Diagnosis of Idiopathic Pulmonary Fibrosis
Publication Date: September 3, 2018
Last Updated: December 15, 2022
Diagnosis
Table 1. Comparison of ATS/ERS/JRS/ALAT Recommendations for the Diagnosis of IPF in the 2011 and 2018 Guidelines
BAL cellular analysis
HRCT Pattern of Probable UIP, Indeterminate for UIP, and Alternative Diagnosis:
We suggest performing BAL cellular analysis. (C, )
We suggest performing BAL cellular analysis. (C, )
620
HRCT Pattern of UIP:
We suggest NOT performing BAL cellular analysis. (C, )
We suggest NOT performing BAL cellular analysis. (C, )
620
Surgical lung biopsy
HRCT Pattern of Probable UIP, Indeterminate for UIP, and Alternative Diagnosis:
We suggest performing surgical lung biopsy. (C, )
We suggest performing surgical lung biopsy. (C, )
620
HRCT Pattern of UIP:
We recommend NOT performing surgical lung biopsy. (S, )
We recommend NOT performing surgical lung biopsy. (S, )
620
Transbronchial lung biopsy
HRCT Pattern of Probable UIP, Indeterminate for UIP, and Alternative Diagnosis:
No recommendation for or against (, )
No recommendation for or against (, )
620
HRCT Pattern of UIP:
We recommend NOT performing transbronchial lung biopsy. (S, )
We recommend NOT performing transbronchial lung biopsy. (S, )
620
Lung cryobiopsy
HRCT Pattern of Probable UIP, Indeterminate for UIP, and Alternative Diagnosis:
No recommendation for or against (, )
No recommendation for or against (, )
620
HRCT Pattern of UIP:
We recommend NOT performing cryobiopsy. (S, )
We recommend NOT performing cryobiopsy. (S, )
620
Medical history of medication use and environmental exposures
We recommend taking a detailed history of both medication use and environmental exposures at home, work, and other places the patient frequently visits to exclude potential causes of ILD (motherhood statement). (, )
2011 Guideline: Did Not Distinguish among Patients with Different HRCT Patterns:
Diagnosis of IPF requires exclusion of other known causes of ILD (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity).
Diagnosis of IPF requires exclusion of other known causes of ILD (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity).
620
Serological testing to exclude connective tissue disease
We recommend serological testing to exclude connective tissue diseases as a potential cause of the ILD (motherhood statement). (, )
2011 Guideline: Did Not Distinguish among Patients with Different HRCT Patterns:
Diagnosis of IPF requires exclusion of other known causes of ILD (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity).
Diagnosis of IPF requires exclusion of other known causes of ILD (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity).
620
Multidisciplinary discussion
We suggest multidisciplinary discussion for decision-making. (C, )
2011 Guideline: Did Not Distinguish among Patients with Different HRCT Patterns:
We recommend that a multidisciplinary discussion should be used in the evaluation of IPF.
We recommend that a multidisciplinary discussion should be used in the evaluation of IPF.
620
Serum biomarkers
We recommend NOT measuring serum MMP-7, SPD, CCL-18, or KL-6 for the purpose of distinguishing IPF from other ILDs. (S, )
2011 Guideline: Did Not Distinguish among Patients with Different HRCT Patterns:
Not addressed
Not addressed
620
Overview
Title
Diagnosis of Idiopathic Pulmonary Fibrosis
Authoring Organizations
American Thoracic Society
European Respiratory Society