Erdheim-Chester disease: a rare cause of acute renal failure

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.