Definition of IPF

Abstract

Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonias (IIPs), is a fatal disease with a mean survival time of 2–4 years from the time of diagnosis. Therefore, the early and accurate diagnosis of IPF is important and essential for management and induction of optimal therapies. In 2002, the American Thoracic Society and European Respiratory Society (ATS/ERS) published an international statement on the diagnosis and management of IPF. The 2002 ATS/ERS statement defined IPF as a distinct clinical entity associated with the histology of usual interstitial pneumonia (UIP). The revised evidence-based guidelines for diagnosis and management of IPF were published by collaboration between the ATS, ERS, Japanese Respiratory Society (JRS), and Latin American Thoracic Association (ALAT) in 2011. In the revised 2011 criteria, high-resolution CT (HRCT) has a central role for the diagnosis of IPF. The presence of UIP patterns on HRCT is essential and definitive in the diagnosis of IPF without the need for surgical lung biopsy (SLB). The revised 2011 criteria have emphasized the importance of multidisciplinary discussion between clinicians, radiologists, and pathologists experienced in the diagnosis of IPF.