1. Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra
- Author
-
Manuel Alfredo Podestà, Giorgio Graziani, Francesco Reggiani, Michele Buemi, Salvatore Badalamenti, and Claudio Ponticelli
- Subjects
Anakinra ,Erdheim-Chester disease ,Renal failure ,Internal medicine ,RC31-1245 ,Specialties of internal medicine ,RC581-951 - 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.
- Published
- 2014
- Full Text
- View/download PDF