Last reviewed: June 2019
Last updated: June  2019

Summary

Definition

History and exam

Key diagnostic factors

  • dyspnea
  • cough
  • crackles

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
Full details

Investigations to consider

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

Treatment algorithm

Contributors

Authors VIEW ALL

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 Sanofi and Galapagos for serving as site principal investigator for clinical trials in IPF research.

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.

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.

Peer reviewers VIEW ALL

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.

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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