Diagnostic Criteria for Idiopathic Pulmonary Fibrosis

In this presentation, Dr. Anu Brixey discusses the diagnostic criteria for idiopathic pulmonary fibrosis (IPF). She emphasizes that IPF is not synonymous with usual interstitial pneumonia (UIP), clarifying that while IPF is truly idiopathic, UIP can be either idiopathic or non-idiopathic. The diagnostic criteria for IPF involve excluding known causes of fibrosing lung disease, such as connective tissue diseases or environmental exposures. Dr. Brixey highlights the importance of distinguishing IPF from non-idiopathic causes of UIP because of the specific therapies available, such as pirfenidone and nintedanib. The cost of these medications underscores the need for an accurate diagnosis. Clinical data associated with IPF includes: patients, typically being white males over the age of 60, often current or former smokers, experiencing chronic progressive dyspnea and a dry cough. High-resolution CT imaging reveals specific patterns, such as subpleural basilar predominant distribution, reticulation, peripheral traction bronchiectasis, and honeycombing. Dr. Brixey provides insights into categorizing CT findings based on UIP patterns and stresses the importance of not overcalling ground glass opacities as typical features of UIP. The presentation concludes with considerations for biopsy recommendations and the critical role of radiologists in accurately reporting UIP without determining whether the findings are idiopathic.