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(Circulation. 2009;119:e597-e598.)
© 2009 American Heart Association, Inc.

Images in Cardiovascular Medicine

Cardiac Involvement in Erdheim-Chester Disease

Magnetic Resonance and Computed Tomographic Scan Imaging in a Monocentric Series of 37 Patients

Julien Haroche, MD, PhD; Philippe Cluzel, MD, PhD; Dan Toledano, MD; Gilles Montalescot, MD, PhD; Diane Touitou, MD; Philippe A. Grenier, MD; Jean-Charles Piette, MD; Zahir Amoura, MD

From the Departments of Internal Medicine and French Reference Center for Rare Autoimmune and Systemic Diseases (J.H., J.C.P., Z.A.), Radiology (P.C., D. Toledano, D. Touitou, P.A.G.), and Cardiology (G.M.), Hôpital Pitié-Salpêtrière, Paris, France.

Correspondence to Dr Julien Haroche, Hôpital Pitié-Salpêtrière, 47-83 Boulevard de l’Hôpital, 75013 Paris, France. E-mail julien.haroche@psl.aphp.fr

An extract of the first 250 words of the full text is provided, because this article has no abstract.

Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis, characterized by the xanthomatous infiltration of tissues with foamy CD68⁺/CD1a^– histiocytes. By January 2009, >320 cases had been published in the medical literature. Bone pain is the most frequent symptom. Approximately half of patients have extraskeletal manifestations including exophthalmos, xanthelasma, interstitial lung disease, retroperitoneal "fibrosis" with perirenal or ureteral obstruction, renal failure, diabetes insipidus, and central nervous system and cardiovascular involvement. Interferon- is the recommended first-line therapy but is decided after a case-by-case analysis.¹

Cardiovascular manifestations of ECD are underdiagnosed, as we have shown in our analysis in 2004 of the 178 cases known at that time.² Among these, we analyzed 72 patients with cardiovascular involvement and found 54 (75%) with heart involvement: pericardial infiltration in 32 patients (44%) (leading to tamponade in 5 cases), myocardial infiltration in 22 cases (31%), a right atrial tumor in 6 patients, and a symptomatic valvular heart disease in 6 patients (3 aortic and 3 mitral regurgitations); 19 patients (26%) had heart failure, leading to death in 8 cases; myocardial infarction (MI) was reported in 6 cases, leading to death in 2. Forty of the 72 patients (56%) had a periaortic fibrosis, and 20 of these had a "coated aorta" aspect. Among the 58 patients (81%) with available follow-up, 35 (60%) died. Death was due to the cardiovascular involvement in 31% of the cases, confirming the severe prognosis of ECD with cardiovascular complications. The poor prognosis of ECD with cardiovascular involvement led us . . . [Full Text of this Article]