Haroche Julien, Amoura Zahir, Dion Elisabeth, Wechsler Bertrand, Costedoat-Chalumeau Nathalie, Cacoub Patrice, Isnard Richard, Généreau Thierry, Wechsler Janine, Weber Nina, Graef Claire, Cluzel Philippe, Grenier Philippe, Piette Jean-Charles
From Service de Médecine Interne (JH, ZA, BW, NCC, P Cacoub, JCP), Service de Radiologie Générale (ED, CG, P Cluzel, PG), and Institut de Cardiologie (RI), Hôpital Pitié-Salpêtrière, Paris; Service de Médecine Interne (TG), Hôpital Saint-Antoine, Paris; Département de Pathologie (JW), Hôpital Henri-Mondor, Créteil; Département de Pathologie (NW), Hôpital Victor Dupouy, Argenteuil, France.
Medicine (Baltimore). 2004 Nov;83(6):371-392. doi: 10.1097/01.md.0000145368.17934.91.
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%).
厄尔海德姆-切斯特病（ECD）是一种罕见的、起源不明的非朗格汉斯组织细胞增多症。其特征是泡沫状（“泡沫样”）组织细胞对组织进行黄色瘤或黄色肉芽肿性浸润。截至撰写本文时，已报告178例病例。ECD具有异质性的全身表现。骨痛是最常见的症状。约一半的患者有骨骼外表现。ECD的心血管表现仍未得到充分认识。我们报告6例与主动脉周围纤维化相关的ECD新病例。其中4例患者的整个主动脉呈现“被包裹”的外观。文献综述显示66例ECD患者有心血管受累。因此，我们分析了72例有心血管受累的ECD患者：40例（55.6%）有主动脉周围“纤维化”，32例（44.4%）有心包受累，22例（30.6%）有心肌受累。6例有右心房肿瘤。6例患者发现有症状性瓣膜性心脏病（3例主动脉瓣反流和3例二尖瓣反流）。19例患者（26.4%）发生心力衰竭，其中8例死亡。6例患者有与肾动脉周围狭窄相关的肾血管性高血压。58例（80.6%）患者有随访数据。其中35例（60.3%）患者死亡，证实了ECD的严重预后。心血管并发症导致了35例患者中11例（31.4%）死亡。
