Fibrotic NSIP with Organizing Pneumonia in Scleroderma
Imaging findings
Initial CT demonstrates diffuse parenchymal lung disease with extensive ground glass opacities, consistent with NSIP, alongside superimposed areas suggestive of organizing pneumonia. Over three years, the disease progressed with resolution of much of the dense ground glass and increasing fibrosis, ultimately leading to severely fibrotic lung architecture.
Key takeaways
Non-specific interstitial pneumonia (NSIP) can progress rapidly to severe fibrosis, particularly in the context of connective tissue diseases like Scleroderma. The presence of organizing pneumonia may represent the initial injury that progresses to fibrosis. Severely fibrotic NSIP carries a poor prognosis, often necessitating lung transplantation.
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