Diaphragmatic Myopathy (Myositis) in Graft-versus-Host Disease
Imaging findings
A bone marrow transplant patient with graft-versus-host disease (GVHD) presented with respiratory distress and low lung volumes. Despite having robust, normal-thickness diaphragmatic crura on CT (unchanged from prior exams, indicating no atrophy), fluoroscopy demonstrated significantly reduced but orthograde diaphragmatic excursion when standing, which further worsened in the supine position. The patient also exhibited sky-high CPK and LDH levels.
Key takeaways
Diaphragmatic myopathy, particularly in the context of graft-versus-host disease (GVHD), can lead to severe respiratory dysfunction and reduced diaphragm excursion despite preserved muscle thickness and an absence of atrophy. This condition is characterized by myositis, indicated by elevated muscle enzymes (CPK, LDH). The diaphragm's motion remains orthograde (moves in the correct direction but with reduced amplitude), distinguishing it from diaphragmatic paralysis (where paradoxical motion occurs).
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