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

...Diagnos...

...iagnostic Criteria for IPF Exclusion of o...


...Table 1. Compar...

...L cellular ana...

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

...Pattern of UIP: We suggest NOT performing BAL ce...

...al lung biopsy...

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

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

...onchial lung biopsy...

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

...ttern of UIP: We recommend NOT p...

Lung cryobiops...

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

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

Medical history of medication use and envir...

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

...iplinary discussionWe suggest multi...

...rkersWe recommend NOT measuring serum MM...


...Resolution CT Scanning ParametersHaving trouble v...


...e 3. High-Resolution CT Scanning Patterns Having...


...esolution CT Images Demonstrating a Usual Int...


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


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


...ndeterminate for UIP Pattern ...


...tern Suggestive of an Alternative Diagnosis fo...


...gure 6. Acute Exacerbation of IPF(A...


...ure 7. Histopathology Demonstrating UIP(A) Low-...


...able 4. Histopathology Patterns and Features Havi...


...IPF Diagnosis Based Upon HRCT and Biopsy...


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