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J R Soc Med. 2005 June; 98(6): 296.
PMCID: PMC1142256

Erdheim–Chester disease

Dr Lee and his colleagues (April 2005 JRSM1) describe a case of renal failure in which the radiographic appearance of bone pointed to Erdheim–Chester disease, a histiocytosis. Among the organs affected in this condition they mention pericardium. We think it important to emphasize the frequency with which heart, pericardium and great vessels are affected. Recording 6 new patients with cardiovascular involvement, Haroche et al.2 have reviewed all 178 reported cases of Erdheim–Chester disease. 56% had periaortic fibrosis, 44% pericardial infiltration and/or effusions and 31% myocardial infiltration. Interestingly, 6 patients had a right atrial myxoma. Of the 58 patients with follow-up data, 31% died from cardiovascular complications.


1. Lee JHM, Stodell M and Fowler JC. Renal bone disease. J R Soc Med 2005;98: 165-6 [PMC free article] [PubMed]
2. Haroche J, Amoura Z, Dion E, et al. Cardiovascular involvement, an overlooked feature of Erdheim–Chester disease: report of 6 new cases and a literature review. Medicine 2004;83: 371-92 [PubMed]

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