Skip to main content

Main menu

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Open access
    • COVID-19 submission information
    • Peer reviewer login
  • Alerts
  • Podcasts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Open access
    • COVID-19 submission information
    • Peer reviewer login
  • Alerts
  • Podcasts
  • Subscriptions

Diagnosing idiopathic pulmonary fibrosis in 2018: bridging recommendations made by experts serving different societies

Luca Richeldi, Kevin C. Wilson, Ganesh Raghu
European Respiratory Journal 2018 52: 1801485; DOI: 10.1183/13993003.01485-2018
Luca Richeldi
1Fondazione Policlinico A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Kevin C. Wilson
2Dept of Medicine, Boston University School of Medicine, Boston, MA, USA
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ganesh Raghu
3Dept of Medicine, University of Washington, Seattle, WA, USA
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • PDF
Loading

Abstract

Two new documents on the diagnosis of IPF have been published in 2018: their similarities and differences are described and discussed http://ow.ly/856P30lnB2O

Two guidance documents for the diagnosis of idiopathic pulmonary fibrosis (IPF) have been recently published by international experts representing major respiratory and radiological societies [1, 2]. Similar documents from other societies are anticipated. This is clearly a positive sign of the increasing attention being paid by the scientific community to a group of fibrotic lung diseases of which IPF is the prototype.

Clinical practice guidelines (CPGs) guide clinicians in the management of patients with a specific disease, based upon evolving knowledge and evidence. They are expected to adhere to Institute of Medicine standards [3] which include, for every recommendation, a full systematic review of the evidence performed by methodologists who have neither financial nor intellectual conflicts, a discussion of the synthesised evidence by content experts whose potential conflicts of interest are carefully managed, and the formulation of recommendations based upon consideration of the balance of benefits versus harms and burdens, quality of evidence, patient values and preferences, cost, and feasibility.

CPGs impact at least three important domains. First, they inform clinical practice, where the diagnostic approach needs to be standardised based on the most up-to-date evidence generated by clinical research. Second, they harmonise the population of patients enrolled in different multicentre randomised trials. This helps regulatory agencies accurately assess the safety and the efficacy of new treatments in a defined spectrum of patients. Last, but not least, they support single patients and patient advocacy groups/associations to better understand the nature of diseases like IPF, a rare condition for which a large portion of the information available on the internet is inaccurate [4].

Consensus statements similarly guide clinical management; however, recommendations are based on the consensus of content experts whose opinions reflect their knowledge and experience. Consensus statements do not adhere to the same robust methodology as CPGs and, therefore, usually do not have the same impact as CPGs in terms of acceptance and implementation by policy makers, regulating agencies, and stakeholders. Despite their different methods, CPGs and consensus statements have many similarities. Both involve a large number of multidisciplinary experts in the field; both are sponsored, developed, endorsed and published by scientific societies; and both answer specific clinical questions with recommendations (table 1). Given such similarities, it is not surprising that the guidance provided by CPGs and consensus statements are often similar and overlapping.

View this table:
  • View inline
  • View popup
TABLE 1

Diagnosis of idiopathic pulmonary fibrosis (IPF): similarities and differences between the 2018 ATS/ERS/JRS/ALAT clinical practice guideline and the 2018 Fleischner white paper

This is highlighted by two recent publications on the diagnosis of IPF: the American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/Latin American Thoracic Society (ALAT) guidelines on the diagnosis of IPF [1] and the Fleischner Society consensus statement on diagnostic criteria for IPF [2].

The ATS/ERS/JRS/ALAT guidelines and Fleischner Society statement reach similar conclusions and recommendations (table 2), with only minimal semantic differences. In particular, three components are nearly identical. First, both documents acknowledge the critical role of the clinician in evaluating patients suspected to have IPF. To make an accurate diagnosis, clinicians must consider the clinical context at presentation (e.g. the age of the patient), eliminate the possibility of significant environmental or medication exposures, and exclude the presence of connective tissue disease. Second, both documents emphasise the critical importance of high-quality, high-resolution computed tomography (HRCT) images in the evaluation of patients suspected to have IPF. This is illustrated by the documents' detailed descriptions of the technical aspects needed to generate adequate images of the lungs from volumetric scanning of the chest. Third, both documents conclude that discussions among experienced experts from multiple disciplines (i.e. “multidisciplinary discussions”) are necessary to make an accurate diagnosis of IPF. Multidisciplinary discussions should include the clinician, a radiologist and, when histopathology is available, a pathologist. A rheumatologist should also be included on a case-by-case basis.

