The Role of Radiology in Progressive Fibrosing Interstitial Lung Disease

Abstract

In this article, we describe the role of radiology for diagnosis and follow-up of progressive fibrosing interstitial lung disease (PF-ILD). Patients with PF-ILD are at increased risk for early death without treatment. Clinical diagnosis of PF-ILD has been described in the literature. This manuscript reviews the radiographic diagnosis of PF-ILD and the unique CT characteristics associated with specific types of fibrosis. Ultimately, we believe that radiology has the potential to recognize progression early and thus make an important contribution to the multidisciplinary discussion for this important diagnosis.

Novel insights in fibrotic pulmonary sarcoidosis

“…Interestingly, the radiologist can measure the angle between a line traversing the right upper lobe bronchus and a sagittal line connecting the sternum to the vertebral body [42]. The angle, called right upper lobe bronchus angle (RUL-BA), increases with the fibrotic phase of the sarcoidosis and could represent a biomarker for progression [43].…”

“…Some CT features may discriminate between active lung inflammation and fibrosis: inflammatory findings include a bilateral distribution of micronodules, perilymphatic and bronchocentric distribution, and perihilar ground-glass opacities [31 & ,33]. Interestingly, the radiologist can measure the angle between a line traversing the right upper lobe bronchus and a sagittal line connecting the sternum to the vertebral body [42]. The angle, called right upper lobe bronchus angle (RUL-BA), increases with the fibrotic phase of the sarcoidosis and could represent a biomarker for progression [43].…”