Progressive fibrosing interstitial lung diseases (PF-ILD) are a heterogeneous group of interstitial lung diseases clinically characterized by accelerated respiratory failure, frequent disease exacerbation, decreased quality of life, and earlier mortality. Idiopathic pulmonary fibrosis (IPF) is the most common PF-ILD. It is characterized by progressive pulmonary fibrosis and a loss of lung function, with a median survival of 4 years from diagnosis. Not all ILDs are associated with progressive fibrosis; however, those that develop this phenotype possess a similar clinical course to IPF.
Progressive fibrosis of the lung parenchyma is self-sustaining and causes progressive deterioration in lung function and respiratory symptoms. It is estimated that 18%-32% of patients diagnosed with non-IPF-ILD develop progressive fibrosis. While immunosuppressive therapy appears to benefit patients with inflammatory ILD, it is associated with increased morbidity and mortality in patients with IPF, highlighting the importance of an accurate diagnosis to direct patient care.
Can you identify PF-ILD? Test your knowledge with this quick quiz.
Medscape © 2022 WebMD, LLC
Any views expressed above are the author's own and do not necessarily reflect the views of WebMD or Medscape.
Cite this: Zab Mosenifar. Fast Five Quiz: Progressive Fibrosing Interstitial Lung Disease Presentation and Diagnosis - Medscape - Jun 16, 2022.