Nephrology Dialysis Transplantation最新文献

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Hypertensive nephropathy: revisiting the causal link between hypertension and kidney disease. 高血压肾病:重新审视高血压和肾脏疾病之间的因果关系。
IF 4.8 2区 医学
Nephrology Dialysis Transplantation Pub Date : 2025-06-30 DOI: 10.1093/ndt/gfaf014
Rajiv Agarwal
{"title":"Hypertensive nephropathy: revisiting the causal link between hypertension and kidney disease.","authors":"Rajiv Agarwal","doi":"10.1093/ndt/gfaf014","DOIUrl":"10.1093/ndt/gfaf014","url":null,"abstract":"","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":" ","pages":"1270-1272"},"PeriodicalIF":4.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143123267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Correction to: Global health inequalities of chronic kidney disease: a meta-analysis. 修正:慢性肾病的全球健康不平等:一项荟萃分析。
IF 4.8 2区 医学
Nephrology Dialysis Transplantation Pub Date : 2025-06-30 DOI: 10.1093/ndt/gfaf080
{"title":"Correction to: Global health inequalities of chronic kidney disease: a meta-analysis.","authors":"","doi":"10.1093/ndt/gfaf080","DOIUrl":"10.1093/ndt/gfaf080","url":null,"abstract":"","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":" ","pages":"1442"},"PeriodicalIF":4.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12207607/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143991167","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Optimal glucocorticoid therapy in lupus nephritis. 狼疮性肾炎最佳糖皮质激素治疗。
IF 4.8 2区 医学
Nephrology Dialysis Transplantation Pub Date : 2025-06-30 DOI: 10.1093/ndt/gfae294
Gabriel Figueroa-Parra, Mario Bautista-Vargas, Erika Navarro-Mendoza, Alí Duarte-García
{"title":"Optimal glucocorticoid therapy in lupus nephritis.","authors":"Gabriel Figueroa-Parra, Mario Bautista-Vargas, Erika Navarro-Mendoza, Alí Duarte-García","doi":"10.1093/ndt/gfae294","DOIUrl":"10.1093/ndt/gfae294","url":null,"abstract":"<p><p>This review provides an in-depth analysis of glucocorticoid therapy for lupus nephritis (LN), a severe manifestation of systemic lupus erythematosus that affects up to 51.7% of patients. LN significantly increases the risk of mortality and progression to end-stage kidney disease. Glucocorticoids have been central to LN treatment for decades due to their anti-inflammatory properties, but optimal dosing strategies remain uncertain. The review discusses the historical evolution of glucocorticoid use, highlighting the shift from high-dose regimens to combined approaches with immunosuppressants and lower glucocorticoid doses to minimize adverse effects. Mechanistically, glucocorticoids exert effects through genomic and non-genomic pathways, modulating immune responses and metabolism. Long-term use is associated with risks such as infection, osteoporosis, hyperglycemia and cardiovascular disease. The review examines different dosing strategies, including intravenous pulse therapy and oral regimens, and presents evidence of their efficacy and safety. It also explores alternative approaches, such as low-dose and glucocorticoid-free regimens, which show promise but require further study. The review concludes by emphasizing the need for future research to optimize glucocorticoid regimens, refine tapering protocols and identify safer therapeutic combinations, as glucocorticoids remain a cornerstone in LN management despite their challenges.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":" ","pages":"1284-1293"},"PeriodicalIF":4.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142872696","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Stratified medicine for blood pressure targets in type 2 diabetes mellitus. 2型糖尿病患者血压指标的分层药物治疗。
IF 4.8 2区 医学
Nephrology Dialysis Transplantation Pub Date : 2025-06-30 DOI: 10.1093/ndt/gfaf007
Beatriz Fernandez-Fernandez, Jose M Valdivielso, Liffert Vogt, Pantelis Sarafidis, Alberto Ortiz
