CTICasesShrinking Lung Syndrome in Lupus Myositis
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Imaging findings
Patient with lupus presented with gradual restriction on PFTs, worsening dyspnea, and mosaic attenuation. Radiographs showed elevated right hemidiaphragm and slightly elevated left hemidiaphragm compared to prior studies. CT showed crowded lower lobes and gradual loss of lung volume. Comparison to prior CT (2014) showed bilateral diaphragm thinning and fatty infiltration of chest wall and paraspinal muscles. Expiratory CT showed only mild air trapping.
Key takeaways
Shrinking lung syndrome (SLS) in lupus patients is characterized by progressive loss of lung volume, often due to diaphragmatic dysfunction. This case showed bilateral diaphragm thinning, suggestive of a lupus myopathy affecting the diaphragm, possibly exacerbated by steroids. Improvement with steroid therapy (after a lupus nephritis flare) suggests an autoimmune component. The MVV (maximum voluntary volume) test and paradoxical abdominal wall movement support diaphragmatic dysfunction. This case could be publishable if diaphragm thickness recovery on steroids is documented.
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