| Summary: | The recently published American Thoracic Society/European Respiratory Society statement distinguishes idiopathic pulmonary fibrosis (IPF), also known as usual interstitial pneumonia (UIP), from the other idiopathic interstitial pneumonias (IIPs) (1). Although the current classification of IIPs is different from the one developed by Liebow and Carrington (2) in the 1960s, the description of UIP has not changed, and it is still recognized as having distinctive clinical and pathological features that distinguish it from the other IIPs. IPF responds differently to systemic corticosteroid (steroid) therapy and has a different prognosis than the other IIPs, such as nonspecific interstitial pneumonitis, which previously were felt to be variants of the same condition (1,3,4). Despite therapy, most patients with IPF experience a progressive decline in pulmonary function, leading to respiratory failure and death, unless they undergo lung transplantation.
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