pulmonary Fibrosis: Phenotypes and Comorbidities

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Idiopathic Pulmonary Fibrosis

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

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic disorder characterized by heterogeneity in its presentation and clinical course. Clinicians have attempted to delineate distinct clinical phenotypes within this disorder as a means to provide additional diagnostic, prognostic, or treatment information. Traditionally, phenotypes have been recognized based upon clinical features, physiologic testing, radiographic appearance, or disease course. It is believed that in the future, phenotyping will be supplanted by molecular endotyping, which incorporates genetic, metabolic, transcriptional, and environmental factors to provide insight into the underlying molecular pathways leading to fibrosis. Molecular endotyping holds promise to enhance early diagnosis of IPF, provide more detailed prognostic information, and allow for individualization of treatment. This chapter will discuss the various IPF phenotypes and their unique distinguishing features. Patients with IPF are at risk for a number of comorbidities that can negatively affect quality of life, functional status, and survival including gastroesophageal reflux disease, venous thromboembolism, and cardiac disease. This chapter will also discuss the prevalence, impact, and recommended management strategies of comorbidities that are frequently encountered in patients with IPF.

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Author information

Authors and Affiliations

  1. Advanced Lung Disease and Transplant Clinic, Inova Fairfax Hospital, Falls Church, VA, USA

    Christopher S. King

  2. Internal Medicine, Inova Fairfax Hospital, Falls Church, VA, USA

    Shambhu Aryal

  3. Department of Medicine, Inova Fairfax Hospital, Falls Church, VA, USA

    Steven D. Nathan

Authors
  1. Christopher S. King
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  2. Shambhu Aryal
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  3. Steven D. Nathan
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Corresponding author

Correspondence to Steven D. Nathan .

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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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King, C.S., Aryal, S., Nathan, S.D. (2019). Idiopathic Pulmonary Fibrosis: Phenotypes and Comorbidities. In: Meyer, K., Nathan, S. (eds) Idiopathic Pulmonary Fibrosis. Respiratory Medicine. Humana Press, Cham. https://doi.org/10.1007/978-3-319-99975-3_11

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  • DOI: https://doi.org/10.1007/978-3-319-99975-3_11

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  • Online ISBN: 978-3-319-99975-3

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