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...

...riteria for IPF Exclusion of other known causes...


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

...cellular anal...

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

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

Surgical lung...

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

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

...ansbronchial lung biopsy...

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

...tern of UIP: We recommend NOT performing...

...ung cryobiopsy...

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

...of UIP: We recommend NOT performing cryob...

...dical history of medication use and environm...

...erological testing to exclude connective tissue...

...sciplinary discussionWe suggest multidisciplinary...

...biomarkersWe recommend NOT measuring serum...


...gh-Resolution CT Scanning ParametersHaving trou...


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


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


...re 2. Probable UIP Pattern(A–C) Transve...


Figure 3. Indeterminate for UIP Pattern (ea...


...ure 4. Indeterminate for UIP...


...igure 5. CT Pattern Suggestive of an Alte...


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


...e 7. Histopathology Demonstrating UIP(A) Low-ma...


...4. Histopathology Patterns and Features Hav...


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


Figure 9. Diagnostic Algorithm for IPF