Glomerular diseases最新文献

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Comparison between Old and New GFR Estimating Equations in Children and Adults with Glomerular Disease in the NEPTUNE Study. NEPTUNE研究中儿童和成人肾小球疾病中新旧GFR估算方程的比较
Glomerular diseases Pub Date : 2025-04-15 eCollection Date: 2025-01-01 DOI: 10.1159/000545934
Qian Liu, Valerie Owusu-Hienno, Abigail R Smith, Cathie Spino, Laura H Mariani, Jarcy Zee
{"title":"Comparison between Old and New GFR Estimating Equations in Children and Adults with Glomerular Disease in the NEPTUNE Study.","authors":"Qian Liu, Valerie Owusu-Hienno, Abigail R Smith, Cathie Spino, Laura H Mariani, Jarcy Zee","doi":"10.1159/000545934","DOIUrl":"10.1159/000545934","url":null,"abstract":"<p><strong>Introduction: </strong>New equations developed in the USA for estimating glomerular filtration rate (GFR) eliminated race for adults and widened the age range for children and young adults. The European Kidney Function Consortium (EKFC) equation was also validated and updated for a US adult population. The aftereffects of adopting these new equations on previous research results among patients with glomerular disease are unknown. This study compared eGFR using old and new estimating equations and their impacts on eGFR-based outcomes.</p><p><strong>Methods: </strong>Longitudinal serum creatinine measurements from children and adults enrolled in the Nephrotic Syndrome Study Network (NEPTUNE) were used to calculate eGFR using old bedside Schwartz and CKD-EPI 2009 equations, new U25 and race-free CKD-EPI 2021 equations, and the EKFC equation. Time to disease progression (40% eGFR decline or kidney failure) outcomes were compared using Kaplan-Meier curves and Cox models and longitudinal eGFR outcomes were compared using linear mixed-effects models to assess effects of demographics, clinical characteristics, pathology descriptors, a serum and urine biomarker, and the APOL1 genetic trait.</p><p><strong>Results: </strong><i>N</i> = 756 NEPTUNE study participants were included (median age 21 years, 41% female, and 25% who reported Black race). Disease progression outcomes were similar between using old versus new age-specific equations, whereas event rates were lower using EKFC. Survival curves were largely overlapping, and selected risk factor effects on disease progression were similar. Only sex and race effects on longitudinal eGFR differed between old versus new age-specific equations, whereas larger differences were observed for disease diagnosis effects when using EKFC.</p><p><strong>Conclusion: </strong>New U25 and race-free CKD-EPI 2021 equations had little impact on estimated GFR values and results of survival and longitudinal regression analyses. EKFC results differed and were likely driven by those with very high eGFR.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"219-232"},"PeriodicalIF":0.0,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12105834/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144152609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Glomerular Hematuria for the Diagnosis of Glomerulonephritis. 肾小球血尿对肾小球肾炎的诊断价值。
Glomerular diseases Pub Date : 2025-04-10 eCollection Date: 2025-01-01 DOI: 10.1159/000545051
Anabella Stark, Swetha R Kanduri, Akanksh Ramanand, Sarah Rosenbloom, Vipin Varghese, Dustin R Chalmers, Serenella A Velez, Carolina Gonzalez-Fuentes, Terrance J Wickman, Muner Mohamed, Ali Shueib, Ayaa Zarm, Ivo Lukitsch, Cruz Velasco-Gonzalez, Jay R Seltzer, Juan Carlos Q Velez
{"title":"Glomerular Hematuria for the Diagnosis of Glomerulonephritis.","authors":"Anabella Stark, Swetha R Kanduri, Akanksh Ramanand, Sarah Rosenbloom, Vipin Varghese, Dustin R Chalmers, Serenella A Velez, Carolina Gonzalez-Fuentes, Terrance J Wickman, Muner Mohamed, Ali Shueib, Ayaa Zarm, Ivo Lukitsch, Cruz Velasco-Gonzalez, Jay R Seltzer, Juan Carlos Q Velez","doi":"10.1159/000545051","DOIUrl":"10.1159/000545051","url":null,"abstract":"<p><strong>Introduction: </strong>Reports on the performance of glomerular hematuria for the diagnosis of glomerulonephritis (GN) show heterogeneity in the results and used urological pathologies as controls. We hypothesized that identification of urinary acanthocytes (uACANTHO) and/or urinary red blood cell casts (uRBCCs) by comprehensive microscopic examination of the urinary sediment (uMICRO) can differentiate glomerular disease from non-glomerular renal pathology.