Epidemiological and clinicopathological characteristics of vascular-limited renal AL amyloidosis.

Autor:	Senot N; Department of Internal Medicine, APHP, HôpitalEuropéen Georges Pompidou, 20 rue Leblanc, Paris, France., Gibier JB; Institute of Pathology, Centre Hospitalier Universitaire de Lille, Lille, France., Rabant M; Department of Pathology, Hôpital Necker, APHP, 149 rue de Sèvres, Université Paris Cité, Paris, France., Esteve E; Department of Nephrology, Hôpital Tenon, APHP, Sorbonne Université, INSERM U1155 CORAKID, 4 rue de la Chine, Paris, France., Ferriere E; Department of Nephrology, Hôpital Necker, APHP, 149 rue de Sèvres, Paris, France., Dessaix K; Department of Nephrology, Centre Hospitalier Universitaire de Montpellier, 191 Av. du Doyen Gaston Giraud, Montpellier., Colombat M; Department of Pathology, University Hospital of Toulouse, University Cancer Institute of Toulouse, Toulouse, France., Perrochia H; Pathology Department, Centre Hospitalier Universitaire de Montpellier, Montpellier, France., Olagne J; Department of Nephrology and Transplantation, Strasbourg University Hospital, Strasbourg, France; Department of Pathology, Strasbourg University Hospital, Strasbourg, France., Goujon JM; Department of Pathology, Centre de référence Amylose AL et autres maladies par dépôt d'immunoglobulines monoclonales ; Centre Hospitalier Universitaire de Poitiers, 2 rue de la Milétrie, CS Poitiers., Wayolle N; Néphrologie, Centre Hospitalier de Béthune Beuvry, Béthune, France., Wemeau M; Département d'Hématologie, CH de Roubaix, Roubaix, France., Carpentier B; Hématologie Clinique, Hôpital Saint Vincent de Paul, Lille, France., Pinson P; Unité de Recherche clinique, Hopital Cochin, Assistance Publique-Hopitaux de Paris, Paris, France., Beeker N; Unité de Recherche clinique, Hopital Cochin, Assistance Publique-Hopitaux de Paris, Paris, France., Bridoux F; Department of Nephrology, Centre de référence Amylose AL et autres maladies par dépôt d'immunoglobulines monoclonales ; Centre Hospitalier Universitaire de Poitiers, 2 rue de la Milétrie, CS Poitiers., Cohen C; Department of Nephrology, APHP Hôpital Bichat, 46 Rue Henri Huchard, Paris, INSERM U1149 centre de recherche sur l'inflammation, Université Paris Cité.
Jazyk:	angličtina
Zdroj:	Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association [Nephrol Dial Transplant] 2024 Dec 12. Date of Electronic Publication: 2024 Dec 12.
DOI:	10.1093/ndt/gfae285
Abstrakt:	Background and Hypothesis: Kidney involvement, along with cardiac disease, is the most frequent manifestation of systemic AL amyloidosis usually resulting in nephrotic-range proteinuria. Rarely, deposits predominantly or exclusively affect the intrarenal arterioles or arteries, these vascular-limited forms following a distinct clinical course, but very little is known about these forms. Our work plan at better characterizing renal vascular limited AL amyloidosis. Methods: By mining French Paris hospital database, we found that this unusual phenotype accounts for approximatively 9% of renal AL amyloidosis cases. We retrospectively studied 35 patients with the renal vascular-limited variant of AL amyloidosis on kidney biopsy. Results: All showed predominant or only (n = 21) intra-renal vascular deposits, of lambda isotype in 63%. At diagnosis, median urine protein/creatinine ratio was 0.5 g/g, with serum creatinine of 167 (127-213) µmol/L and estimated glomerular filtration (eGFR) rate of 36.2 (24.3-49.6) ml/min/1,73 m2. Cardiac involvement was present in 67% of cases. A serum and/or urine monoclonal gammopathy was identified in all but one patient and 31 (88%) had an abnormal FLC ratio. Among 28 treated patients, hematological and renal response rates were 75% (including deep hematological response in 43%) and 18%, respectively. Median time from diagnosis to renal event, defined be a composite criterion composed of end-stage renal disease or > 40% decrease in eGFR, was 56 months. Median overall survival (OS) was 59 months, significantly longer in patients who achieved a deep hematological response (178 vs 20 months, p = 0.002). Conclusion: renal vascular limited AL amyloidosis is a probably underdiagnosed disease with markedly reduced eGFR, low-grade proteinuria and severe overall prognosis. Rapid achievement of a deep hematological response is required to preserve long-term renal and patient outcomes. (© The Author(s) 2024. Published by Oxford University Press on behalf of the ERA.)
Databáze:	MEDLINE
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