Nephron最新文献

{"title":"Person-Centred Healthcare: Time to Rethink Nephrology Healthcare.","authors":"Ann Bonner, Jeanette Finderup","doi":"10.1159/000548255","DOIUrl":"10.1159/000548255","url":null,"abstract":"","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-3"},"PeriodicalIF":1.8,"publicationDate":"2025-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144962163","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Relationship of Dietary Inflammatory Index with Anemia in Diabetic Kidney Disease: A Cross-Sectional Study of the NHANES.","authors":"Liyin Guo, Tian Wang","doi":"10.1159/000547798","DOIUrl":"10.1159/000547798","url":null,"abstract":"<p><strong>Introduction: </strong>This study examined the relationship between the dietary inflammatory index (DII) and anemia in patients with diabetic kidney disease (DKD).</p><p><strong>Methods: </strong>All the data were obtained from the National Health and Nutrition Examination Survey (NHANES) from 2007 to 2018. The final analysis included 1918 DKD patients, with 500 experiencing anemia. Weighted multivariate logistic regression models were used to assess the association between DII and anemia, with results expressed as odds ratios.</p><p><strong>Results: </strong>Elevated DII scores correlated with an increased incidence of anemia (OR = 1.13, 95% CI: 1.02-1.25). When analyzed as categorical variables, DII scores of 1.57-2.64 (OR = 1.77, 95% CI: 1.13-2.77) and >2.64 (OR = 1.78, 95% CI: 1.12-2.85) were linked to higher anemia risk. Subgroup analyses revealed consistent associations in individuals aged 65 and older (OR = 1.92, 95% CI: 1.16-3.17), those with serum iron levels <73 μg/dL (OR = 2.01, 95% CI: 1.11-3.62), and males (OR = 2.23, 95% CI: 1.21-4.11). Higher DII scores correlated with greater odds of moderate-severe anemia (OR = 1.22, 95% CI: 1.07-1.38).</p><p><strong>Conclusion: </strong>The results indicate that elevated DII scores are associated with an increased occurrence of anemia in DKD patients, emphasizing the importance of lowering pro-inflammatory food consumption as a potential strategy to prevent anemia in this group.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-11"},"PeriodicalIF":1.8,"publicationDate":"2025-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144962187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Effectiveness of Lesion Detection for Immunoglobulin G4-Related Kidney Diseases by Diffusion-Weighted Imaging.","authors":"Hidenori Amaike, Masatoshi Kanda, Hirotsugu Yamazaki, Koki Nakamura, Li Ma, Ken Nagahata, Hiroyuki Nakamura, Arata Osanami, Naoya Yama, Masamitsu Hatakenaka, Masato Furuhashi, Hiroki Takahashi","doi":"10.1159/000547628","DOIUrl":"10.1159/000547628","url":null,"abstract":"<p><strong>Objectives: </strong>The aim of the study was to compare the efficacy of magnetic resonance imaging (MRI) with that of plain or contrast-enhanced computed tomography (CT) in the detection of renal parenchymal and pelvic lesions of immunoglobulin G4-related kidney disease (IgG4-RKD).</p><p><strong>Methods: </strong>Patients with IgG4-RKD and controls, who performed plain, contrast-enhanced CT and MRI around the kidney region in our hospital, were enrolled. The diagnosis of IgG4-RKD was made by definite cases of IgG4-RKD diagnostic criteria in 2020. Five blinded observers independently assessed image datasets by confidence scores to assess diagnostic accuracy, sensitivity, specificity, areas under the receiver operating characteristic curve (AUROC), and Cronbach's alpha coefficient.</p><p><strong>Results: </strong>A total of 31 patients were included in the study. Fourteen (45.2%) had IgG4-RKD. Five patients with IgG4-RKD had parenchymal lesions, 5 had renal pelvic lesions, and 4 had both. In the parenchymal lesions, there was no significant difference in diagnostic performance between contrast-enhanced CT and diffusion-weighted imaging (DWI)-b800. The AUROC and sensitivity were higher in DWI-b800 than in plain CT (p < 0.05). Cronbach's alpha coefficient was 0.44 for plain CT and over 0.80 for contrast-enhanced CT and DWI-b800. In the pelvic lesions, there were fewer differences in the performance among each sequence. Cronbach's alpha coefficient was over 0.80 for plain CT, contrast-enhanced CT, and DWI-b800.