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

Abstract

Usual interstitial pneumonia (UIP) is a concept that is deeply entrenched in clinical practice and the prognostic significance of UIP is well established, but the field continues to suffer from the lack of a true gold standard for diagnosing fibrotic interstitial lung disease (ILD). The meaning and usage of UIP have shifted over time and this term is prone to misinterpretation and poor diagnostic agreement. For pathologists, it is worth reflecting on the limitations of UIP and our true role in the care of patients with ILD, a controversial topic explored in two point-counterpoint editorials published simultaneously in this journal. Current diagnostic guidelines are ambiguous and difficult to apply in clinical practice. Further complicating matters for the pathologist is the paradigm shift that occurred with the advent of anti-fibrotic agents, necessitating increased focus on the most likely etiology of fibrosis rather than simply the pattern of fibrosis when pulmonologists select appropriate therapy. Despite the wealth of information locked in tissue samples that could provide novel insights into fibrotic ILDs, pulmonologists increasingly shy away from obtaining biopsies, likely because pathologists no longer provide sufficient value to offset the risks of a biopsy procedure, and pathologic assessment is insufficiently reliable to meaningfully inform therapeutic decisionmaking. To increase the value of biopsies, pathologists must first recognize the problems with UIP as a diagnostic term. Second, pathologists must realize that the primary goal of a biopsy is to determine the most likely etiology to target with therapy, requiring a shift in diagnostic focus. Third, pathologists must devise and validate new classifications and criteria that are evidence-based, biologically relevant, easy to use, and predictive of outcome and treatment response. Only after the limitations of UIP are understood will pathologists provide maximum diagnostic value from biopsies to clinicians today and advance the field forward.