CTICasesScleroderma-Associated Non-Specific Interstitial Pneumonia (NSIP)
Imaging findings
High-resolution chest CT demonstrates bilateral, lower-lung-predominant fibrosing interstitial lung disease. The imaging displays three classic findings: "exuberant honeycombing" (microcystic change at the bases), a "straight edge" boundary between normal and abnormal lung, and "anterior upper lobe" subpleural fibrosis.
Key takeaways
Fibrosing non-specific interstitial pneumonia (NSIP) is the most common pattern of interstitial lung disease in systemic sclerosis (scleroderma). The presence of the "three signs"—exuberant honeycombing, the straight edge sign, and the anterior upper lobe sign—is highly specific for a connective tissue disease-associated interstitial pneumonia rather than idiopathic pulmonary fibrosis (IPF).
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