{"title":"Stratified medicine for blood pressure targets in type 2 diabetes mellitus.","authors":"Beatriz Fernandez-Fernandez, Jose M Valdivielso, Liffert Vogt, Pantelis Sarafidis, Alberto Ortiz","doi":"10.1093/ndt/gfaf007","DOIUrl":"10.1093/ndt/gfaf007","url":null,"abstract":"","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":" ","pages":"1263-1266"},"PeriodicalIF":4.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142979318","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Determination of urine volume and glomerular filtration rate using d-serine and d-asparagine. 使用 d-丝氨酸和 d-天冬酰胺测定尿量和肾小球滤过率。
IF 4.8 2区 医学
Nephrology Dialysis Transplantation Pub Date : 2025-06-30 DOI: 10.1093/ndt/gfae279
Ryo Tanaka, Shinsuke Sakai, Ayumu Taniguchi, Masataka Kawamura, Yoko Higa-Maegawa, Soichi Matsumura, Shota Fukae, Shigeaki Nakazawa, Shihoko Kimura-Ohba, Masaru Horio, Shiro Takahara, Ryoichi Imamura, Norio Nonomura, Masayuki Mizui, Yoshitaka Isaka, Yoichi Kakuta, Tomonori Kimura
{"title":"Determination of urine volume and glomerular filtration rate using d-serine and d-asparagine.","authors":"Ryo Tanaka, Shinsuke Sakai, Ayumu Taniguchi, Masataka Kawamura, Yoko Higa-Maegawa, Soichi Matsumura, Shota Fukae, Shigeaki Nakazawa, Shihoko Kimura-Ohba, Masaru Horio, Shiro Takahara, Ryoichi Imamura, Norio Nonomura, Masayuki Mizui, Yoshitaka Isaka, Yoichi Kakuta, Tomonori Kimura","doi":"10.1093/ndt/gfae279","DOIUrl":"10.1093/ndt/gfae279","url":null,"abstract":"<p><strong>Background: </strong>Measurement of glomerular filtration rate (GFR) is subject to inaccurate urine collection. Clearances of d-serine and d-asparagine, rare enantiomers of amino acids, are the measures of GFR since they are almost free of tubular secretion and reabsorption. We hypothesize that d-serine and d-asparagine can accurately determine urine volume and GFR.</p><p><strong>Methods: </strong>This cross-sectional observational study included 209 living kidney transplant donors and recipients for whom GFR was measured using the clearance of inulin. Assuming that urine excretions of d-serine and d-asparagine are constant and using urine levels of d-serine and d-asparagine from each urine collection, an equation for estimated urine volume (eUV) was established. Based on the eUV, the abnormal urine volume was replaced with an estimate with which the GFR was re-evaluated.</p><p><strong>Result: </strong>Clearances of d-serine and d-asparagine were minor in proportional biases when compared with that of creatinine. Using 627 urine collections, the equation for eUV (mL/min) was established as 21.88/urine d-Ser(0.40 + 0.20 × log10(urine d-Asn)). Using eUV, we identified 20 instances where urine collection volumes varied significantly from the estimated values. After replacement with eUV, measured GFR (mGFR) was corrected to adjusted mGFR, which was within approximately 20 mL/min/1.73 m2 of the mGFR.</p><p><strong>Conclusion: </strong>d-Serine and d-asparagine are nearly completely excreted in urine after glomerular filtration, enabling the estimation of urine volume and correct mGFR. Besides reflecting GFR, d-serine and d-asparagine can be used to estimate urine volume. By applying the eUV method, mGFR determined using clearance methods becomes more accurate.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":" ","pages":"1374-1383"},"PeriodicalIF":4.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142687703","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Donor-derived cell-free DNA monitoring for early diagnosis of antibody-mediated rejection after kidney transplantation: a randomized trial. 供体来源的无细胞DNA监测用于肾移植后抗体介导的排斥反应的早期诊断:一项随机试验。
IF 4.8 2区 医学
Nephrology Dialysis Transplantation Pub Date : 2025-06-30 DOI: 10.1093/ndt/gfae282
Aylin Akifova, Klemens Budde, Kerstin Amann, Maike Buettner-Herold, Mira Choi, Michael Oellerich, Julia Beck, Kirsten Bornemann-Kolatzki, Ekkehard Schütz, Friederike Bachmann, Fabian Halleck, Ellen von Hoerschelmann, Nadine Koch, Eva Schrezenmeier, Evelyn Seelow, Johannes Waiser, Bianca Zukunft, Kai-Uwe Eckardt, Jan Halbritter, Ralph Kettritz, Covadonga López Del Moral, Nils Lachmann, Diana Stauch, Matthias Niemann, Danilo Schmidt, Philip F Halloran, Bilgin Osmanodja
