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.

Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra / M.A. Podestà, G. Graziani, F. Reggiani, M. Buemi, S. Badalamenti, C. Ponticelli. - In: KIDNEY RESEARCH AND CLINICAL PRACTICE. - ISSN 2211-9132. - 33:3(2014 Sep 01), pp. 165-167. [10.1016/j.krcp.2014.07.007]

Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra

M.A. Podestà;G. Graziani
;
2014

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.
Anakinra; Erdheim-Chester disease; Renal failure
Settore MED/14 - Nefrologia
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/747783
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