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Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra
( Manuel Alfredo Podesta ),( Giorgio Graziani ),( Francesco Reggiani ),( Michele Buemi ),( Salvatore Badalamenti ),( Claudio Ponticelli ) 대한신장학회 2014 Kidney Research and Clinical Practice Vol.33 No.3
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterizedby infiltrates of lipid-laden CD68þ/CD1a histiocytes, affecting heart,lungs, central nervous system, and bones. Kidney and adjacent structures can alsobe affected, leading to renal failure in about 30% of cases. The diagnosis ischallenging, and treatment is generally based on administration of interferonalpha(IFNα), but preliminary results also showed the therapeutic efficacy ofanakinra, an antagonist of the receptor of interleukin-1 (IL-1). We report the caseof an elderly patient with ECD and severe involvement of the heart and kidneys whowas successfully treated with anakinra.