View this table:
  • View inline
  • View popup
TABLE 2

Diagnostic components for idiopathic pulmonary fibrosis (IPF)

Additional similarities exist. Both documents acknowledge surgical biopsy as the gold standard for obtaining a tissue sample. The ATS/ERS/JRS/ALAT guidelines do not make a recommendation for or against transbronchial cryobiopsy due to a paucity of evidence and insufficient agreement among the expert panel, while the Fleischner Society statement describes transbronchial cryobiopsy as a procedure with an unclear role in clinical practice. Both documents have eliminated the previous category of “possible usual interstitial pneumonia (UIP)” pattern and provide refined features for the UIP pattern, probable UIP pattern, indeterminate for UIP pattern, and patterns that sway away from the diagnosis of UIP and are indicative of an alternative diagnosis. Finally, both documents conclude that, for many individual patients, a definite diagnosis cannot be made, but a highly probable working diagnosis can be achieved with the multidisciplinary discussion.

The only apparent substantial dissimilarity between the two documents is related to the need for a surgical lung biopsy in patients with a probable UIP pattern on HRCT. While the ATS/ERS/JRS/ALAT guidelines recommend surgical lung biopsy in patients with a probable UIP pattern on HRCT, the Fleischner Society statement indicates that a confident diagnosis of IPF can be made without surgical lung biopsy in patients with a consistent clinical context and a probable UIP pattern on HRCT. It is important to note, however, that the ATS/ERS/JRS/ALAT recommendation is a conditional recommendation. This indicates that the guideline panel concluded that biopsy is appropriate for a majority of patients (≥50%), but may not be appropriate for a sizeable minority (up to 49%) of patients; in other words, the guidelines indicate that there is clinical equipoise when deciding whether or not to biopsy a patient with a probable UIP pattern on HRCT. When the clinical context is strongly suggestive of IPF, patients are likely fall into the sizeable minority for whom a biopsy is unnecessary and the recommendation becomes essentially identical to the Fleischner Society recommendation. In other words, the ATS/ERS/JRS/ALAT guidelines and the Fleischner Society Statement recommend the same course of action for patients with both a high clinical likelihood of IPF and a probable UIP pattern on HRCT, but the ATS/ERS/JRS/ALAT recommendation can also be applied to patients for whom the clinical likelihood of IPF is uncertain, providing greater flexibility. Among the 21 expert members of the ATS/ERS/JRS/ALAT guidelines panel, 17 supported a conditional recommendation for biopsy, while only four members supported a conditional recommendation against biopsy.

Given the similarities in the recommendations made by these two official documents on the diagnosis of IPF, it is likely that following the algorithm provided in the ATS/ERS/JRS/ALAT guidelines will yield the same diagnosis as following the guidance provided by the Fleischner Society statement for any given patient suspected of having IPF. Nevertheless, the CPGs for clinical management of IPF have advanced from a consensus-based statement in 2000 to become evidence-based from 2011 onwards [5, 6].

It is hoped that implementation of the new diagnostic criteria and approach by all stakeholders and the IPF community-at-large worldwide will yield accurate diagnoses for patients and, thus, appropriate therapeutic interventions can be initiated promptly to improve outcomes that are clinically meaningful for the patient with IPF.

Footnotes

  • Conflict of interest: L. Richeldi reports personal fees from Sanofi-Aventis (consultancy), Roche (member of advisory board), Celgene (consultancy), Nitto (consultancy), Fibrogen (member of advisory board), Promedior (member of advisory board), Bristol Myers Squibb (consultancy), DynaMed (editorial activity), Pliant Therapeutics (consultancy), Prometic (consultancy), Asahi Kasei (consultancy) and Biogen (consultancy), and grants and personal fees from Boehringer Ingelheim (member of Steering Committee), all outside the submitted work.

  • Conflict of interest: K.C. Wilson reports being the American Thoracic Society Chief of Documents and Patient Education; therefore, he may have an intellectual bias in favour of ATS-sponsored documents.

  • Conflict of interest: G. Raghu reports other (consultant for IPF studies) from BI, BMS, Biogen, Bellerophan, Fibrogen, Gilead, Nitto, Promedior, Sanofi and Veracyte, and grants from NIH (for IPF studies), all outside the submitted work.

