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

...Diagnosis...

...stic Criteria for IPF Exclusion o...


...Table 1. Compari...

BAL cellular analysis

...ttern of Probable UIP, Indeterminate fo...

...CT Pattern of UIP: We suggest NOT...

...urgical lung biop...

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

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

...onchial lung biopsy...

...ttern of Probable UIP, Indeterminate...

...f UIP: We recommend NOT performing transbron...

...ung cryobio...

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

HRCT Pattern of UIP: We recommend...

...story of medication use and environmental exposur...

...testing to exclude connective tissue diseaseW...

...idisciplinary discussionWe suggest multidisci...

...erum biomarkersWe recommend NOT measuring seru...


...h-Resolution CT Scanning ParametersH...


Table 3. High-Resolution CT Scannin...


...e 1. High-Resolution CT Images Demonstratin...


...ure 2. Probable UIP Pattern(A–C) Transv...


...3. Indeterminate for UIP Pattern (early...


...Indeterminate for UIP Pattern ...


...T Pattern Suggestive of an Alternativ...


...cute Exacerbation of IPF(A and B) Tra...


...opathology Demonstrating UIP(A) Low-magn...


...Histopathology Patterns and Features Having tr...


...e 8. IPF Diagnosis Based Upon HRCT and Biopsy P...


...e 9. Diagnostic Algorithm for IPF...