Cardiovascular Involvement, an Overlooked Feature of Erdheim-Chester Disease
| Autor: | Zahir Amoura, Nathalie Costedoat-Chalumeau, Julien Haroche, Claire Graef, Philippe Grenier, Nina Weber, Bertrand Wechsler, Patrice Cacoub, Elisabeth Dion, Philippe Cluzel, Janine Wechsler, Jean-Charles Piette, T Généreau, Richard Isnard |
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| Rok vydání: | 2004 |
| Předmět: |
Adult
Male Erdheim-Chester Disease medicine.medical_specialty Heart Diseases genetic structures Renal Artery Obstruction Gastroenterology Renovascular hypertension Internal medicine Humans Medicine Bone pain Aorta Aged business.industry valvular heart disease General Medicine Middle Aged medicine.disease Fibrosis Surgery Histiocytosis Stenosis Hypertension Renovascular Mitral incompetence Heart failure Erdheim–Chester disease Female medicine.symptom business |
| Zdroj: | Medicine. 83:371-392 |
| ISSN: | 0025-7974 |
| Popis: | Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown origin. It is characterized by xanthomatous or xanthogranulomatous infiltration of tissues by spumous ("foamy") histiocytes. As of this writing, 178 cases have been reported. ECD is characterized by heterogeneous systemic manifestations. Bone pain is the most frequent symptom. About half of all patients have extraskeletal manifestations. Cardiovascular manifestations of ECD remain underestimated. We report 6 new cases of ECD associated with periaortic fibrosis. In 4 of these cases, the whole aorta had a "coated" aspect. A literature review revealed 66 cases of ECD with cardiovascular involvement. We therefore analyzed 72 ECD patients with cardiovascular involvement: 40 (55.6%) had periaortic "fibrosis," 32 (44.4%) had pericardial involvement, and 22 (30.6%) had myocardial involvement. Six had a right atrial tumor. Symptomatic valvular heart disease (3 aortic and 3 mitral regurgitations) was found in 6 patients. Nineteen patients (26.4%) had heart failure, leading to death in 8 cases. Six patients had renovascular hypertension related to perirenal artery stenosis. Data concerning follow-up were available for 58 (80.6%) patients. Of these, 35 (60.3%) patients died, confirming the severe prognosis of ECD. Cardiovascular complications were responsible for the death of 11 of the 35 patients (31.4%). |
| Databáze: | OpenAIRE |
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