Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra.
10.1016/j.krcp.2014.07.007
- Author:
Manuel Alfredo PODESTA
1
;
Giorgio GRAZIANI
;
Francesco REGGIANI
;
Michele BUEMI
;
Salvatore BADALAMENTI
;
Claudio PONTICELLI
Author Information
1. Nephrology and Dialysis Unit, Humanitas Clinical and Research Center, Milano, Italy. giorgio.graziani@humanitas.it
- Publication Type:Case Report
- Keywords:
Anakinra;
Erdheim-Chester disease;
Renal failure
- MeSH:
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*
- From:Kidney Research and Clinical Practice
2014;33(3):165-167
- CountryRepublic of Korea
- Language:English
-
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.