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

doi:10.1016/j.krcp.2014.07.007

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

Istituto Clinico Humanitas

Research output: Contribution to journal › Article › peer-review

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.

Original language	English
Pages (from-to)	165-167
Number of pages	3
Journal	Kidney Research and Clinical Practice
Volume	33
Issue number	3
DOIs	https://doi.org/10.1016/j.krcp.2014.07.007
Publication status	Published - Sep 1 2014

Keywords

Anakinra
Erdheim-Chester disease
Renal failure

ASJC Scopus subject areas

Nephrology

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10.1016/j.krcp.2014.07.007

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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.

In: Kidney Research and Clinical Practice, Vol. 33, No. 3, 01.09.2014, p. 165-167.

Research output: Contribution to journal › Article › peer-review

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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.",

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AU - Podestà, Manuel Alfredo

AU - Graziani, Giorgio

AU - Reggiani, Francesco

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AU - Badalamenti, Salvatore

AU - Ponticelli, Claudio

PY - 2014/9/1

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N2 - 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.

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

Abstract

Keywords

ASJC Scopus subject areas

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