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

Zab Mosenifar, MD


April 24, 2023

According to the ATS/ERS/JRS/ALAT Clinical Practice Guidelines, physiologic evidence of disease progression is defined as the presence of either of these two findings within 1 year of follow-up, if the findings are attributable to worsening fibrosis:

  • Absolute decline in forced vital capacity (FVC) ≥ 5%

  • Absolute decline in diffusing capacity for carbon dioxide (DLCO) (corrected for Hb) ≥ 10%

Although newly developed pulmonary hypertension and an acute exacerbation may be a sign of disease progression, they are highly variable and may be altered by the clinical context.

Learn more about fibrotic lung disease.


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