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Idiopathic Pulmonary Fibrosis: Phenotypes and Comorbidities

  • Christopher S. King
  • Shambhu Aryal
  • Steven D. NathanEmail author
Chapter
Part of the Respiratory Medicine book series (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.

Keywords

Pulmonary fibrosis Phenotype Pulmonary hypertension Pulmonary disease Chronic obstructive Connective tissue diseases Comorbidity Emphysema Gastroesophageal reflux disease Anxiety Depression Sleep disordered breathing 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Christopher S. King
    • 1
  • Shambhu Aryal
    • 2
  • Steven D. Nathan
    • 3
    Email author
  1. 1.Advanced Lung Disease and Transplant ClinicInova Fairfax HospitalFalls ChurchUSA
  2. 2.Internal MedicineInova Fairfax HospitalFalls ChurchUSA
  3. 3.Department of MedicineInova Fairfax HospitalFalls ChurchUSA

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