Kidney Res Clin Pract.  2014 Sep;33(3):165-167. 10.1016/j.krcp.2014.07.007.

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

• Podesta MA
• Graziani G
• Reggiani F
• Buemi M
• Badalamenti S
• Ponticelli C

• KMID: 2028694
• DOI: http://doi.org/10.1016/j.krcp.2014.07.007

Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by infiltrates of lipid-laden CD68+/CD1- 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 (IFNalpha), 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.

Keyword

Anakinra; Erdheim-Chester disease; Renal failure

MeSH Terms

Aged
Central Nervous System
Diagnosis
Erdheim-Chester Disease
Heart
Histiocytes
Histiocytosis
Humans
Interferon-alpha
Interleukin 1 Receptor Antagonist Protein*
Interleukin-1
Kidney
Lung
Renal Insufficiency*
Interferon-alpha
Interleukin 1 Receptor Antagonist Protein
Interleukin-1