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

...iagnosis...

...c Criteria for IPF Exclusion of other known c...


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

...cellular analysis...

...Pattern of Probable UIP, Indetermi...

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

...ical lung biopsy...

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

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

...nsbronchial lung biop...

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

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

...cryobiopsy...

...tern of Probable UIP, Indeterminat...

...f UIP: We recommend NOT performing cryobiopsy. (...

...l history of medication use and environment...

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

...y discussionWe suggest multidisciplinary...

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


Table 2. High-Resolution CT Scanning ParametersHa...


...le 3. High-Resolution CT Scanning Patte...


...gure 1. High-Resolution CT Images Demonstrating a...


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


...igure 3. Indeterminate for UIP Patt...


.... Indeterminate for UIP Pattern 


...ure 5. CT Pattern Suggestive of an Alternativ...


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


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


Table 4. Histopathology Patterns and Featu...


...ure 8. IPF Diagnosis Based Upon HRCT and Bi...


...Diagnostic Algorithm for IPF