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.
|Titolo:||Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra|
GRAZIANI, GIORGIO (Corresponding)
|Parole Chiave:||Anakinra; Erdheim-Chester disease; Renal failure|
|Settore Scientifico Disciplinare:||Settore MED/14 - Nefrologia|
|Data di pubblicazione:||1-set-2014|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1016/j.krcp.2014.07.007|
|Appare nelle tipologie:||01 - Articolo su periodico|