Usual interstitial pneumonia is an almost uniformly fatal form of fibrosing interstitial lung disease. It is the most common idiopathic interstitial pneumonia, and currently, there is no effective therapy. Lung biopsy is often needed for diagnosis, and pathologists must be able to recognize its features and distinguish it from other interstitial lung diseases that have a better prognosis and a more favorable response to therapy. This review is an attempt to clarify the diagnostic pathologic features of usual interstitial pneumonia and to provide guidelines for its distinction from other interstitial lung diseases that enter the differential diagnosis.
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