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Restrictive Allograft Syndrome

Imaging findings

Chest CT in a 60-year-old lung transplant recipient (who had silicosis with progressive massive fibrosis prior to transplant) shows development of progressive, symmetric, and severe upper lobe predominant fibrosis, traction bronchiectasis, and volume loss in the allograft five years post-transplant, without recurrent silicotic nodules. Pleural thickening is present at the lung bases.

Key takeaways

Restrictive allograft syndrome (RAS) is a distinct phenotype of chronic lung allograft dysfunction (CLAD) characterized by progressive lung fibrosis, pleural thickening, and restrictive physiology, carrying a worse prognosis than bronchiolitis obliterans syndrome (BOS). While it typically manifests as upper-lobe-predominant pleuroparenchymal fibroelastosis-like changes, in this patient it can mimic recurrent silicosis, though the lack of centrilobular nodularity points to RAS.

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