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Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever
Familial Mediterranean fever (FMF) is a genetic autoinflammatory disease with autosomal recessive transmission, characterized by periodic fever attacks with self-limited serositis. Secondary amyloidosis due to amyloid A renal deposition represents the most fearsome complication in up to 8.6% of pati...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8541210/ https://www.ncbi.nlm.nih.gov/pubmed/34684086 http://dx.doi.org/10.3390/medicina57101049 |
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author | Siligato, Rossella Gembillo, Guido Calabrese, Vincenzo Conti, Giovanni Santoro, Domenico |
author_facet | Siligato, Rossella Gembillo, Guido Calabrese, Vincenzo Conti, Giovanni Santoro, Domenico |
author_sort | Siligato, Rossella |
collection | PubMed |
description | Familial Mediterranean fever (FMF) is a genetic autoinflammatory disease with autosomal recessive transmission, characterized by periodic fever attacks with self-limited serositis. Secondary amyloidosis due to amyloid A renal deposition represents the most fearsome complication in up to 8.6% of patients. Amyloidosis A typically reveals a nephrotic syndrome with a rapid progression to end-stage kidney disease still. It may also involve the cardiovascular system, the gastrointestinal tract and the central nervous system. Other glomerulonephritis may equally affect FMF patients, including vasculitis such as IgA vasculitis and polyarteritis nodosa. A differential diagnosis among different primary and secondary causes of nephrotic syndrome is mandatory to determine the right therapeutic choice for the patients. Early detection of microalbuminuria is the first signal of kidney impairment in FMF, but new markers such as Neutrophil Gelatinase-Associated Lipocalin (NGAL) may radically change renal outcomes. Serum amyloid A protein (SAA) is currently considered a reliable indicator of subclinical inflammation and compliance to therapy. According to new evidence, SAA may also have an active pathogenic role in the regulation of NALP3 inflammasome activity as well as being a predictor of the clinical course of AA amyloidosis. Beyond colchicine, new monoclonal antibodies such as IL-1 inhibitors anakinra and canakinumab, and anti-IL-6 tocilizumab may represent a key in optimizing FMF treatment and prevention or control of AA amyloidosis. |
format | Online Article Text |
id | pubmed-8541210 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-85412102021-10-24 Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever Siligato, Rossella Gembillo, Guido Calabrese, Vincenzo Conti, Giovanni Santoro, Domenico Medicina (Kaunas) Review Familial Mediterranean fever (FMF) is a genetic autoinflammatory disease with autosomal recessive transmission, characterized by periodic fever attacks with self-limited serositis. Secondary amyloidosis due to amyloid A renal deposition represents the most fearsome complication in up to 8.6% of patients. Amyloidosis A typically reveals a nephrotic syndrome with a rapid progression to end-stage kidney disease still. It may also involve the cardiovascular system, the gastrointestinal tract and the central nervous system. Other glomerulonephritis may equally affect FMF patients, including vasculitis such as IgA vasculitis and polyarteritis nodosa. A differential diagnosis among different primary and secondary causes of nephrotic syndrome is mandatory to determine the right therapeutic choice for the patients. Early detection of microalbuminuria is the first signal of kidney impairment in FMF, but new markers such as Neutrophil Gelatinase-Associated Lipocalin (NGAL) may radically change renal outcomes. Serum amyloid A protein (SAA) is currently considered a reliable indicator of subclinical inflammation and compliance to therapy. According to new evidence, SAA may also have an active pathogenic role in the regulation of NALP3 inflammasome activity as well as being a predictor of the clinical course of AA amyloidosis. Beyond colchicine, new monoclonal antibodies such as IL-1 inhibitors anakinra and canakinumab, and anti-IL-6 tocilizumab may represent a key in optimizing FMF treatment and prevention or control of AA amyloidosis. MDPI 2021-10-01 /pmc/articles/PMC8541210/ /pubmed/34684086 http://dx.doi.org/10.3390/medicina57101049 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Siligato, Rossella Gembillo, Guido Calabrese, Vincenzo Conti, Giovanni Santoro, Domenico Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever |
title | Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever |
title_full | Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever |
title_fullStr | Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever |
title_full_unstemmed | Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever |
title_short | Amyloidosis and Glomerular Diseases in Familial Mediterranean Fever |
title_sort | amyloidosis and glomerular diseases in familial mediterranean fever |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8541210/ https://www.ncbi.nlm.nih.gov/pubmed/34684086 http://dx.doi.org/10.3390/medicina57101049 |
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