O'Rourke R, Wong D C, Fleming S, Walker D
Radiology Department, The Wesley Hospital, Brisbane, Queensland, Australia.
Australas Radiol. 2007 Oct;51 Spec No.:B48-51. doi: 10.1111/j.1440-1673.2007.01821.x.
Erdheim-Chester disease (ECD) is a rare systemic histiocytic infiltrative disease of unknown aetiology. In radiology it is characterized by symmetrical sclerotic bone lesions predominantly affecting the diaphyses and metaphyses of long bones. Perivascular fibrosis has been reported in the literature as being a feature of this disease and we report one such case that presented with an encased aorta and renal arteries leading to acute renal failure. The diagnosis of ECD was delayed until a biopsy of the retroperitoneal infiltrate was performed. Further imaging with fluorine 18 deoxyglucose positron emission tomography, bone scintigraphy, plain films of the long bones and CT of the chest, abdomen and pelvis were performed to assess the extent of the patient's systemic disease involvement. To our knowledge, this is the first reported case of ECD presenting with acute renal failure secondary to bilateral occlusion of the renal arteries.
厄尔德海姆-切斯特病（ECD）是一种病因不明的罕见系统性组织细胞浸润性疾病。在放射学上，其特征为对称性硬化性骨病变，主要累及长骨的骨干和干骺端。文献报道血管周围纤维化是该疾病的一个特征，我们报告了这样一例病例，该病例表现为主动脉和肾动脉被包裹，导致急性肾衰竭。ECD的诊断一直延迟到对腹膜后浸润灶进行活检。进一步进行了氟-18脱氧葡萄糖正电子发射断层扫描、骨闪烁显像、长骨平片以及胸部、腹部和骨盆的CT检查，以评估患者全身疾病的累及范围。据我们所知，这是首例报告的因双侧肾动脉闭塞继发急性肾衰竭的ECD病例。
