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

Zab Mosenifar, MD


June 16, 2022

The PF-ILD phenotype is a relatively new concept that is still under investigation. Currently, no precise threshold or rate of decline has been formally accepted to identify a progressive phenotype in clinical practice. Assessment of the progression of fibrosis is typically based on successive lung-function tests performed at 3- to 6-month intervals. Because small discrepancies in forced vital capacity may be confounded by measurement errors, multimodal assessment of disease progression is necessary, including deteriorating symptoms and exercise capacity, increased fibrosis on imaging, decreased diffusing capacity of the lung for carbon monoxide, the need for oxygen supplementation, and clinical events predicting early death (eg, acute exacerbation of fibrosis or nonelective hospitalization). To date, no serum biomarker has been validated for monitoring disease progression, according to Kolb and colleagues.

Learn more about fibrotic lung disease.


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