</p><p><strong>Conclusion: </strong>Plain MRI, especially in DWI-b800, can effectively detect renal parenchymal lesions in IgG4-RKD. In cases where the use of a contrast agent of CT is difficult, DWI-b800 can be an alternative for the screening of IgG4-RKD.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-11"},"PeriodicalIF":1.8,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144855851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association of Urine-to-Plasma Urea Ratio with Outcomes in Patients with Chronic Kidney Disease: A Retrospective Cohort Study.","authors":"Usha Kumari, Keiichi Sumida, Barry M Wall, Csaba P Kovesdy","doi":"10.1159/000547780","DOIUrl":"10.1159/000547780","url":null,"abstract":"<p><strong>Background: </strong>Estimated glomerular filtration rate (eGFR) and proteinuria are the gold-standard markers for kidney function used traditionally to assess kidney outcomes. Increased evidence of tubule involvement in nearly all causes of chronic kidney disease (CKD) warrants exploring the association between markers of tubular function with clinical outcomes in patients with CKD.</p><p><strong>Methods: </strong>We conducted a single-center retrospective cohort study in 626 patients with eGFR <60 mL/min/1.73 m2. We examined the association between spot urine-to-plasma (U/P) urea and the incidence of kidney failure, all-cause death, and their composite outcome using the Kaplan-Meier method and multivariable-adjusted Cox proportional hazard models.</p><p><strong>Results: </strong>Our cohort predominantly consisted of males (96%) with a mean ± standard deviation age of 67.4 ± 10.8 years and eGFR of 23.6 ± 10.5 mL/min/1.73 m2. There were 448 composite outcome events (event rate: 159/1,000 patient-years; 95% confidence interval [CI]: 145-175) over a median follow-up of 4.5 years. Lower U/P urea was associated with a higher risk of the composite outcome in unadjusted (hazard ratio and 95% CI for 1 log-unit lower U/P urea: 1.98 [1.73-2.26], p < 0.001) and after multivariable adjustment (1.67 [1.37-2.07], p < 001). Associations were similar for kidney failure and for all-cause death.</p><p><strong>Conclusion: </strong>Lower U/P urea, a marker of tubular dysfunction, is associated with a higher risk of adverse clinical outcomes in patients with moderate and advanced CKD, independent of eGFR and proteinuria.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-9"},"PeriodicalIF":1.8,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789607","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinicopathological Investigation of Medial Smooth Muscle Cell Atrophy in Small Arteries in Kidney Allografts.","authors":"Noriyuki Kounoue, Hideyo Oguchi, Masaki Muramatsu, Tetuo Mikami, Yoshihiro Itabashi, Takeshi Kawamura, Yuko Hamasaki, Yutaka Yamaguchi, Ken Sakai","doi":"10.1159/000547777","DOIUrl":"10.1159/000547777","url":null,"abstract":"<p><strong>Introduction: </strong>Atrophy of smooth muscle cells (SMCs) in the media of small arteries is occasionally observed, especially in long-term kidney allograft biopsies. The intima-media ratio is used as an index of arteriosclerosis in native kidneys, and medial SMC atrophy suggests severe arteriosclerosis. We aimed to investigate the clinicopathological significance of medial SMC atrophy in small arteries in biopsies from long-term kidney transplants.</p><p><strong>Methods: </strong>Samples were obtained from kidney allograft biopsies carried out from January 2016 to December 2019, and biopsies obtained 10 years after transplantation were included in the study. The distal small arteries with the most atrophic SMCs in longitudinal sections in each biopsy specimen were selected (outer diameter <150 µm and ≥60 µm). The outer diameter and width of the media, intima, and lumen of each artery were measured. SMC atrophy was evaluated as the media-outer diameter ratio.</p><p><strong>Results: </strong>Fifty biopsies were eligible. The mean media-outer diameter ratio was 0.27 ± 0.11. Donor age and allograft age were significantly inversely correlated with media-outer diameter ratio (rank correlation coefficient -0.3522, p = 0.0141 and -0.3700, p = 0.0096, respectively). After separation into two groups according to the media-outer diameter ratio, donor age and allograft age were both significantly higher and more focal segmental glomerular sclerosis (FSGS) lesions were observed in the low media-outer diameter ratio group. Multivariate analysis revealed that media-outer diameter ratio was significantly related to donor age, allograft age, and FSGS.