{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T14:29:36Z","timestamp":1790173776098,"version":"4.1.0"},"reference-count":37,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2024,12,12]],"date-time":"2024-12-12T00:00:00Z","timestamp":1733961600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Association Fran\u00e7aise Contre l'Amylose"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,6,30]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>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, with these vascular-limited forms following a distinct clinical course, but very little is known about these forms. Our work planned to better characterize renal vascular-limited AL amyloidosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>By mining a 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.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>All showed predominant or only (n\u00a0=\u00a021) intra-renal vascular deposits, of lambda isotype in 63%. At diagnosis, median urine protein\/creatinine ratio was 0.5\u00a0g\/g, with serum creatinine of 181 (133\u2013216) \u00b5mol\/L and estimated glomerular filtration (eGFR) rate of 36.2 (24.3\u201349.6) 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 free light chain 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 &amp;gt;40% decrease in eGFR, was 56\u00a0months. Median overall survival was 59\u00a0months\u2014significantly longer in patients who achieved a deep hematological response (178 vs 20\u00a0months, P\u00a0=\u00a0.002).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>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.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ndt\/gfae285","type":"journal-article","created":{"date-parts":[[2024,12,13]],"date-time":"2024-12-13T07:07:16Z","timestamp":1734073636000},"page":"1396-1407","source":"Crossref","is-referenced-by-count":4,"title":["Epidemiological and clinicopathological characteristics of vascular-limited renal AL amyloidosis"],"prefix":"10.1093","volume":"40","author":[{"given":"No\u00e9mie","family":"Senot","sequence":"first","affiliation":[{"name":"H\u00f4pitalEurop\u00e9en Georges Pompidou Department of Internal Medicine, APHP, , Paris ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean Baptiste","family":"Gibier","sequence":"additional","affiliation":[{"name":"Centre Hospitalier Universitaire de Lille Institute of Pathology, , Lille 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