Fast Five Quiz: Progressive Fibrosing Interstitial Lung Disease

Zab Mosenifar, MD, FACP, FCCP


April 12, 2022

Progression of fibrosing ILD is primarily reflected in a decline in lung function, worsening of symptoms, and deterioration in health-related quality of life. In clinical studies, disease progression is most commonly assessed through measurement of FVC and diffusion capacity of the lungs for carbon monoxide. The course of lung function decline in patients with IPF is variable. However, IPF, as other fibrosing ILDs, is progressive in its clinical course.

There is increasing interest in the use of radiologic markers as predictors of disease progression in patients with fibrosing ILDs. For example, honeycombing and traction bronchiectasis have been associated with a worse prognosis, and greater fibrotic changes on high-resolution computed tomography (HRCT) are associated with a higher risk for mortality. Nonetheless, the use of radiologic scoring systems as predictors of prognosis in clinical practice is hampered by subjectivity and variability in reading HRCT and will likely not be widely implemented until low-cost, automated scoring systems are available.

Genetic mutations and telomere length have also been investigated as predictors of disease progression in patients with fibrosing ILDs, but survival risk varies between subtypes, warranting further investigation before routine use in clinical practice.

Finally, several blood biomarkers have been investigated as predictors of disease progression in patients with fibrosing ILDs, including Krebs von den Lungen-6 protein (KL-6), which is associated with a higher rate of disease progression in patients with IPF and CTD-ILDs, as well as surfactant protein D. To date, however, no serum biomarker has been shown to be a sufficient prognostic marker to justify its use in clinical practice, although KL-6 is routinely used at some ILD centers in Japan.

Learn more about the pathogenesis of ILDs.


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