</p><p><strong>Methods: </strong>Records of patients seen for consultation for acute kidney injury or proteinuria/hematuria who had specimens examined by uMICRO and a kidney biopsy performed within 2 weeks of uMICRO were extracted. We assessed the sensitivity (SENS), specificity (SPEC), and positive and negative predictive value (PPV, NPV) of uACANTHO and/or uRBCC for the diagnosis of biopsy-proven GN or for any glomerulopathy (GP).</p><p><strong>Results: </strong>Of 915 patients who completed uMICRO, 276 patients were included (mean age 53, 54% women). Median serum creatinine was 3.5 mg/dL. A total of 219 (79%) were categorized as GP, whereas 57 (21%) had non-GP diagnosis (e.g., tubular). Within the GP category, 114 (41%) had GN (e.g., IgA nephropathy, pauci-immune GN), whereas 105 (38%) had non-GN GP (e.g., podocytopathies). The SENS, SPEC, PPV, and NPV of uACANTHO for diagnosing GN were 68%, 86%, 78%, and 79%, respectively, whereas for GP SENS, SPEC, PPV, and NPV were 45%, 100%, 100%, and 32%, respectively. For GN, combining uACANTHO and/or uRBCC resulted in improvement of the SENS, SPEC, PPV, and NPV to 75%, 86%, 79%, and 83%, respectively. Either uACANTHO or uRBCC were found in 47/51 (92%) cases of crescentic/necrotizing GN.</p><p><strong>Conclusion: </strong>Identification of glomerular hematuria by uMICRO aids in the diagnosis of GN. Combining the identification of uACANTHO and uRBCC enhances the diagnostic yield of uMICRO for GN and offers good NPV for crescentic/necrotizing GN. uACANTHO are pathognomonic for GP.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"206-218"},"PeriodicalIF":0.0,"publicationDate":"2025-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12097761/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144129695","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Quo Vadis Standardization of Anti-Nephrin Antibody Detection? 抗肾素抗体检测的标准化现状?
Glomerular diseases Pub Date : 2025-04-03 eCollection Date: 2025-01-01 DOI: 10.1159/000545706
Safak Mirioglu, Annette Bruchfeld, Fernando Caravaca-Fontan, Jürgen Floege, Eleni Frangou, Sarah M Moran, Kate I Stevens, Y K Onno Teng, Stefanie Steiger, Andreas Kronbichler
{"title":"Quo Vadis Standardization of Anti-Nephrin Antibody Detection?","authors":"Safak Mirioglu, Annette Bruchfeld, Fernando Caravaca-Fontan, Jürgen Floege, Eleni Frangou, Sarah M Moran, Kate I Stevens, Y K Onno Teng, Stefanie Steiger, Andreas Kronbichler","doi":"10.1159/000545706","DOIUrl":"https://doi.org/10.1159/000545706","url":null,"abstract":"","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"200-205"},"PeriodicalIF":0.0,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12058110/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144013895","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Updates on Glomerular Diseases: A Summary of Inaugural GlomCon Hawaii 2024. 肾小球疾病的最新进展:首届夏威夷肾小球大会总结
Glomerular diseases Pub Date : 2025-04-03 eCollection Date: 2025-01-01 DOI: 10.1159/000543863
Niloufar Ebrahimi, Ali Poyan Mehr, Harish Seethapathy, Ayman Al Jurdi, Mohamed Hassanein, Vinay Srinivasan, Zainab Obaidi, Edgar Lerma, Sayna Norouzi
{"title":"Updates on Glomerular Diseases: A Summary of Inaugural GlomCon Hawaii 2024.","authors":"Niloufar Ebrahimi, Ali Poyan Mehr, Harish Seethapathy, Ayman Al Jurdi, Mohamed Hassanein, Vinay Srinivasan, Zainab Obaidi, Edgar Lerma, Sayna Norouzi","doi":"10.1159/000543863","DOIUrl":"10.1159/000543863","url":null,"abstract":"","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"158-167"},"PeriodicalIF":0.0,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11968094/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143782180","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Kidney Outcomes with Corticosteroid Treatment in IgA Nephropathy According to the Oxford-MEST-C Classification. 根据Oxford-MEST-C分级,IgA肾病皮质类固醇治疗的肾脏预后。
Glomerular diseases Pub Date : 2025-03-20 eCollection Date: 2025-01-01 DOI: 10.1159/000545382
Bancha Satirapoj, Thapana Chueaboonchai, Naowanit Nata, Ouppatham Supasyndh
