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

...agnosis...

...Criteria for IPF Exclusion of other k...


...e 1. Comparison of ATS/ERS/JRS/ALAT Recommendati...

...cellular analysis

...Probable UIP, Indeterminate for UIP, and...

...T Pattern of UIP: We suggest NOT performing...

...cal lung biopsy...

...T Pattern of Probable UIP, Indeterminate for...

...ttern of UIP: We recommend NOT performing surg...

...nsbronchial lung biopsy...

...Pattern of Probable UIP, Indeterminate for UI...

...T Pattern of UIP: We recommend NOT perform...

...ng cryobio...

...ern of Probable UIP, Indeterminate for U...

...f UIP: We recommend NOT performi...

...y of medication use and environmental exposuresW...

...gical testing to exclude connective tissue disease...

...inary discussionWe suggest multidisciplinary di...

...um biomarkersWe recommend NOT measuring serum MM...


...h-Resolution CT Scanning ParametersHaving...


...ble 3. High-Resolution CT Scanning P...


...gh-Resolution CT Images Demonstrating a Usual Inte...


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


...e 3. Indeterminate for UIP Pattern...


...Indeterminate for UIP PatternÂ...


...T Pattern Suggestive of an Alternative Dia...


...re 6. Acute Exacerbation of IPF(A and B) Transve...


...igure 7. Histopathology Demonstrat...


Table 4. Histopathology Patterns and Fea...


...gure 8. IPF Diagnosis Based Upon HRCT and...


...9. Diagnostic Algorithm fo...