Usual interstitial pneumonia (UIP), the underlying histology of idiopathic pulmonary fibrosis (IPF), has a poor prognosis (median survival, 3 years), and to date there is no impressively effective therapy. Therefore, at least for prognosis, and in some cases for early transplant referral, it is important to confirm this diagnosis.
Expert radiologists and clinicians can accomplish this in approximately 50% of cases of UIP with a high degree of specificity.1 When typical radiologic features are present and tissue acquisition is deemed unnecessary, most clinicians feel that the disease is already advanced. However, there are a number of patients without typical radiologic findings, and also younger subjects, in whom a histologic diagnosis is often necessary. In addition, a number of treatment trials are in motion for IPF and a tissue diagnosis is required for inclusion in these trials.