[Fibrillary glomerulonephritis].

La glomérulonéphrite fibrillaire.
DNAJB9 Fibrillary glomerulonephritis Glomérulonéphrite fibrillaire Nephrotic syndrome Syndrome néphrotique

Journal

La Revue de medecine interne
ISSN: 1768-3122
Titre abrégé: Rev Med Interne
Pays: France
ID NLM: 8101383

Informations de publication

Date de publication:
15 May 2024
Historique:
received: 11 12 2023
revised: 15 04 2024
accepted: 02 05 2024
medline: 17 5 2024
pubmed: 17 5 2024
entrez: 16 5 2024
Statut: aheadofprint

Résumé

Fibrillary glomerulonephritis (FGN) is a glomerular disease described since 1977, with a prevalence in renal biopsies of less than 1%. It presents as renal failure, proteinuria, haematuria and hypertension in middle-aged adults. It is defined histologically, using light microscopy, which reveals organised deposits of fibrils measuring around 20nm, which are negative for Congo red staining. Electron microscopy, the first gold standard for diagnosis, has now been superseded by immunohistochemistry using the anti-DNAJB9 antibody. The discovery of this molecule has revolutionised the diagnosis of GNF, thanks to its excellent sensitivity and specificity (98% and 99% respectively). The association of GNF with hepatitis C virus, autoimmune diseases, neoplasia or haemopathy is debated. Renal prognosis is guarded, with 50% of patients progressing to end-stage renal failure within 2 to 4years of diagnosis. In the absence of randomised controlled trials, the recommended treatment is based on nephroprotective measures, corticosteroid therapy and possibly a second-line immunosuppressant such as rituximab. After renal transplantation, recovery or recurrence is possible. The pathophysiology of the disease is still poorly understood, and further studies are needed.

Identifiants

pubmed: 38755072
pii: S0248-8663(24)00567-8
doi: 10.1016/j.revmed.2024.05.005
pii:
doi:

Types de publication

English Abstract Journal Article Review

Langues

fre

Sous-ensembles de citation

IM

Informations de copyright

Copyright © 2024 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

Auteurs

C Dumas De La Roque (C)

Département de médecine interne et immunologie clinique, hôpital de la Pitié-Salpêtrière, 75013 Paris, France; Centre de référence maladies auto-immunes systémiques rares, Paris, France; Sorbonne université, Paris, France.

I Brocheriou (I)

Service d'anatomie pathologique, hôpital de la Pitié-Salpêtrière, 75013 Paris, France; Sorbonne université, Paris, France.

A Mirouse (A)

Département de médecine interne et immunologie clinique, hôpital de la Pitié-Salpêtrière, 75013 Paris, France; Centre de référence maladies auto-immunes systémiques rares, Paris, France; Sorbonne université, Paris, France.

P Cacoub (P)

Département de médecine interne et immunologie clinique, hôpital de la Pitié-Salpêtrière, 75013 Paris, France; Centre de référence maladies auto-immunes systémiques rares, Paris, France; Sorbonne université, Paris, France.

A Le Joncour (A)

Département de médecine interne et immunologie clinique, hôpital de la Pitié-Salpêtrière, 75013 Paris, France; Centre de référence maladies auto-immunes systémiques rares, Paris, France; Sorbonne université, Paris, France. Electronic address: Alexandre.lejoncour@aphp.fr.

Classifications MeSH