Last reviewed: 1 Nov 2021
Last updated: 04 Nov 2021

Summary

Definition

History and exam

Other diagnostic factors

  • weight loss, fatigue, and malaise
  • clubbing

Risk factors

  • advanced age
  • male sex
  • family history
  • cigarette smoking
  • organic and inorganic dust exposure
  • gastroesophageal reflux
  • infection
  • diabetes

Diagnostic investigations

1st investigations to order

  • CXR
  • high-resolution CT (HRCT)
  • antinuclear antibodies (by immunofluorescence)
  • rheumatoid factor
  • anti-cyclic citrullinated peptide
  • myositis panel

Investigations to consider

  • pulmonary function tests
  • surgical lung biopsy
  • bronchoalveolar lavage (BAL)
  • transbronchial biopsy
  • C-reactive protein (CRP)
  • erythrocyte sedimentation rate (ESR)

Treatment algorithm

Contributors

Authors

Mary Elizabeth Kreider, MD, MS

Associate Professor of Clinical Medicine

Pulmonary, Allergy, and Critical Care Division

Perelman School of Medicine

University of Pennsylvania

Philadelphia

PA

Disclosures

MEK has received research support from Galapagos for serving as site principal investigator for clinical trials in IPF research.

Jake G. Natalini, MD

Fellow

Pulmonary, Allergy, and Critical Care Division

Perelman School of Medicine

University of Pennsylvania

Philadelphia

PA

Disclosures

JGN declares that he has no competing interests.

Acknowledgements

Dr Mary Elizabeth Kreider and Dr Jake G. Natalini would like to gratefully acknowledge Dr Judd David Flesch, Dr Gregory Tino, and Dr Jeffrey C. Munson, previous contributors to this topic.

Disclosures

GT has served as a consultant for InterMune. GT has served as a principal investigator for clinical trials in idiopathic pulmonary fibrosis. JCM and JDF declare that they have no competing interests.

Peer reviewers

Stephen Nathan, MD

Medical Director

Lung Transplant & Advanced Lung Disease Program

Inova Fairfax Hospital

Falls Church

VA

Disclosures

SN is an author of a reference cited in this monograph.

Athol Wells, MD, FRCP

Professor of Respiratory Medicine

Interstitial Lung Disease Unit

Royal Brompton Hospital

London

UK

Disclosures

AW declares that he has no competing interests.

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