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...

...agnostic Criteria for IPF Exclusion of...


...mparison of ATS/ERS/JRS/ALAT Recommendatio...

BAL cellular analysis

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

...n of UIP: We suggest NOT performing...

...cal lung biops...

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

...Pattern of UIP: We recommend NOT perf...

...sbronchial lung biops...

...of Probable UIP, Indeterminate for UIP,...

...ern of UIP: We recommend NOT performin...

Lung cryobi...

...RCT Pattern of Probable UIP, Indeterminate...

...RCT Pattern of UIP: We recommend NOT perfo...

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

...ing to exclude connective tissue dise...

Multidisciplinary discussionWe suggest multi...

...m biomarkersWe recommend NOT measuring serum...


...2. High-Resolution CT Scanning Pa...


...High-Resolution CT Scanning Patterns Having trou...


...High-Resolution CT Images Demonstrating a Usu...


...robable UIP Pattern(A–C) Transverse computed tom...


...determinate for UIP Pattern (early UIP pattern)(...


...determinate for UIP Pattern ...


...CT Pattern Suggestive of an Alternati...


...ure 6. Acute Exacerbation of IPF(A and B) Transv...


...7. Histopathology Demonstrating UIP(A)...


...le 4. Histopathology Patterns and Fea...


.... IPF Diagnosis Based Upon HRCT and Bio...


...gure 9. Diagnostic Algorithm...