A long-term survival case of Erdheim-Chester disease on maintenance hemodialysis

Erdheim-Chester disease, a rare non-Langerhans histiocytosis, involves multiple organs, including kidney. Renal dysfunction sometimes occurs, and is attributed to ureteral obstruction and renal artery stenosis by histiocytic infiltration. However, to our knowledge, case reports of end-stage renal disease requiring renal replacement therapy due to Erdheim-Chester disease are very few. Here, we report a 69-year-old woman who was diagnosed with Erdheim-Chester disease 10 years ago. She had multiple organ involvement, such as bone, skin, heart, pituitary gland, kidney, and retroperitoneum. She had been treated with interferon-alpha, but discontinued after 2 years due to depression and repeated infection. She did not desire treatment with other drugs, so we continued supportive care. Her renal function gradually deteriorated, and hemodialysis was initiated 4 years ago. Subsequently, she is still doing well without any major symptoms. This report describes an unusual case of Erdheim-Chester disease requiring maintenance hemodialysis that longer prognosis than expected was obtained regardless of multiple organ involvement and no specific treatment after interferon-alpha cessation.