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...

Descripción completa

Detalles Bibliográficos
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
Descripción
Sumario: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.

Ejemplares similares