|
KMID : 0359920140330030165
|
|
Korean Journal of Nephrology
2014 Volume.33 No. 3 p.165 ~ p.167
|
|
|
Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra
|
|
Podesta Manuel Alfredo
Graziani Giorgio
Reggiani Francesco
Buemi Michele
Badalamenti Salvatore
Ponticelli Claudio
|
|
|
Abstract
|
|
|
|
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by in?ltrates of lipid-laden CD68þ/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 ef?cacy 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.
|
|
|
KEYWORD
|
|
|
Anakinra, Erdheim-Chester disease, Renal failure,
|
|
|
FullTexts / Linksout information
|
|
|
|
|
|
Listed journal information
|
|
  
|