Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by infiltrates of lipid-laden CD68+/CD1- histiocytes, affecting heart, lungs, central nervous system, and bones. Kidney and adjacent structures can also be affected, leading to renal failure in about 30% of cases. The diagnosis is challenging, and treatment is generally based on administration of interferon-alpha (IFNalpha), but preliminary results also showed the therapeutic efficacy of anakinra, an antagonist of the receptor of interleukin-1 (IL-1). We report the case of an elderly patient with ECD and severe involvement of the heart and kidneys who was successfully treated with anakinra.
Aged ; Central Nervous System ; Diagnosis ; Erdheim-Chester Disease ; Heart ; Histiocytes ; Histiocytosis ; Humans ; Interferon-alpha ; Interleukin 1 Receptor Antagonist Protein* ; Interleukin-1 ; Kidney ; Lung ; Renal Insufficiency*