Progressive pulmonary fibrosis: current perspectives in diagnostic imaging.

Abstract

A subset of patients with interstitial lung diseases (ILDs) experiences disease progression despite standard treatment protocols. Similar to idiopathic pulmonary fibrosis, the archetype of progressive fibrotic ILDs, these patients exhibit worsening clinical symptoms, declining lung function, and progressive radiological changes, often resulting in shortened survival. This progressive disease pattern is classified under the term progressive pulmonary fibrosis or progressive fibrosing ILD. Radiological imaging, particularly high-resolution computed tomography (HRCT), is integral to diagnosing ILDs and plays a critical role within multidisciplinary ILD boards. HRCT is instrumental in identifying patients at a higher risk for disease progression and may provide valuable prognostic insights. Additionally, serial imaging is essential for detecting progression over time. While visual assessment remains the primary method for evaluating disease advancement, emerging quantitative techniques, including those utilizing machine learning, are currently undergoing validation.