{"title":"Donor-derived cell-free DNA monitoring for early diagnosis of antibody-mediated rejection after kidney transplantation: a randomized trial.","authors":"Aylin Akifova, Klemens Budde, Kerstin Amann, Maike Buettner-Herold, Mira Choi, Michael Oellerich, Julia Beck, Kirsten Bornemann-Kolatzki, Ekkehard Schütz, Friederike Bachmann, Fabian Halleck, Ellen von Hoerschelmann, Nadine Koch, Eva Schrezenmeier, Evelyn Seelow, Johannes Waiser, Bianca Zukunft, Kai-Uwe Eckardt, Jan Halbritter, Ralph Kettritz, Covadonga López Del Moral, Nils Lachmann, Diana Stauch, Matthias Niemann, Danilo Schmidt, Philip F Halloran, Bilgin Osmanodja","doi":"10.1093/ndt/gfae282","DOIUrl":"10.1093/ndt/gfae282","url":null,"abstract":"<p><strong>Background: </strong>Donor-derived cell-free DNA (dd-cfDNA) shows good diagnostic performance for the detection of antibody-mediated rejection (AMR) in kidney transplant recipients (KTR). However, the clinical benefits of dd-cfDNA monitoring need to be established. Early diagnosis of AMR at potentially reversible stages may be increasingly important due to emerging treatment options for AMR. We hypothesized that monitoring dd-cfDNA in KTR with de novo donor-specific anti-HLA antibodies (dnDSA) and performing kidney biopsy in case of increased dd-cfDNA may reduce time to AMR diagnosis in comparison with clinical indication biopsy.</p><p><strong>Methods: </strong>In this diagnostic, single-center, open-label, randomized clinical trial, we assigned 40 KTR with prevalent dnDSA and estimated glomerular filtration rate ≥20 mL/min/1.73 m2, but without previous biopsy-proven AMR, to either dd-cfDNA-guided biopsy (intervention group) or clinician-guided biopsy (control group) over a 12-month period. In both groups, dd-cfDNA was assessed at inclusion and 1, 3, 6, 9 and 12 months. In the intervention group, dd-cfDNA >50 copies/mL indicated a biopsy. Biopsies for clinical indication could be performed at any point during the study period in both groups. A protocol biopsy was scheduled after 12 months for patients without dd-cfDNA-guided biopsy or clinical indication biopsy until study completion. The primary endpoint was time from study inclusion to diagnosis of active or chronic active AMR.</p><p><strong>Results: </strong>Thirty-nine of 40 patients had functioning grafts at study completion. From these, 26 patients underwent biopsy, 13 in each group. AMR was diagnosed earlier in the intervention group than in the control group [median 2.8 months, interquartile range (IQR) 1.7-5.3 vs median 14.5 months, IQR 13.3-16.7, P = .003]. Longitudinal dd-cfDNA monitoring had 77% positive predictive value and 85% negative predictive value for AMR.</p><p><strong>Conclusions: </strong>Dd-cfDNA-guided biopsy in KTR with prevalent dnDSA can reduce the time to AMR diagnosis and hereby expedite therapy initiation.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov, NCT04897438.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":" ","pages":"1384-1395"},"PeriodicalIF":4.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12207606/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142824310","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Challenging the narrative of Alport syndrome spectrum: no link with cystic phenotype. 挑战阿尔波特综合征谱的叙述:与囊性表型无关。
IF 4.8 2区 医学
Nephrology Dialysis Transplantation Pub Date : 2025-06-30 DOI: 10.1093/ndt/gfae290
Marie-Sophie Pagniez, Yannis Lombardi, Victor Fages, Romain Larrue, Timothée Laboux, Clémence Gatinois, Emmanuel Letavernier, Claire Rigothier, François Glowacki, Laurent Mesnard, Thomas Robert
