Diagnosis of Idiopathic Pulmonary Fibrosis
Diagnosis
Table 1. Comparison of ATS/ERS/JRS/ALAT Recommendations for the Diagnosis of IPF in the 2011 and 2018 Guidelines
BAL cellular analysis
We suggest performing BAL cellular analysis. (C, )
We suggest NOT performing BAL cellular analysis. (C, )
Surgical lung biopsy
We suggest performing surgical lung biopsy. (C, )
We recommend NOT performing surgical lung biopsy. (S, )
Transbronchial lung biopsy
No recommendation for or against (, )
We recommend NOT performing transbronchial lung biopsy. (S, )
Lung cryobiopsy
No recommendation for or against (, )
We recommend NOT performing cryobiopsy. (S, )
Medical history of medication use and environmental exposures
Diagnosis of IPF requires exclusion of other known causes of ILD (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity).
Serological testing to exclude connective tissue disease
Diagnosis of IPF requires exclusion of other known causes of ILD (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity).
Multidisciplinary discussion
We recommend that a multidisciplinary discussion should be used in the evaluation of IPF.
Serum biomarkers
Not addressed
Recommendation Grading
Overview
Title
Diagnosis of Idiopathic Pulmonary Fibrosis
Authoring Organizations
American Thoracic Society
European Respiratory Society
Publication Month/Year
September 3, 2018
Last Updated Month/Year
October 4, 2024
Supplemental Implementation Tools
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Target Patient Population
Adult patients with newly detected interstitial lung disease (ILD)
PICO Questions
Should Patients with Newly Detected ILD of Unknown Cause Who Are Clinically Suspected of Having IPF Undergo a Detailed, Prompted History of Medication Use and Environmental Exposures at Home, Work, and Other Places the Patient Frequently Visits to Exclude Potential Causes of the ILD?
Should Patients with Newly Detected ILD of Unknown Cause Who Are Clinically Suspected of Having IPF Undergo Serological Testing to Exclude Exclude CTDs as Potential Causes of the ILD?
Should Patients with Newly Detected ILD of Unknown Cause Who Are Clinically Suspected of Having IPF Undergo Cellular Analysis of Their BAL Fluid?
For Patients with Newly Detected ILD of Unknown Cause Who Are Clinically Suspected of Having IPF, Should SLB Be Performed to Ascertain the Histopathology Diagnosis of UIP Pattern?
For Patients with Newly Detected ILD of Unknown Cause Who Are Clinically Suspected of Having IPF, Is TBBx a Reasonable Alternative to SLB to Ascertain the Histopathology Diagnosis of UIP Pattern?
For Patients with Newly Detected ILD of Unknown Cause Who Are Clinically Suspected of Having IPF, Is Transbronchial Lung Cryobiopsy a Reasonable Alternative to SLB to Ascertain the Histopathology Diagnosis of UIP Pattern?
Should Patients with Newly Detected ILD of Unknown Cause Who Are Clinically Suspected of Having IPF Be the Subject of MDD for Decision-making?
Should Patients with Newly Detected ILD of Unknown Cause Who Are Clinically Suspected of Having IPF Undergo Serum Biomarker (MMP-7, SPD, CCL-18, KL-6) Measurement for the Purpose of Diagnosis?
Inclusion Criteria
Male, Female, Adult, Older adult
Health Care Settings
Outpatient, Radiology services
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Diagnosis, Assessment and screening
Keywords
interstitial lung disease, Idiopathic pulmonary fibrosis (IPF), IPF, ILD