[fr] La glomérulonéphrite fibrillaire non amyloïde est une pathologie glomérulaire à dépôts fibrillaires, non amyloïdes, composés principalement d'immunoglobulines G polyclonales. Il s'agit d'une glomérulopathie idiopathique responsable de protéinurie sévère, souvent néphrotique et d'insuffisance rénale arrivant à un stade terminal dans 40% des cas à cinq ans. Elle peut prendre différents aspects anatomopathologiques déterminants en terme de pronostic rénal. La négativité des dépôts en coloration rouge Congo et l'épaisseur des fibrilles en microscopie électronique permettent le diagnostic différentiel avec les dépôts amyloïdes. Il n'y a pas de traitement efficace. Après transplantation rénale, la récidive est fréquente avec un pronostic rénal cependant moins péjoratif.
Disciplines :
Urology & nephrology
Author, co-author :
GROSCH, Stéphanie ; Centre Hospitalier Universitaire de Liège - CHU > Néphrologie
Krzesinski, Jean-Marie ; Université de Liège - ULiège > Département des sciences cliniques > Néphrologie
BOVY, Christophe ; Centre Hospitalier Universitaire de Liège - CHU > Néphrologie
Language :
French
Title :
La glomérulonéphrite fibrillaire non amyloïde : une cause rare de syndrome néphrotique
Alternative titles :
[en] Fibrillary nonamyloïd glomerulonephritis : a rare etiology of nephrotic syndrome
Publication date :
2011
Journal title :
Néphrologie et Thérapeutique
ISSN :
1769-7255
eISSN :
1872-9177
Publisher :
Elsevier, Paris, France
Volume :
7
Pages :
494-498
Peer reviewed :
Peer Reviewed verified by ORBi
Commentary :
The fibrillary nonamyloïd glomerulonephritis is a glomerulopathy with fibrillar, nonamyloïd deposits of predominantly polyclonal immunoglobulin G. It is an idiopathic glomerulopathy responsible for heavy proteinuria, generally in the nephrotic range, and renal failure up to end stade in 40% of the cases after five years. The histologic pattern is variable, correlating with renal prognosis. The Congo red-negativity of the deposits and the size of the fibrils on electron microscopy make the differential diagnosis with amyloid deposits. There is no specific therapy. Recurrence in the transplant is frequent, but with better renal prognosis.
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