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

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...

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Autores principales: Podestà, Manuel Alfredo, Graziani, Giorgio, Reggiani, Francesco, Buemi, Michele, Badalamenti, Salvatore, Ponticelli, Claudio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2014
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4714171/
https://www.ncbi.nlm.nih.gov/pubmed/26877969
http://dx.doi.org/10.1016/j.krcp.2014.07.007
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author Podestà, Manuel Alfredo
Graziani, Giorgio
Reggiani, Francesco
Buemi, Michele
Badalamenti, Salvatore
Ponticelli, Claudio
author_facet Podestà, Manuel Alfredo
Graziani, Giorgio
Reggiani, Francesco
Buemi, Michele
Badalamenti, Salvatore
Ponticelli, Claudio
author_sort Podestà, Manuel Alfredo
collection PubMed
description 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.
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spelling pubmed-47141712016-02-12 Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra Podestà, Manuel Alfredo Graziani, Giorgio Reggiani, Francesco Buemi, Michele Badalamenti, Salvatore Ponticelli, Claudio Kidney Res Clin Pract Case Report 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. Elsevier 2014-09 2014-09-10 /pmc/articles/PMC4714171/ /pubmed/26877969 http://dx.doi.org/10.1016/j.krcp.2014.07.007 Text en © 2014. The Korean Society of Nephrology. Published by Elsevier. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Podestà, Manuel Alfredo
Graziani, Giorgio
Reggiani, Francesco
Buemi, Michele
Badalamenti, Salvatore
Ponticelli, Claudio
Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra
title Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra
title_full Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra
title_fullStr Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra
title_full_unstemmed Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra
title_short Improvement of Erdheim-Chester disease-related renal failure after treatment with anakinra
title_sort improvement of erdheim-chester disease-related renal failure after treatment with anakinra
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4714171/
https://www.ncbi.nlm.nih.gov/pubmed/26877969
http://dx.doi.org/10.1016/j.krcp.2014.07.007
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