Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra

Manuel Alfredo Podestà, Giorgio Graziani, Francesco Reggiani, Michele Buemi, Salvatore Badalamenti, Claudio Ponticelli

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)165-167
Number of pages3
JournalKidney Research and Clinical Practice
Issue number3
Publication statusPublished - Sep 1 2014


  • Anakinra
  • Erdheim-Chester disease
  • Renal failure

ASJC Scopus subject areas

  • Nephrology


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