Diagnosis of Idiopathic Pulmonary Fibrosis

Publication Date: September 3, 2018

Key Points

Key Points

Adult patients with newly detected interstitial lung disease (ILD) of apparently unknown cause are clinically suspected of having idiopathic pulmonary fibrosis (IPF) if they have unexplained symptomatic or asymptomatic patterns of bilateral fibrosis on a chest radiograph or chest computed tomography (CT) scan, bibasilar inspiratory crackles, and an age typically older than 60 years.

Rarely, middle-aged adults (>40 yr and <60 yr), especially those with risks for familial pulmonary fibrosis, may otherwise manifest the same clinical scenario as the typical patient older than 60 years.

The recommendations in this guideline are for the patterns and distributions of images obtained by high-resolution CT (HRCT) imaging and, thus, require that patients be subjected to HRCT of the chest for evaluation.

Diagnosis

...agnosi...

...c Criteria for IPF Exclusion of other known...


...son of ATS/ERS/JRS/ALAT Recommendations...

BAL cellular analys...

...n of Probable UIP, Indeterminate for UIP, and...

...ern of UIP: We suggest NOT performing B...

...rgical lung biopsy

...RCT Pattern of Probable UIP, Indeter...

HRCT Pattern of UIP: We recommen...

...nsbronchial lung bio...

...f Probable UIP, Indeterminate for UIP,...

...T Pattern of UIP: We recommend NO...

...cryobiopsy...

...f Probable UIP, Indeterminate for UIP, and Altern...

...UIP: We recommend NOT performing...

...l history of medication use and environmental exp...

...ological testing to exclude connective tiss...

...idisciplinary discussionWe suggest mu...

Serum biomarkersWe recommend NOT measuring...


...esolution CT Scanning ParametersHa...


...igh-Resolution CT Scanning Pattern...


...igh-Resolution CT Images Demonstrating a Usual Int...


...Probable UIP Pattern(A–C) Transverse compu...


...e 3. Indeterminate for UIP Pattern (earl...


...determinate for UIP Pattern ...


...T Pattern Suggestive of an Alternative Diagnosis...


...te Exacerbation of IPF(A and B) Transverse...


.... Histopathology Demonstrating UIP(A) Low-magnific...


...stopathology Patterns and Features Having t...


...Diagnosis Based Upon HRCT and Biop...


...igure 9. Diagnostic Algori...