一例最初被误诊为奥蒙德病的厄尔德海姆-切斯特病病例。
A case of Erdheim-Chester disease initially mistaken for Ormond's disease.
作者信息
Loddenkemper Konstanze, Hoyer Bimba, Loddenkemper Christoph, Hermann Kay-Geert, Rogalla Patrick, Förster Gregor, Buttgereit Frank, Hiepe Falk, Burmester Gerd-Rüdiger
机构信息
Department of Rheumatology and Clinical Immunology, Charité University Medicine, Berlin, Germany.
出版信息
Nat Clin Pract Rheumatol. 2008 Jan;4(1):50-5. doi: 10.1038/ncprheum0677.
BACKGROUND
A 54-year-old man presented with fever, abdominal pain, anemia, elevated C-reactive protein level and decreased renal function. Idiopathic retroperitoneal fibrosis (Ormond's disease) had been diagnosed in the past, leading to surgical ureterolysis. Re-evaluation of the CT scans and histological biopsy samples revealed unusual sclerosis of the long bones and diffuse infiltrates of foamy histiocytes in the bone marrow and the retroperitoneum.
INVESTIGATIONS
Physical examination, laboratory tests including hemoglobin concentration, erythrocyte sedimentation rate, C-reactive protein level, beta2-microglobulin level, creatinine level, CT of the chest and abdomen, bone scintigraphy, bone marrow and soft tissue biopsies and immunohistochemistry.
DIAGNOSIS
Erdheim-Chester disease with retroperitoneal fibrosis and bone sclerosis.
MANAGEMENT
Treatment with glucocorticoids failed. The patient's symptoms improved significantly after initiation of interferon-alpha therapy.
背景
一名54岁男性出现发热、腹痛、贫血、C反应蛋白水平升高及肾功能减退。既往诊断为特发性腹膜后纤维化（奥蒙德病），并行手术输尿管松解术。对CT扫描及组织活检样本的重新评估显示长骨出现异常硬化，骨髓及腹膜后有泡沫状组织细胞弥漫浸润。
检查
体格检查、实验室检查，包括血红蛋白浓度、红细胞沉降率、C反应蛋白水平、β2微球蛋白水平、肌酐水平、胸部及腹部CT、骨闪烁显像、骨髓及软组织活检以及免疫组化检查。
诊断
伴有腹膜后纤维化及骨硬化的厄尔德海姆-切斯特病。
治疗
糖皮质激素治疗失败。患者在开始使用α干扰素治疗后症状显著改善。
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