CTICasesLupus-Associated Interstitial Lung Disease (Fibrotic NSIP with Cystic Changes)
Imaging findings
High-resolution CT shows well-demarcated, "island-like" areas of subpleural fibrosis and macrocystic changes. These changes characteristically involve the corners of the lungs, specifically the anterior corners of the upper lobes and the lung bases. Notably, adjacent lung parenchyma is spared, and there is a conspicuous lack of significant volume loss or traction bronchiectasis compared to typical usual interstitial pneumonia (UIP).
Key takeaways
Although the cystic changes in these systemic lupus erythematosus (SLE) patients can radiologically resemble honeycombing, they do not represent typical UIP or idiopathic pulmonary fibrosis (IPF). Histopathologically, these well-demarcated areas represent a mixture of fibrotic NSIP, organizing pneumonia, and airspace enlargement. A potential etiology includes recurrent pleural or serosal inflammation causing chronic, localized subpleural parenchymal damage.
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