Image of Erdheim–Chester disease requiring hemodialysis

Excerpt

Erdheim–Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis, first described in 1930, with a wide range of manifestations through the xanthomatous infiltration of tissues [1]. A 52-year-old Japanese male who was diagnosed as ECD 6 years ago has been treated with IFN-α for preventing progression of cardiovascular and renal involvements. Computed tomography (CT) showed soft-tissue attenuation along the wall of the bilateral renal pelvis and ureter, enlarged right kidney, and thinned left renal parenchyma associated with hydronephrosis (Fig. 1). T2-weighted magnetic resonance imaging (MRI) showed low intensity along the wall of the bilateral renal pelvis and ureter, which suggested chronic inflammation or fibrosis rather than tumor or lymphoma (Fig. 2). Potential causes of renal dysfunction included chronic ureteral obstruction and xanthomatous invasion. As in this case, end stage renal disease requiring hemodialysis due to ECD is very rare, to our knowledge, but interferon-α treatment dramatically decreased mortality [2]. Follow-up of this case would be beneficial for management of hemodialysis in such patients.

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