CTICasesShrinking Lung Syndrome in Systemic Lupus Erythematosus
Imaging findings
CT demonstrates markedly low lung volumes with bibasilar crowding and atelectasis, in the absence of underlying parenchymal lung disease or pulmonary fibrosis. Associated findings include mild cardiomegaly, a trace pericardial effusion, and small bilateral pleural effusions. The diaphragmatic crura appear morphologically normal and robust, without evidence of thinning or muscle atrophy.
Key takeaways
Shrinking lung syndrome is a rare manifestation of systemic lupus erythematosus characterized by progressive dyspnea, elevated hemidiaphragms, and severely reduced lung volumes on pulmonary function tests without parenchymal interstitial disease. The underlying pathophysiology remains controversial, with proposed mechanisms including diaphragmatic dysfunction, phrenic neuropathy, or pleural-related chest wall restriction. Although some cases exhibit thinning of the diaphragmatic crura on CT, the diaphragmatic musculature may appear completely normal in size and thickness.
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