Table 1 Unanswered questions in ILD diagnosis and sources of confusion and controversy.

From: Usual interstitial pneumonia: a clinically significant pattern, but not the final word

Is UIP a diagnosis or merely a pattern?

Is pathology the gold standard for a diagnosis of UIP?

Does a diagnosis of UIP imply a diagnosis of IPF?

How does one “prove” the etiology of a given case of UIP?

What should be done when radiologic and pathologic findings are discordant?

Should biopsy interpretation be influenced by clinical and imaging findings?

What histologic features reliably distinguish etiologies of fibrotic ILD?

What is the threshold for number or extent of granulomas/interstitial inflammation/airway-centered changes/etc. to issue an alternate non-UIP diagnosis?

Can UIP be diagnosed at an earlier stage, and if so, what terminology should be used?

Does UIP indicate a specific pathobiology, or simply a common disease endpoint (cf. hepatic cirrhosis)?

When does organizing pneumonia or acute lung injury argue against UIP, or suggest acute exacerbation of UIP of IPF?

How should the broader histologic context influence interpretation of an individual histologic feature?

  1. ILD interstitial lung disease, UIP usual interstitial pneumonia.