eprintid: 10206040
rev_number: 7
eprint_status: archive
userid: 699
dir: disk0/10/20/60/40
datestamp: 2025-03-13 12:21:26
lastmod: 2025-03-13 12:21:26
status_changed: 2025-03-13 12:21:26
type: article
metadata_visibility: show
sword_depositor: 699
creators_name: Allinovi, Marco
creators_name: Trivioli, Giorgio
creators_name: Gaudio, Costanza
creators_name: L'Imperio, Vincenzo
creators_name: Rauf, Muhammad U
creators_name: Gillmore, Julian D
title: The evolving spectrum of kidney amyloidosis: advances in diagnosis, typing and treatment
ispublished: inpress
divisions: UCL
divisions: B02
divisions: C10
divisions: D17
divisions: G90
keywords: ALECT2, amyloidosis, daratumumab, MGRS, proteomics, kidney
transplantation
note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
abstract: Kidney amyloidosis encompasses a spectrum of heterogeneous conditions in which damage is caused by the deposition of various misfolded proteins that aggregate into fibrils. The main form of renal amyloidosis in the Western countries is immunoglobulin light chain (AL) amyloidosis, which is usually secondary to a plasma cell clone or less frequently a B cell clone, while rarer causes include AA amyloidosis, ALECT2 and hereditary amyloidoses. The main renal manifestations include nephrotic syndrome and kidney dysfunction with modest or absent proteinuria. The course is progressive and renal and overall survival is reduced in many patients. While biopsies are usually positive by Congo Red staining in all types of amyloidosis, precise identification of the amyloid fibril protein is essential and is best achieved with immunohistochemistry or proteomic studies, such as mass spectrometry. This method also allows the discovery of novel amyloidogenic proteins and has contributed to expand the list of amyloid types. The current treatment strategy is based on suppressing new amyloid fibril production through chemotherapy in AL amyloidosis, control of inflammation in AA amyloidosis and “gene silencing” therapies in hereditary forms, such as the one linked with transthyretin. Novel approaches aim at enhancing natural amyloid clearance in order to reduce the rate of organ failure. Kidney transplantation in patients who achieved response has shown outcomes comparable to the general transplant population. In this review, we present the key aspects of renal amyloidosis and discuss novel concepts in this evolving field.
date: 2025-02-26
date_type: published
publisher: Oxford University Press (OUP)
official_url: https://doi.org/10.1093/ndt/gfaf042
full_text_type: other
language: eng
verified: verified_manual
elements_id: 2366402
doi: 10.1093/ndt/gfaf042
medium: Print-Electronic
pii: 8043263
lyricists_name: Gillmore, Julian
lyricists_id: JGILL78
actors_name: Flynn, Bernadette
actors_id: BFFLY94
actors_role: owner
full_text_status: restricted
publication: Nephrology Dialysis Transplantation
article_number: gfaf042
event_location: England
issn: 0931-0509
citation:        Allinovi, Marco;    Trivioli, Giorgio;    Gaudio, Costanza;    L'Imperio, Vincenzo;    Rauf, Muhammad U;    Gillmore, Julian D;      (2025)    The evolving spectrum of kidney amyloidosis: advances in diagnosis, typing and treatment.                   Nephrology Dialysis Transplantation      , Article gfaf042.  10.1093/ndt/gfaf042 <https://doi.org/10.1093/ndt%2Fgfaf042>.    (In press).   
 
document_url: https://discovery.ucl.ac.uk/id/eprint/10206040/1/gfaf042.pdf