CTICasesLupus-Associated Pulmonary Fibrosis
Imaging findings
Chest CT in a patient in her early 40s with systemic lupus erythematosus demonstrates progressive pulmonary fibrosis with non-usual interstitial pneumonia (non-UIP) features. Findings include the straight-edge sign (abrupt demarcation of peripheral basilar fibrosis), the anterior upper lobe sign (fibrosis localized to the anterior aspects of the upper lobes), and the four corner sign. Dilated, patent esophagus is present, suggesting scleroderma/lupus overlap.
Key takeaways
Pulmonary fibrosis secondary to connective tissue diseases (CTD-ILD), such as lupus or scleroderma, frequently manifests with distinct imaging signs that differentiate it from idiopathic pulmonary fibrosis (IPF/UIP). These signs include the straight-edge sign, four-corner sign, and anterior upper-lobe sign. Esophageal dilation is a common concomitant finding, raising concern for an overlap syndrome or chronic microaspiration as a contributor to fibrotic progression.
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