TY - JOUR
T1 - Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra
AU - Podestà, Manuel Alfredo
AU - Graziani, Giorgio
AU - Reggiani, Francesco
AU - Buemi, Michele
AU - Badalamenti, Salvatore
AU - Ponticelli, Claudio
PY - 2014/9/1
Y1 - 2014/9/1
N2 - 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.
AB - 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.
KW - Anakinra
KW - Erdheim-Chester disease
KW - Renal failure
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U2 - 10.1016/j.krcp.2014.07.007
DO - 10.1016/j.krcp.2014.07.007
M3 - Article
AN - SCOPUS:84907875298
VL - 33
SP - 165
EP - 167
JO - Kidney Research and Clinical Practice
JF - Kidney Research and Clinical Practice
SN - 2211-9132
IS - 3
ER -