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