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The experience of canakinumab in renal amyloidosis secondary to Familial Mediterranean fever

INTRODUCTION: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited recurrent attacks of fever and serositis. Patients may develop renal amyloidosis. Colchicine prevents attacks and renal amyloidosis. Five to 10 % of the patients with FMF are resistant or...

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Autores principales: Sozeri, Betul, Gulez, Nesrin, Ergin, Malik, Serdaroglu, Erkin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5010548/
https://www.ncbi.nlm.nih.gov/pubmed/27590627
http://dx.doi.org/10.1186/s40348-016-0058-2
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author Sozeri, Betul
Gulez, Nesrin
Ergin, Malik
Serdaroglu, Erkin
author_facet Sozeri, Betul
Gulez, Nesrin
Ergin, Malik
Serdaroglu, Erkin
author_sort Sozeri, Betul
collection PubMed
description INTRODUCTION: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited recurrent attacks of fever and serositis. Patients may develop renal amyloidosis. Colchicine prevents attacks and renal amyloidosis. Five to 10 % of the patients with FMF are resistant or intolerant to colchicine. CASE DESCRIPTION: Herein, we reported our experience with clinical-laboratory features and treatment responses of a pediatric FMF patient with amyloidosis treated with canakinumab. We observed a significant decrease in proteinuria and increase growth in the patient. DISCUSSION AND EVALUATION: The most serious complication of FMF is the development of AA type amyloidosis which is characterized by proteinuria. Colchicine is the prototype drug that decreases production of amyloidogenic precursor protein. Occasionally, colchicine inadequate patient is observed, as in our case. Canakinumab is a human anti-IL-1β monoclonal antibody. Previously, canakinumab efficacy were shown in a limited number of studies. CONCLUSIONS: Our data, though limited to only one patient, emphasize that therapeutic intervention with canakinumab seems to be improve kidney function in colchicine-resistant FMF with renal amyloidosis.
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spelling pubmed-50105482016-09-22 The experience of canakinumab in renal amyloidosis secondary to Familial Mediterranean fever Sozeri, Betul Gulez, Nesrin Ergin, Malik Serdaroglu, Erkin Mol Cell Pediatr Case Study INTRODUCTION: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited recurrent attacks of fever and serositis. Patients may develop renal amyloidosis. Colchicine prevents attacks and renal amyloidosis. Five to 10 % of the patients with FMF are resistant or intolerant to colchicine. CASE DESCRIPTION: Herein, we reported our experience with clinical-laboratory features and treatment responses of a pediatric FMF patient with amyloidosis treated with canakinumab. We observed a significant decrease in proteinuria and increase growth in the patient. DISCUSSION AND EVALUATION: The most serious complication of FMF is the development of AA type amyloidosis which is characterized by proteinuria. Colchicine is the prototype drug that decreases production of amyloidogenic precursor protein. Occasionally, colchicine inadequate patient is observed, as in our case. Canakinumab is a human anti-IL-1β monoclonal antibody. Previously, canakinumab efficacy were shown in a limited number of studies. CONCLUSIONS: Our data, though limited to only one patient, emphasize that therapeutic intervention with canakinumab seems to be improve kidney function in colchicine-resistant FMF with renal amyloidosis. Springer Berlin Heidelberg 2016-08-15 /pmc/articles/PMC5010548/ /pubmed/27590627 http://dx.doi.org/10.1186/s40348-016-0058-2 Text en © The Author(s). 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
spellingShingle Case Study
Sozeri, Betul
Gulez, Nesrin
Ergin, Malik
Serdaroglu, Erkin
The experience of canakinumab in renal amyloidosis secondary to Familial Mediterranean fever
title The experience of canakinumab in renal amyloidosis secondary to Familial Mediterranean fever
title_full The experience of canakinumab in renal amyloidosis secondary to Familial Mediterranean fever
title_fullStr The experience of canakinumab in renal amyloidosis secondary to Familial Mediterranean fever
title_full_unstemmed The experience of canakinumab in renal amyloidosis secondary to Familial Mediterranean fever
title_short The experience of canakinumab in renal amyloidosis secondary to Familial Mediterranean fever
title_sort experience of canakinumab in renal amyloidosis secondary to familial mediterranean fever
topic Case Study
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5010548/
https://www.ncbi.nlm.nih.gov/pubmed/27590627
http://dx.doi.org/10.1186/s40348-016-0058-2
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