{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T23:42:47Z","timestamp":1761176567796,"version":"build-2065373602"},"reference-count":0,"publisher":"Oxford University Press (OUP)","issue":"Supplement_3","license":[{"start":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T00:00:00Z","timestamp":1759276800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,10,21]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Transthyretin amyloidosis (ATTR) is a progressive disease caused by multisystemic deposition of transthyretin (TTR), affecting organs such as the heart and kidneys. Gene variants that lead to TTR instability cause hereditary ATTR (ATTRv). The progression of heart failure, often with preserved ejection fraction (HfpEF), can exacerbate chronic kidney disease (CKD), further compromising long-term survival and limiting therapeutic options.<\/jats:p>\n                    <jats:p>The dysautonomia characteristic of hereditary ATTR presents challenges for standard therapies, limiting the use of ACE inhibitors and ARBs due to the risk of hypotension. Additionally, the use of SGLT2 inhibitors is restricted because neurogenic bladder predisposes patients to urinary tract infections. Currently, there is no data available regarding the use of non-steroidal mineralocorticoid receptor antagonists (nsMRA) in ATTRv.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>This study evaluated the impact of finerenone, added to standard of care therapy, in ATTR V30M patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients with ATTR V30M amyloidosis were initiated on finerenone (10 mg daily). Outcomes assessed included estimated glomerular filtration rate (eGFR by cystatin and combined with cystatin and creatinine), proteinuria markers (albumin-creatinine ratio (ACR) and protein-creatinine ratio (PCR)), kidney failure risk equation (KFRE) at 2 and 5 years (Y), renal score of the FASTEX model (usually applied in Fabry disease), potassium levels, cardiac biomarkers (NT-proBNP), heart amyloid load estimated by Tc-99-DPD scintigraphy, and Gillmore score (GS) for ATTR cardiomyopathy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The cohort included 21 patients (9 males) with a mean age of 42.81 \u00b1 15.73 years and mean disease course of 18.33 \u00b1 9.44 years. Three patients (14.29%) had diabetes. Neurogenic bladder was present in 11 patients (52.38%). All patients had echocardiograms with preserved ejection fractions, and 10 patients (47.62%) had positive DPD scintigraphy. Anti-amyloid therapy included liver transplantation (N\u00a0=\u00a013; 61.90%), tafamidis (N\u00a0=\u00a05; 23.81%), and patisiran (N\u00a0=\u00a03; 14.29%). The mean follow-up period with finerenone was 5.44 \u00b1 2.82 months.<\/jats:p>\n                    <jats:p>Table 1 shows that kidney function remained stable after finerenone initiation: combined eGFR (41.6 \u00b1 14.7 vs 42.35 \u00b1 14.18 mL\/min, P\u00a0=\u00a00.615) and cystatin eGFR (36.05 \u00b1 13.07 vs 36.80 \u00b1 13.81 mL\/min, P\u00a0=\u00a00.623). Proteinuria had a trend towards reduction: ACR (518.30 \u00b1 985.70 vs 369.09 \u00b1 870.72 mg\/g, P\u00a0=\u00a00.054) and PCR (0.89 \u00b1 1.39 vs 0.63 \u00b1 1.14 mg\/mmol, P\u00a0=\u00a00.023). Kidney failure risk scores showed no significant changes: KFRE 2Y (3.51 \u00b1 5.15% vs 2.99 \u00b1 5.65%, P\u00a0=\u00a00.394) and KFRE 5Y (11.42 \u00b1 15.55% vs 9.62 \u00b1 16.88%, P\u00a0=\u00a00.300). However, the renal score FASTEX significantly improved (5.15 \u00b1 1.90 vs 4.55 \u00b1 1.82, P\u00a0=\u00a00.002). Potassium levels remained consistent (4.51 \u00b1 0.65 vs 4.59 \u00b1 0.70 mmol\/L, P\u00a0=\u00a00.579) despite finerenone.<\/jats:p>\n                    <jats:p>Cardiac biomarkers (NT-pro BNP) did not change significantly (1513.95 \u00b1 2172.93 vs 1163.00 \u00b1 929.20 pg\/mL, P\u00a0=\u00a00.329). Gillmore score at baseline categorized 6 patients at score I, 12 patients at score II, and 2 patients at score III. After finerenone initiation, 2 patients improved (II to I and III to II), while 1 patient worsened (II to III) due to kidney function deterioration.