</p><p><strong>Conclusions: </strong>Medial SMC atrophy appears to be related to donor age, allograft age, and FSGS in kidney allografts. Disruption of vascular contraction as a result of medial SMC atrophy in aging allografts may lead to the development of FSGS.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-8"},"PeriodicalIF":1.8,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"T-Cell Receptor CRαβ⁺CD4^-CD8^- (Double-Negative) T Cells May Predict Pathological Kidney Findings in Patients with Suspected Lupus Nephritis.","authors":"Akinari Sekine, Takehiko Wada, Naoki Sawa, Eiko Hasegawa, Kei Kono, Yuki Oba, Yoshifumi Ubara, Junichi Hoshino, Kenichi Ohashi","doi":"10.1159/000547797","DOIUrl":"10.1159/000547797","url":null,"abstract":"<p><strong>Introduction: </strong>T-cell receptor αβ+CD4-CD8- (double-negative) T cells (DNT) exist as a small population of lymphocytes in the peripheral blood of healthy individuals, but have been reported to be increased in autoimmune lymphoproliferative syndrome (ALPS) and other collagen diseases, such as systemic lupus erythematosus (SLE), and to be involved in disease activity. However, there are no reports on their relationship with histological classification of lupus nephritis, influencing the management of SLE.</p><p><strong>Methods: </strong>The study included 15 patients with SLE who underwent kidney biopsy for suspected lupus nephritis at Toranomon Hospital from June 2014 to March 2019 and also had their peripheral DNT cell levels measured. We defined high DNT as more than 1.5% DNT among peripheral blood lymphocytes, in accordance with the ALPS diagnostic criteria, and divided patients into a high and low DNT group.</p><p><strong>Results: </strong>In the 15 cases analyzed, mild glomerular lesions tended to be more common in patients in the high DNT group than those in the low group (60% vs. 10%, respectively; p = 0.077, Fisher's exact test), as did moderate to severe leukocytic inflammation in the interstitium (100% vs. 20%, respectively; p = 0.004, Fisher's exact test).</p><p><strong>Conclusions: </strong>Elevated peripheral DNT cell levels may be associated with mild glomerular lesions but moderate to severe interstitial inflammation in SLE patients. A prospective clinical study with a larger patient cohort is warranted to validate this possibility and investigate its potential causality.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-8"},"PeriodicalIF":1.8,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12503654/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789610","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Trials for Developing Treatment and Early Diagnostic Methods for Chronic Active Antibody-Mediated Rejection in Kidney Transplantation: A Review.","authors":"Kiyohiko Hotta","doi":"10.1159/000547781","DOIUrl":"10.1159/000547781","url":null,"abstract":"<p><strong>Background: </strong>Development of immunosuppressants has reduced the incidence of acute T-cell-mediated rejection (TCMR) and significantly improved the short-term outcome of kidney transplantation. However, in the long term, many cases of transplanted kidney failure are due to chronic active antibody-mediated rejection (CAAMR) caused by donor-specific antibodies. Overcoming this is essential for improving long-term outcomes. The mechanism of CAAMR remains largely unknown and no effective treatment is currently available.</p><p><strong>Summary: </strong>Various therapeutic approaches have been attempted for cases that have progressed to CAAMR. However, an effective treatment method has yet to be established. Therefore, efforts are underway to develop diagnostic methods for identifying patients at risk of developing CAAMR either before its occurrence or as early as possible. Recent reports have identified potential biomarkers in the urine and blood for the early diagnosis of CAAMR. In addition, the molecular analyses of kidney allograft biopsy tissues are being explored for early diagnostic methods. This review summarizes the current treatments for CAAMR and the trials aimed at developing early-stage diagnostic methods for detecting CAAMR.