  • Received August 4, 2018.
  • Accepted August 6, 2018.
  • Copyright ©ERS 2018

References

  1. ↵
    1. Raghu G,
    2. Remy-Jardin M,
    3. Myers JL, et al.
    Diagnosis of Idiopathic Pulmonary Fibrosis: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2018; in press [https://doi.org/10.1164/rccm.201807-1255ST].
  2. ↵
    1. Lynch DA,
    2. Sverzellati N,
    3. Travis WD, et al.
    Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med 2018; 6: 138–153.
    OpenUrl
  3. ↵
    1. IOM (Institute of Medicine)
    . Clinical Practice Guidelines We Can Trust. Washington, The National Academies Press, 2011.
  4. ↵
    1. Fisher JH,
    2. O'Connor D,
    3. Flexman AM, et al.
    Accuracy and reliability of internet resources for information on idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2016; 194: 218–225.
    OpenUrl
  5. ↵
    1. Raghu G
    . Idiopathic pulmonary fibrosis: guidelines for clinical management have advanced from consensus-based in 2000 to evidence-based in 2011. Eur Respir J 2011; 37: 743–746.
    OpenUrlFREE Full Text
  6. ↵
    1. Raghu G,
    2. Rochwerg B,
    3. Zhang Y, et al.
    An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: treatment of idiopathic pulmonary fibrosis. An update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med 2015; 192: e3–e19.
    OpenUrlCrossRefPubMed
PreviousNext
Back to top
View this article with LENS
Vol 52 Issue 3 Table of Contents
European Respiratory Journal: 52 (3)
  • Table of Contents
  • Index by author
Email

Thank you for your interest in spreading the word on European Respiratory Society .

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Diagnosing idiopathic pulmonary fibrosis in 2018: bridging recommendations made by experts serving different societies
(Your Name) has sent you a message from European Respiratory Society
(Your Name) thought you would like to see the European Respiratory Society web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Print
Citation Tools
Diagnosing idiopathic pulmonary fibrosis in 2018: bridging recommendations made by experts serving different societies
Luca Richeldi, Kevin C. Wilson, Ganesh Raghu
European Respiratory Journal Sep 2018, 52 (3) 1801485; DOI: 10.1183/13993003.01485-2018

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero

Share
Diagnosing idiopathic pulmonary fibrosis in 2018: bridging recommendations made by experts serving different societies
Luca Richeldi, Kevin C. Wilson, Ganesh Raghu
European Respiratory Journal Sep 2018, 52 (3) 1801485; DOI: 10.1183/13993003.01485-2018
del.icio.us logo Digg logo Reddit logo Technorati logo Twitter logo CiteULike logo Connotea logo Facebook logo Google logo Mendeley logo
Full Text (PDF)

Jump To

  • Article
    • Abstract
    • Footnotes
    • References
  • Figures & Data
  • Info & Metrics
  • PDF
  • Tweet Widget
  • Facebook Like
  • Google Plus One

More in this TOC Section

  • Addressing the effect of ancestry on lung volume
  • Consensus statement on quality standards for managing children with bronchiectasis
  • Bronchodilators in bronchiectasis: there is light but it is still too dim
Show more Editorials

Related Articles

Navigate

  • Home
  • Current issue
  • Archive

About the ERJ

  • Journal information
  • Editorial board
  • Reviewers
  • Press
  • Permissions and reprints
  • Advertising

The European Respiratory Society

  • Society home
  • myERS
  • Privacy policy
  • Accessibility

ERS publications

  • European Respiratory Journal
  • ERJ Open Research
  • European Respiratory Review
  • Breathe
  • ERS books online
  • ERS Bookshop

Help

  • Feedback

For authors

  • Instructions for authors
  • Publication ethics and malpractice
  • Submit a manuscript

For readers

  • Alerts
  • Subjects
  • Podcasts
  • RSS

Subscriptions

  • Accessing the ERS publications

Contact us

European Respiratory Society
442 Glossop Road
Sheffield S10 2PX
United Kingdom
Tel: +44 114 2672860
Email: journals@ersnet.org

ISSN

Print ISSN:  0903-1936
Online ISSN: 1399-3003

Copyright © 2022 by the European Respiratory Society