<\/jats:p>\n                    <jats:p>Two patients discontinued therapy due to hyperkalemia and intercurrent acute kidney injury. One patient transiently discontinued finerenone due to suprapubic discomfort. No major adverse events, such as hypotension or urinary tract infections, were reported.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In ATTRv, finerenone has shown efficacy in kidney protection, including in the setting of heart failure with preserved ejection fraction (HfpEF). It significantly reduces proteinuria (PCR) while demonstrating good tolerability, avoiding the deleterious hypotension associated with dysautonomia and minimizing complications related to neurogenic bladder. Furthermore, its use does not lead to significant changes in potassium levels. In addition to amyloid-modifying therapies, finerenone represents a promising strategy for safeguarding the kidney-heart axis in ATTR amyloidosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ndt\/gfaf116.0265","type":"journal-article","created":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T10:20:38Z","timestamp":1761128438000},"source":"Crossref","is-referenced-by-count":0,"title":["#2968 Hereditary transthyretin amyloidosis (ATTRv) finerenone along the spectrum of kidney and heart disease"],"prefix":"10.1093","volume":"40","author":[{"given":"Ana Cristina","family":"Cunha","sequence":"first","affiliation":[{"name":"Unidade Local de Sa\u00fade Santo Ant\u00f3nio, Porto, Portugal Department of Nephrology,"}]},{"given":"Jo\u00e3o","family":"Bessa","sequence":"additional","affiliation":[{"name":"Unidade Local de Sa\u00fade Santo Ant\u00f3nio, Porto, Portugal Department of Nephrology,"}]},{"given":"Andreia","family":"Campos","sequence":"additional","affiliation":[{"name":"Unidade Local de Sa\u00fade Santo Ant\u00f3nio, Porto, Portugal Department of Nephrology,"}]},{"given":"Jos\u00e9 Pedro","family":"Escaleira","sequence":"additional","affiliation":[{"name":"Unidade Local de Sa\u00fade de Santo Ant\u00f3nio (ULS Santo Ant\u00f3nio), Porto, Portugal Department of Nuclear Medicine,"},{"name":"UMIB\u2014Unit for Multidisciplinary Research in Biomedicine, ICBAS \u2013 School of Medicine and Biomedical Sciences, University of Porto (UP), Porto, Portugal"},{"name":"ITR\u2013Laboratory for Integrative and Translational Research in Population Health, UP, Porto, Portugal"}]},{"given":"Josefina","family":"Santos","sequence":"additional","affiliation":[{"name":"Unidade Local de Sa\u00fade Santo Ant\u00f3nio, Porto, Portugal Department of Nephrology,"}]},{"given":"Lu\u00edsa Correia Lopes","family":"Lobato","sequence":"additional","affiliation":[{"name":"Unidade Local de Sa\u00fade Santo Ant\u00f3nio, Porto, Portugal Department of Nephrology,"},{"name":"UMIB\u2014Unit for Multidisciplinary Research in Biomedicine, ICBAS \u2013 School of Medicine and Biomedical Sciences, University of Porto (UP), Porto, Portugal"},{"name":"ITR\u2013Laboratory for Integrative and Translational Research in Population Health, UP, Porto, Portugal"},{"name":"Unidade Corino de Andrade, ULS Santo Ant\u00f3nio, Porto, Portugal"}]}],"member":"286","published-online":{"date-parts":[[2025,10,21]]},"container-title":["Nephrology Dialysis Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ndt\/article-pdf\/40\/Supplement_3\/gfaf116.0265\/64834266\/gfaf116.0265.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ndt\/article-pdf\/40\/Supplement_3\/gfaf116.0265\/64834266\/gfaf116.0265.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T10:20:38Z","timestamp":1761128438000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ndt\/article\/doi\/10.1093\/ndt\/gfaf116.0265\/8294774"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10]]},"references-count":0,"journal-issue":{"issue":"Supplement_3","published-online":{"date-parts":[[2025,10,21]]},"published-print":{"date-parts":[[2025,10,21]]}},"URL":"https:\/\/doi.org\/10.1093\/ndt\/gfaf116.0265","relation":{},"ISSN":["0931-0509","1460-2385"],"issn-type":[{"value":"0931-0509","type":"print"},{"value":"1460-2385","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2025,10]]},"published":{"date-parts":[[2025,10]]},"article-number":"gfaf116.0265"}}