2000
DOI: 10.2214/ajr.174.5.1741327
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Pulmonary Involvement with Erdheim-Chester Disease

Abstract: The most common findings of Erdheim-Chester disease with pulmonary involvement include an interstitial process characterized by smooth interlobular septal thickening and centrilobular nodular opacities, fissural thickening, and pleural effusions. On CT, six of nine patients had pericardial fluid and thickening or extrathoracic soft-tissue masses. Such findings are characteristic of Erdheim-Chester disease with pulmonary involvement. Definitive diagnosis requires correlating skeletal findings and lung biopsy fi… Show more

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Cited by 78 publications
(28 citation statements)
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“…The prevalence of lung involvement is variable in the literature [1,4,5,6]. One case series reported 53% of patients having lung involvement [6].…”
Section: Discussionmentioning
confidence: 99%
“…The prevalence of lung involvement is variable in the literature [1,4,5,6]. One case series reported 53% of patients having lung involvement [6].…”
Section: Discussionmentioning
confidence: 99%
“…The extra-skeletal sites reported to be involved are mediastinum or pericardium, heart, lungs, gastrointestinal tract, kidneys, retroperitoneum, central nervous system, skin and orbits [9]. The lung involvement shows a constellation of findings strongly suggesting ECD: smooth interlobular septal thickening, centrilobular nodular opacities, multifocal regions of ground-glass attenuation, fissural thickening and pleural effusions [11]. Microscopically ECD is characterised by the presence of aggregates of foamy histiocytic infiltrates with fibrosis and thickened bone trabeculae [3,6,7].…”
Section: Discussionmentioning
confidence: 99%
“…Typically, osseous involvement includes bilateral symmetrical sclerosis involving the diaphyses and metaphyses of long bones. 42,44 Bone pain is more common in the lower extremities. Fever and weight loss are also common.…”
Section: Pulmonary Langerhans Cell Histiocytosismentioning
confidence: 99%