title: Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra.
abstract: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by infiltrates of lipid-laden CD68(+)/CD1a(-) 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 (IFNα), 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.
authors.affiliation: Nephrology and Dialysis Unit, Humanitas Clinical and Research Center, Milano, Italy.
authors.forename: Manuel Alfredo
authors.initials: MA
authors.lastname: Podestà
authors.affiliation: Nephrology and Dialysis Unit, Humanitas Clinical and Research Center, Milano, Italy.
authors.forename: Giorgio
authors.initials: G
authors.lastname: Graziani
authors.affiliation: Nephrology and Dialysis Unit, Humanitas Clinical and Research Center, Milano, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Reggiani
authors.affiliation: Department of Internal Medicine, University of Messina, Messina, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: Buemi
authors.affiliation: Nephrology and Dialysis Unit, Humanitas Clinical and Research Center, Milano, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Badalamenti
authors.affiliation: Nephrology and Dialysis Unit, Humanitas Clinical and Research Center, Milano, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Ponticelli
keywords: Anakinra; Erdheim-Chester disease; Renal failure