{"title":"Challenging the narrative of Alport syndrome spectrum: no link with cystic phenotype.","authors":"Marie-Sophie Pagniez, Yannis Lombardi, Victor Fages, Romain Larrue, Timothée Laboux, Clémence Gatinois, Emmanuel Letavernier, Claire Rigothier, François Glowacki, Laurent Mesnard, Thomas Robert","doi":"10.1093/ndt/gfae290","DOIUrl":"10.1093/ndt/gfae290","url":null,"abstract":"<p><strong>Background: </strong>Alport syndromes (AS) are the second leading genetic cause of kidney failure. Whether the multiple kidney cysts (MKC) phenotype belongs to the AS spectrum remains debated.</p><p><strong>Methods: </strong>This multicenter retrospective study focused on patients genotyped with pathogenic COL4A3, COL4A4, or COL4A5 variants (classified as ACMG-AMP 4 or 5) between January 2011 and January 2023 across four French university hospitals. The study aimed to compare characteristics between two groups based on the presence or absence of MKC, defined by three or more cysts per kidney. The MKC group was compared to a control group with negative exome sequencing results for undetermined kidney disease (ES-UKD) to assess the association between MKC and AS.</p><p><strong>Results: </strong>Among the 257 AS patients included, 38 (14.8%) presented MKC without variation from hereditary cystic kidney panel. MKC showed a significant association with male gender (P = 0.004), cardiovascular risk factors, and loss of function variants (P = 0.012). Kidney failure onset appeared significantly later, by 6 years, in MKC patients (P = 0.035). Comparison with the ES-UKD (n = 990) control group showed no significant association between AS and MKC by univariate and multivariate analysis. Multivariate analysis identified patient age and male gender (P < 0.001) as factors linked to MKC.</p><p><strong>Conclusions: </strong>A 14.8% prevalence of MKC was found in our cohort of 257 patients with AS. MKC-AS patients exhibited clinical and histological characteristics akin to nephroangiosclerosis. Our comprehensive analysis, incorporating a sizable ES-UKD cohort, revealed no significant association between MKC and AS, thus questioning the inclusion of MKC within the spectrum of AS.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":" ","pages":"1408-1415"},"PeriodicalIF":4.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854571","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Acid excretion is impaired in calcium oxalate stone formers. 草酸钙结石患者的排酸功能受损。
IF 4.8 2区 医学
Nephrology Dialysis Transplantation Pub Date : 2025-06-30 DOI: 10.1093/ndt/gfaf038
Pedro H Imenez Silva, Nasser A Dhayat, Daniel G Fuster, Harald Seeger, Alexander Ritter, Thomas Ernandez, Florian Buchkremer, Beat Roth, Olivier Bonny, Isabel Rubio-Aliaga, Carsten A Wagner
{"title":"Acid excretion is impaired in calcium oxalate stone formers.","authors":"Pedro H Imenez Silva, Nasser A Dhayat, Daniel G Fuster, Harald Seeger, Alexander Ritter, Thomas Ernandez, Florian Buchkremer, Beat Roth, Olivier Bonny, Isabel Rubio-Aliaga, Carsten A Wagner","doi":"10.1093/ndt/gfaf038","DOIUrl":"10.1093/ndt/gfaf038","url":null,"abstract":"","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":" ","pages":"1433-1435"},"PeriodicalIF":4.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12207603/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143468617","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Infiltrative classical monocyte-derived and SPP1 lipid-associated macrophages mediate inflammation and fibrosis in ANCA-associated glomerulonephritis. 浸润性经典单核细胞来源和SPP1脂质相关巨噬细胞介导anca相关性肾小球肾炎的炎症和纤维化。
IF 4.8 2区 医学
Nephrology Dialysis Transplantation Pub Date : 2025-06-30 DOI: 10.1093/ndt/gfae292
Yosta Vegting, Aldo Jongejan, Annette E Neele, Nike Claessen, Gal Sela, Koen H M Prange, Jesper Kers, Joris J T H Roelofs, Joost W van der Heijden, Onno J de Boer, Ester B M Remmerswaal, Liffert Vogt, Frederike J Bemelman, Menno P J de Winther, Perry D Moerland, Marc L Hilhorst
