Fast Five Quiz: Interstitial Lung Disease Progressive Fibrosing Management

Zab Mosenifar, MD


February 01, 2022

Figure 1. 3D CT scan of the lungs of a patient with ILD and interstitial fibrosis that has developed from chronic obstructive pulmonary disease (COPD). ILD is a group of lung diseases that affect the tissue in the alveoli (air sacs) of the lungs. Most of these diseases later develop fibrosis (the formation of thickened scar tissue) in the lungs. ILD results in loss of lung volume, impairing gaseous exchange and causing shortness of breath.

Imaging is currently considered the best predictor of progression; previous decline in forced vital capacity is not a reliable indicator of future decline.

Diagnostic biomarkers for fibrotic lung disease have not been validated; however, increased circulating fibrocytes have been suggested in patients with IPF, though data regarding vascular endothelial growth factor is contradictory. Chitotriosidase detected in bronchoalveolar lavage fluid may possibly correlate with the severity of sarcoidosis.

Functional parameters used to monitor and evaluate disease progression include the percentage value on the predicted forced vital capacity, the diffusion capacity of the lung for carbon monoxide, and 6-minute-walk-distance data. Worsening dyspnea is also a primary indicator of disease progression, as are acute exacerbations.

Learn more about the treatment of PF-ILD.


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