</p><p><strong>Key messages: </strong>Various new diagnostic methods have successively attempted to replace histological examination in the diagnosis of kidney transplant rejection; most have focused on TCMR or acute antibody-mediated rejection. The development of early diagnostic methods for CAAMR is still in its infancy and future advancements are anticipated.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-8"},"PeriodicalIF":1.8,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789608","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of the 2021 Chronic Kidney Disease-Epidemiology Collaboration Glomerular Filtration Rate Estimating Equation on Risk of Healthcare Utilisation for Hospitalisations for Cardiovascular and Kidney Disease.","authors":"Jiashen Cai, Jia Liang Kwek, Hanis Abdul Kadir, Ngiap Chuan Tan, Andrew Teck Wee Ang, Jason Chon Jun Choo, Chieh Suai Tan, Cynthia Ciwei Lim","doi":"10.1159/000547627","DOIUrl":"10.1159/000547627","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Aim: &lt;/strong&gt;Reduced kidney function is a known risk amplifier for atherosclerotic cardiovascular disease (ASCVD) and adverse kidney events. Accurate assessment of kidney function using estimated glomerular filtration rate (eGFR) is therefore essential for evaluating ASCVD risk and kidney prognosis. We aimed to compare the revised 2021 Chronic Kidney Disease-Epidemiology Collaboration (CKD-EPI) [2021-eGFRcr(AS)] and European Kidney Function Consortium (EKFCcr) with the 2009 CKD-EPI [2009-eGFRcr(ASR)] equations in predicting the risk of hospitalisations for acute myocardial infarction (AMI), acute kidney disease (AKD), and chronic kidney disease (CKD) in a multi-ethnic Asian cohort.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This was a multi-centre, retrospective cohort study of adults who attended the ambulatory clinics at the Singapore General Hospital and SingHealth Polyclinics. Individuals were included if they had at least one serum creatinine and albuminuria result in 2014 and at least one follow-up visit between 2015 and 2018. Demographic data, comorbidities, biochemistry, and hospitalisations were retrieved from electronic medical records. eGFR was calculated using the 2009-eGFRcr(ASR) and 2021-eGFRcr(AS) and EKFCcr equations. Multivariable logistic regression models for the associations between eGFR categories and hospitalisations for AMI, AKD, and CKD were evaluated for their goodness-of-fit and discrimination.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Among 10,137 individuals in the study, the mean age was 65.5 (10.8) years. The mean eGFRs were 85.6 (20.4), 89.3 (20.0), and 79.6 (19.5) mL/min/1.73 m2 according to the 2009-eGFRcr(ASR), 2021-eGFRcr(AS), and EKFCcr equations, respectively. Compared to the 2009-eGFRcr(ASR) equation, 28.8-33.3% of individuals were reclassified to a less severe eGFR category by the 2021-eGFRcr(AS) equation, while 1.6%-36.6% were reclassified to a more severe eGFR category by the EKFCcr equation. Over a mean follow-up of 44.9 (12.6) months, hospitalisations for AMI, AKD, and CKD occurred in 42 (0.4%), 228 (2.4%), and 189 (2.0%) of patients, respectively. More severe eGFR categories were independently associated with all the outcomes. For hospitalisation for CKD, the model with the 2021-eGFRcr(AS) equation had significantly better discrimination (area under the receiver operating characteristic curve difference +0.010 (p = 0.016) and better fit (Vuong Z statistic = -2.175, p = 0.015) compared to the model with the 2009-eGFR(ASR). However, the discrimination and fit of models for predicting AMI and AKI hospitalisations were similar between 2021-eGFRcr(AS) and 2009-eGFR(ASR) equations were similar. The EKFCcr-based models did not demonstrate improved discrimination or fit for hospitalisation for AMI, AKD, and CKD, compared to 2009-eGFRcr(ASR)-based model.