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Progressive Systemic Sclerosis with Pulmonary Ossification

Imaging findings

High-resolution CT demonstrates progressive dependent-predominant ground-glass opacities, mild fibrosis, esophageal dilatation with a fluid level, and extensive, highly dense dendriform pulmonary ossification restricted to the areas of parenchymal fibrosis.

Key takeaways

Dendriform pulmonary ossification is a rare bone-forming process that can complicate any chronic fibrotic interstitial lung disease, including systemic sclerosis-associated nonspecific interstitial pneumonia (NSIP). While chronic microaspiration secondary to esophageal dysmotility is a suspected trigger, active fibroblasts within the fibrosing lung can directly differentiate into osteoblasts and lay down mature bone.

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