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 other kno...


...able 1. Comparison of ATS/ERS/JRS/AL...

BAL cellular a...

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

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

Surgical lung b...

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

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

Transbronchial lung biop...

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

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

...ung cryobiops...

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

...n of UIP: We recommend NOT performing cryobiopsy...

...dical history of medication use and environmental...

...testing to exclude connective tissue d...

...isciplinary discussionWe suggest multidisci...

...um biomarkersWe recommend NOT measuring...


...High-Resolution CT Scanning ParametersH...


...High-Resolution CT Scanning Patterns ...


...Resolution CT Images Demonstrating a Usual Inter...


...2. Probable UIP Pattern(A–C) Transverse com...


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


...Indeterminate for UIP Pattern ...


...tern Suggestive of an Alternative Diagnosis for L...


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


...re 7. Histopathology Demonstrating...


...ble 4. Histopathology Patterns and Feature...


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


...agnostic Algorithm for IPF...