CTICasesProgression and acute exacerbation of scleroderma-associated NSIP
Imaging findings
CT in 2014 shows classic fibrotic NSIP with lower lobe volume loss and subpleural sparing. CT five years later shows complete loss of the subpleural sparing, progressive fibrosis, and a superimposed acute exacerbation manifested by new, diffuse ground-glass opacities.
Key takeaways
Subpleural sparing is a highly specific sign for NSIP, but can disappear as the fibrotic process progresses. Patients with systemic sclerosis-associated ILD are at risk for acute exacerbations (rapid progression of ground-glass opacities), which carry high mortality.
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