Summary
Definition
History and exam
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
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.
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
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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