Idiopathic pulmonary fibrosis

Last reviewed: 7 Jun 2022
Last updated: 04 Nov 2021

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • dyspnoea
  • cough
  • crackles
More key diagnostic factors

Other diagnostic factors

  • weight loss, fatigue, and malaise
  • clubbing
Other diagnostic factors

Risk factors

  • advanced age
  • male sex
  • family history
  • cigarette smoking
  • organic and inorganic dust exposure
  • gastro-oesophageal reflux
  • infection
  • diabetes
More risk factors

Diagnostic investigations

1st investigations to order

  • CXR
  • high-resolution CT (HRCT)
  • anti-nuclear antibodies (by immunofluorescence)
  • rheumatoid factor
  • anti-cyclic citrullinated peptide
  • myositis panel
More 1st investigations to order

Investigations to consider

  • pulmonary function tests
  • surgical lung biopsy
  • bronchoalveolar lavage (BAL)
  • trans-bronchial biopsy
  • C-reactive protein (CRP)
  • erythrocyte sedimentation rate (ESR)
More investigations to consider

Treatment algorithm

ACUTE

acute exacerbation

ONGOING

all patients not currently experiencing acute exacerbation

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. JDF amd JCM 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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