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Néphrologie & thérapeutique, v.13 no.4, 2017년, pp.258 - 264
Stojanovic, Katia Stankovic (Service de mé) , Georgin-Lavialle, Sophie (decine interne, centre de ré) , Grateau, Gilles (fé)
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Abstract AA amyloidosis remains one of the three main types of systemic amyloidosis with AL and ATTR. Its incidence has been however decreasing recently in Western countries. Chronic inflammatory diseases are currently the first cause of AA amyloidosis, including rheumatoid arthritis, spondyloarthr...
Résumé L’amylose AA reste l’une des trois grandes variétés d’amylose multisystémique, avec l’amylose AL et les formes héréditaires. Son incidence semble toutefois diminuer dans les pays occidentaux, où les maladies inflammatoires sont la principale cause d’amylose, au premier rang desquelles se tient la polyarthrite rhumatoïde, suivie par la spondylarthrite ankylosante et les syndromes auto-inflammatoires. Parmi les tumeurs, il faut signaler la maladie de Castleman, dont l’ablation chirurgicale permet parfois la résolution des symptômes de l’amylose. Une inflammation prolongée est un prérequis au développement d’une amylose AA ; la protéine majoritaire des dépôts est un fragment de la protéine serum amyloid A (SAA), l’une des protéines de la réaction inflammatoire. Cependant, d’autres facteurs, notamment génétiques, sont impliqués dans la susceptibilité à la survenue de l’amylose AA. La néphropathie est la principale manifestation clinique de l’amylose de type AA. La recherche de protéinurie et la mesure de la créatinine plasmatique restent les éléments de dépistage de l’amylose au cours de toute maladie qui comporte une inflammation chronique. Le diagnostic précis de l’amylose AA nécessite de confronter l’ensemble des données cliniques et histologiques et notamment de l’immunohistochimie, afin de ne pas la confondre avec l’une des autres variétés. Une fois installée, l’amylose AA reste de mauvais pronostic en raison de l’insuffisance rénale terminale, qui est le terme ultime de la néphropathie, et de l’atteinte digestive qui s’accompagne de dénutrition profonde. Les traitements actuels sont ciblés sur la maîtrise de l’inflammation ; des médicaments inhibant l’interaction de la protéine AA aux composants communs de l’amylose (composant amyloïde P et héparane sulfate) sont en cours d’évaluation.
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