Fast Five Quiz: Progressive Fibrosing Interstitial Lung Disease Presentation and Diagnosis

Zab Mosenifar, MD


June 16, 2022

Patients with ILD associated with connective tissue disease and fibrotic hypersensitivity pneumonitis appear to have a better prognosis than patients with IPF.

Retrospective studies have identified risk factors for progression and mortality in PF-ILD, including lower diffusing capacity of the lung for carbon monoxide at baseline. Other risk factors include male sex, older age, lower forced vital capacity at baseline, and certain morphologic features, such as a radiologic and/or histologic pattern of usual interstitial pneumonia.

Egan and colleagues proposed a classification scheme of advanced versus limited disease based on a cutoff value of diffusing capacity of the lung for carbon monoxide greater than 40%.

Learn more about staging PF-ILD.


Comments on Medscape are moderated and should be professional in tone and on topic. You must declare any conflicts of interest related to your comments and responses. Please see our Commenting Guide for further information. We reserve the right to remove posts at our sole discretion.