{"title":"Infiltrative classical monocyte-derived and SPP1 lipid-associated macrophages mediate inflammation and fibrosis in ANCA-associated glomerulonephritis.","authors":"Yosta Vegting, Aldo Jongejan, Annette E Neele, Nike Claessen, Gal Sela, Koen H M Prange, Jesper Kers, Joris J T H Roelofs, Joost W van der Heijden, Onno J de Boer, Ester B M Remmerswaal, Liffert Vogt, Frederike J Bemelman, Menno P J de Winther, Perry D Moerland, Marc L Hilhorst","doi":"10.1093/ndt/gfae292","DOIUrl":"10.1093/ndt/gfae292","url":null,"abstract":"<p><strong>Background: </strong>Kidney macrophage infiltration is a histological hallmark of vasculitic lesions and is strongly linked to disease activity in anti-neutrophil cytoplasmic antibodies (ANCA)-associated glomerulonephritis (AGN). The precise mechanisms by which kidney macrophages influence local inflammation and long-term damage remain largely unknown.</p><p><strong>Methods: </strong>Here, we investigate kidney macrophage diversity using single-cell transcriptome analysis of 25 485 freshly retrieved unfrozen, high-quality kidney CD45+ immune cells from five AGN patients during active disease, a lupus nephritis and a nephrectomy control. Detailed subclustering of myeloid cells was performed to identify disease-specific macrophage subtypes. Next, transcriptome differences between macrophage subsets and disease serotypes were assessed. Findings were validated by immunostainings of an extended cohort of kidney biopsies and flow cytometric analysis of peripheral blood monocytes.</p><p><strong>Results: </strong>Four main macrophage subsets were identified, including a classical monocyte-derived macrophage (MDM) subset expressing a chemotactic (CXCL2, CXCL3, CXCL8, CCL3) and pro-inflammatory (IL1β, TNF) set of markers and an osteopontin/SPP1+ lipid-associated macrophage (SPP1 LAMs) subtype exhibiting distinctive upregulation of fibrotic genesets. AGN samples revealed a markedly increased proportion of CD163+ macrophages, predominantly composed of classical MDMs, accompanied by resident-like C1Q macrophages, and SPP1 LAMs. An analogous trend was observed in the expansion of peripheral blood classical monocytes during active disease. The proteinase 3 (PR3)-AGN subtype exhibited heightened classical MDM and SPP1 LAM infiltration and markers of acute inflammation, while interferon signaling and markers of chronicity were reduced compared with myeloperoxidase-AGN.</p><p><strong>Conclusions: </strong>Our findings highlight the expression of inflammatory and fibrotic genes by kidney macrophage subsets in AGN. Classical monocyte dysregulation might contribute to inflammation in the pathogenesis of AGN. Targeting these specific monocyte/macrophage subsets may potentially control the inflammatory cascade and attenuate resulting fibrosis in AGN and kidney disease in general.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":" ","pages":"1416-1427"},"PeriodicalIF":4.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Epidemiological and clinicopathological characteristics of vascular-limited renal AL amyloidosis. 血管局限性肾AL淀粉样变的流行病学和临床病理特征。
IF 4.8 2区 医学
Nephrology Dialysis Transplantation Pub Date : 2025-06-30 DOI: 10.1093/ndt/gfae285
Noémie Senot, Jean Baptiste Gibier, Marion Rabant, Emmanuel Esteve, Elsa Ferriere, Kathleen Dessaix, Magali Colombat, Helene Perrochia, Jerome Olagne, Jean Michel Goujon, Nicolas Wayolle, Mathieu Wemeau, Benjamin Carpentier, Pierre Pinson, Nathanael Beeker, Frank Bridoux, Camille Cohen
{"title":"Epidemiological and clinicopathological characteristics of vascular-limited renal AL amyloidosis.","authors":"Noémie Senot, Jean Baptiste Gibier, Marion Rabant, Emmanuel Esteve, Elsa Ferriere, Kathleen Dessaix, Magali Colombat, Helene Perrochia, Jerome Olagne, Jean Michel Goujon, Nicolas Wayolle, Mathieu Wemeau, Benjamin Carpentier, Pierre Pinson, Nathanael Beeker, Frank Bridoux, Camille Cohen","doi":"10.1093/ndt/gfae285","DOIUrl":"10.1093/ndt/gfae285","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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 181 (133-216) 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 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 >40% decrease in eGFR, was 56 months. Median overall survival was 59 months-significantly longer in patients who achieved a deep hematological response (178 vs 20 months, P = .002).</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":" ","pages":"1396-1407"},"PeriodicalIF":4.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142818666","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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