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;Lower eGFR ascertained by the 2009-eGFRcr(ASR), 2021-eGFRcr(AS), and EKFCcr equations were independently associated with greater risks","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-13"},"PeriodicalIF":1.8,"publicationDate":"2025-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12503690/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144784810","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Case of Early Occurrence of Post-Transplant Lymphoproliferative Disorders in the Allograft after Kidney Transplantation.","authors":"Takuzo Fujiwara, Moto Tokunaga, Norihiro Kusumi, Naoto Terami, Masashi Kitagawa, Kosuke Ota, Yusuke Takahashi, Yoko Shinou, Tetsuya Isoda","doi":"10.1159/000547697","DOIUrl":"10.1159/000547697","url":null,"abstract":"<p><strong>Introduction: </strong>Post-transplant lymphoproliferative disorder (PTLD) is a serious complication after transplantation (Tx). The incidence pattern appears to be divided into two groups, depending on the onset time, as within 1 or 2 years after Tx and thereafter (early and late, respectively). We report very early onset and rapid progression of PTLD after kidney Tx.</p><p><strong>Case presentation: </strong>A 49-year-old man with a 12-year history of hemodialysis underwent deceased donor kidney Tx from a 62-year-old male donor. Serological examination revealed negative antibodies in the recipient for viral capsid and Epstein-Barr virus (EBV)-associated nuclear antigens. Graft function recovery was delayed, possibly because of recurrent bacterial urinary tract infections, and the patient was weaned from dialysis on day 17. The protocol biopsy on day 37 showed moderate mononuclear cell infiltration in the interstitium with tubulitis. Urine output decreased, and the serum creatinine level rose abruptly at approximately the same time as we initiated steroid pulse therapy. The allograft biopsy was repeated on day 52 and demonstrated large atypical lymphoid cells in the parenchyma. Tumor cells were positive for CD20 and negative for CD3 with immunostaining and positive on EBV-encoded RNA in situ hybridization. The diagnosis was EBV-associated PTLD, histologically diffuse large B-cell lymphoma. Hemodialysis was resumed, and graftectomy was performed on day 58. At 12 months post-graftectomy, the patient was alive on dialysis, and imaging studies had revealed no extra-graft lesions.</p><p><strong>Conclusion: </strong>Early onset and rapid progression of PTLD after Tx should be considered by transplant surgeons and physicians.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-5"},"PeriodicalIF":1.8,"publicationDate":"2025-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12503658/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144784800","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"EXT1/EXT2-Associated Membranous Nephropathy Secondary to TAFRO Syndrome: A Case Report.","authors":"Hisashi Kamido, Yuki Oba, Shigekazu Kurihara, Masayuki Yamanouchi, Tatsuya Suwabe, Kei Kono, Kenichi Ohashi, Shinya Yamamoto, Takahiro Tsuji, Yoshifumi Ubara, Naoki Sawa","doi":"10.1159/000547782","DOIUrl":"10.1159/000547782","url":null,"abstract":"<p><strong>Introduction: </strong>Renal involvement in TAFRO syndrome usually is present as acute kidney injury with oligoproteinuria. Renal pathology is typically characterized by glomerular microangiopathy without immune deposits, and there have been no reports of membranous nephropathy. While idiopathic multicentric Castleman disease (iMCD), which shares a similar pathophysiology with TAFRO syndrome, has documented several cases of membranous nephropathy, the underlying mechanisms remain unclear.</p><p><strong>Case presentation: </strong>We present a case of TAFRO syndrome presenting with nephrotic syndrome, and kidney biopsy revealed exostosin 1/exostosin 2 (EXT1/EXT2)-associated membranous nephropathy. EXT1/EXT2 is considered a potential target antigen in autoimmune membranous nephropathy, suggesting their potential pathogenic role in this case. In iMCD cases with membranous nephropathy, IL-6 levels tend to be slightly low, while VEGF levels are significantly elevated, as seen in the present case. This cytokine profile may contribute to the differences in renal pathological findings and may also be involved in the response to treatment.</p><p><strong>Conclusion: </strong>This case may enhance our understanding of the pathophysiology of membranous nephropathy in TAFRO syndrome and iMCD.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-7"},"PeriodicalIF":1.8,"publicationDate":"2025-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12503695/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144775859","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}