CTICasesPleuroparenchymal Fibroelastosis as a Manifestation of Graft-versus-Host Disease
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Imaging findings
A radiograph showed upper lobe predominant thickening of peripheral pleural tissue, subpleural thickening, apical findings, and volume loss, with tracheal deviation. Initial CT in 2007 showed apical thickening extending lower than usual, with progression seen in 2009 including inflammatory changes. By 2016, there was more extensive disease with peribronchial involvement in the lower lobes and background air trapping, consistent with constrictive bronchiolitis.
Key takeaways
This patient had a bone marrow transplant and developed graft-versus-host disease (GVHD). The findings are consistent with pleuroparenchymal fibroelastosis (PPFE) as a rare manifestation of GVHD. The patient was managed with low-dose immune suppression (mycophenolate) to stabilize the condition over 9 years of evolution, but remained dyspneic. Tissue diagnosis was not available, but the imaging findings are characteristic for this uncommon manifestation of GVHD.
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