Guidelines Summary

The 2015 guidelines on idiopathic pulmonary fibrosis (IPF) by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Association include the following points^{[77, 78]}:

The guidelines recommend against the use of the following agents for the treatment of idiopathic pulmonary fibrosis: anticoagulation (warfarin); imatinib, a selective tyrosine kinase inhibitor against platelet-derived growth factor (PDGF) receptors; combination prednisone, azathioprine, and N-acetylcysteine; and selective endothelin receptor antagonist (ambrisentan).
The guidelines also give a conditional recommendation against the use of sildenafil, macitentan, and bosentan.
The panel has continued the 2011 conditional recommendation against the use of N-acetylcysteine monotherapy for IPF. They have also continued the 2011 conditional recommendation for the use of antacid therapy in patients without symptoms of GER.
The recommendation for the use of the following agents for the treatment of idiopathic pulmonary fibrosis is conditional: nintedanib, a tyrosine kinase inhibitor that targets multiple tyrosine kinases, including vascular endothelial growth factor, fibroblast growth factor, and PDGF receptors; and pirfenidone.

The clinical practice guidelines on the diagnosis of idiopathic pulmonary fibrosis were released on September 1, 2018, also by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society.^{[3, 79]}The collaborative guidelines outline basic recommendations for clinical observation, HRCT scanning, bronchoscopy, histopathology, and serum biomarker measurements, as follows:

Obtain a detailed history for possible environmental exposures or medication use to exclude potential causes of interstitial lung disease (ILD).
Perform serologic testing to exclude connective-tissue disease.
With newly diagnosed ILD, perform HRCT scanning of the chest; determine if findings indicate a pattern of (1) usual interstitial pneumonia (UIP), (2) probable UIP, (3) indeterminate of UIP, or (4) alternate diagnosis.
For probable UIP, indeterminate findings, or alternate diagnosis, bronchoalveolar lavage and surgical lung biopsy are recommended; evidence is insufficient to recommend for or against transbronchial lung biopsy or lung cryobiopsy.
For a UIP pattern, bronchoalveolar lavage (cellular analysis) and surgical lung biopsy are not recommended (risks outweigh benefits); guidelines strongly recommend against transbronchial lung biopsy or lung cryobiopsy in this situation.
Multidisciplinary discussions (MDDs) should include interactions between pulmonologists, possibly rheumatologists (on a case-by-case basis), pathologists, and radiologists. MDDs are recommended with newly detected ILD of an unknown cause in patients whose findings clinically suggest idiopathic pulmonary fibrosis. MDDs likely provide the greatest benefit in situations in which HRCT patterns indicate probable UIP, indeterminate findings, or alternative diagnoses, or when clinical, histologic, or radiologic data are incongruous.
For serum biomarker measurements, measuring serum matrix metalloproteinase 7, chemokine ligand 18, Krebs von den Lungen-6, or surfactant protein D is not recommended solely to distinguish idiopathic pulmonary fibrosis from other ILDs, owing to high false-positive and false-negative results.

Medication

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Media Gallery

Chest radiograph of a patient with idiopathic pulmonary fibrosis showing bilateral lower lobe reticular opacities (red circles).
Classic subpleural honeycombing (red circle) in a patient with a diagnosis of idiopathic pulmonary fibrosis.
A patient with IPF and a confirmed histologic diagnosis of usual interstitial pneumonia. Note the reticular opacities (red circle) distributed in both lung bases and the minimal ground-glass opacities (blue circle).
A patient with nonspecific interstitial pneumonia. Note the predominance of ground-glass opacities (blue circles) and a few reticular lines (red arrow).
Patchwork distribution of abnormalities in a classic example of usual interstitial pneumonia (low-magnification photomicrograph; hematoxylin and eosin stain; original magnification, X4). Courtesy of Chad Stone, MD.
High-resolution CT coronal view showing lower lobe predominant honeycombing (blue arrow) with irregular septal thickening and traction bronchiectasis compatible with typical usual interstitial pneumonia pattern.

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Author

Alaa Abu Sayf, MD Senior Staff Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Hospital, Wayne State University School of Medicine

Alaa Abu Sayf, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel R Ouellette, MD, FCCP Associate Professor of Medicine, Wayne State University School of Medicine; Medical Director, Pulmonary Medicine General Practice Unit (F2), Senior Staff and Attending Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Hospital

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, Society of Critical Care Medicine

Disclosure: Received research grant from: Sanofi Pharmaceutical.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Guy W Soo Hoo, MD, MPH Professor of Clinical Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Medical Intensive Care Unit, Chief, Pulmonary, Critical Care and Sleep Section, West Los Angeles VA Healthcare Center, Veteran Affairs Greater Los Angeles Healthcare System

Guy W Soo Hoo, MD, MPH is a member of the following medical societies: American Association for Respiratory Care, American College of Chest Physicians, American College of Physicians, American Thoracic Society, California Thoracic Society, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP Thomas H Davis Chair in Pulmonary Medicine, Chief, Section on Pulmonary, Critical Care, Allergy and Immunologic Diseases, Professor of Internal Medicine, Pediatrics, and Translational Science, Associate Director, Center for Genomics and Personalized Medicine Research, Wake Forest University School of Medicine; Executive Director of the Respiratory Service Line, Wake Forest Baptist Medical Center

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society, Sigma Xi, The Scientific Research Honor Society

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Integrity CE, Merck<br/>Received income in an amount equal to or greater than $250 from: – Array Biopharma, AstraZeneca, Aerocrine, Airsonett AB, Boehringer-Ingelheim, Experts in Asthma, Gilead, GlaxoSmithKline, Merck, Novartis, Ono Pharmaceuticals, Pfizer, PPD Development, Quintiles, Sunovion, Saatchi & Saatichi, Targacept, TEVA, Theron.

Amanda M K Godfrey, MD Associate Staff, Department of Internal Medicine, IHA Pulmonary, Critical Care, and Sleep Consultants; Associate Staff, Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, St Joseph Mercy Ann Arbor

Amanda M K Godfrey, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Medical Association, American Thoracic Society, Michigan State Medical Society

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Rajesh G. Patel, MD, and Javier I. Diaz, MD, to the development and writing of this article.

Sections Idiopathic Pulmonary Fibrosis (IPF)
Overview
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	Predictor	Points
Sex	Female	0
Sex	Male	1
Age (years)	≥60	0
	61-65	1
	>65	2
FVC (% predicted)	>75	0
	50-75	1
	< 50	2
DLCO (% predicted)	>55	0
	36-55	1
	≤35	2
	Cannot perform	3

Stage	I	II	III
Points	0-3	4-5	6-8
Mortality
1-year	5.6	16.2	39.2
2-year	10.9	29.9	62.1
3-year	16.3	42.1	76.8

Idiopathic Pulmonary Fibrosis (IPF) Guidelines

Guidelines Summary

References

Tables

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