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

Zab Mosenifar, MD


April 02, 2021

Progressive fibrosis (PF) in interstitial lung disease (ILD) is characterized by lung function deterioration, worsening respiratory symptoms, and decreased quality of life. Patients with PF-ILD who have a progressive phenotype may experience progressive fibrosis despite treatment with immunomodulatory therapy. In these patients, the disease may behave like idiopathic pulmonary fibrosis (IPF), which is the most common ILD. But the relative rarity of PF-ILDs makes the differential diagnosis very challenging and requires a multidisciplinary effort. The approach of studying PF-ILDs as a merged group based on a common outcome phenotype is garnering support.

Although there is no formal definition for progressive disease, a progressive phenotype can emerge in the absence of pulmonary fibrosis. 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.

Can you identify PF-ILD? Test your knowledge with this quick quiz.


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