{"title":"Kidney Outcomes with Corticosteroid Treatment in IgA Nephropathy According to the Oxford-MEST-C Classification.","authors":"Bancha Satirapoj, Thapana Chueaboonchai, Naowanit Nata, Ouppatham Supasyndh","doi":"10.1159/000545382","DOIUrl":"https://doi.org/10.1159/000545382","url":null,"abstract":"<p><strong>Introduction: </strong>Despite optimization of renin-angiotensin-aldosterone system (RAAS) inhibition, patients with IgA nephropathy remain at risk for kidney failure. The effect of steroids on kidney outcomes in IgA nephropathy with different renal pathologic lesions has been uncertain.</p><p><strong>Objective: </strong>This study aimed to evaluate the efficacy of steroid treatment in IgA nephropathy patients classified according to the Oxford-MEST-C classification.</p><p><strong>Methods: </strong>We retrospectively studied 67 patients with biopsy-proven IgA nephropathy who were receiving optimized RAAS inhibitor therapy and had persistent proteinuria >1 g/day between January 2016 and December 2020. Clinical parameters, including estimated glomerular filtration rate (GFR) decline, were compared between the corticosteroid and supportive treatment groups.</p><p><strong>Results: </strong>Overall, 68.7% of patients received treatment with corticosteroids. The median estimated GFR decline was significantly lower in the steroid group compared to the controls {-0.65 (interquartile range [IQR] -3.45 to 7) vs. -5.75 (IQR -10.65 to -0.7) mL/min/1.73 m<sup>2</sup>/year, <i>p</i> = 0.025}. The slope of estimated GFR was also significantly different between the steroid and control groups in patients with a baseline GFR >50 mL/min/1.73 m<sup>2</sup> (3.90 ± 11.42 vs. -9.31 ± 5.08 mL/min/1.73 m<sup>2</sup>/year, <i>p</i> = 0.011), mesangial hypercellularity M0 score (4.69 ± 11.37 vs. -2.63 ± 6.42 mL/min/1.73 m<sup>2</sup>/year, <i>p</i> = 0.049), and C0 score (2.48 ± 12.63 vs. -5.58 ± 8.4 mL/min/1.73 m<sup>2</sup>/year, <i>p</i> = 0.026). Additionally, rapid GFR decline (>5 mL/min/1.73 m<sup>2</sup>/year) occurred in 9 patients (19.6%) in the steroid group compared with 11 participants (52.4%) in the control group (<i>p</i> = 0.006).</p><p><strong>Conclusion: </strong>Corticosteroid therapy, in addition to optimized RAAS inhibition, lowers the risk of kidney disease progression in patients with IgA nephropathy, particularly those with a baseline GFR >50 mL/min/1.73 m<sup>2</sup> and those classified with Oxford scores M0 and C0.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"191-199"},"PeriodicalIF":0.0,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12043279/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143993755","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Diagnostic Immunostaining of Renal Biopsies: An Overview of Markers for Glomerular Diseases. 肾活检诊断免疫染色:肾小球疾病标志物综述
Glomerular diseases Pub Date : 2025-03-18 eCollection Date: 2025-01-01 DOI: 10.1159/000545311
Vinita Agrawal, Alok Sharma
{"title":"Diagnostic Immunostaining of Renal Biopsies: An Overview of Markers for Glomerular Diseases.","authors":"Vinita Agrawal, Alok Sharma","doi":"10.1159/000545311","DOIUrl":"https://doi.org/10.1159/000545311","url":null,"abstract":"<p><strong>Background: </strong>The analysis of a renal biopsy is made complex by multifactorial etiologies involving different renal compartments. Recent proteomic data, pattern-based classification, and a better understanding of various glomerular renal diseases have underscored the importance of immunohistology as an integral part of the diagnostic evaluation of renal biopsies. These include immunofluorescence on formalin-fixed paraffin-embedded renal tissue (IF-P), IgG subclass staining, typing of amyloid, and other organized deposits, classification of membranous nephropathy, etc.</p><p><strong>Summary: </strong>We describe the recent immunohistological markers on immunofluorescence (IF) and immunohistochemistry (IHC) on fresh and formalin-fixed paraffin-embedded renal native biopsies for proper evaluation and classification of glomerular diseases. The article also provides information on the diagnostic utility, interpretation, and established antibody clones described in the literature for various glomerular diseases. The indications of IF-P in renal biopsies are also outlined.</p><p><strong>Key messages: </strong>Immunohistology has become integral to diagnosing and classifying various glomerular renal diseases. A specific protein or antigen-based classification has prognostic and therapeutic implications. Additionally, it provides clue for screening the patient for an underlying etiology.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"176-190"},"PeriodicalIF":0.0,"publicationDate":"2025-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12040309/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144058603","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Designing Clinical Trials for the Treatment of Membranous Nephropathy in the Anti-Phospholipase A2 Receptor 1 Era: Results of a NephCure Membranous Nephropathy Workshop. 设计抗磷脂酶A2受体1时代膜性肾病治疗的临床试验:肾治疗膜性肾病研讨会的结果。
Glomerular diseases Pub Date : 2025-03-14 eCollection Date: 2025-01-01 DOI: 10.1159/000544808
Marco Prunotto, Patrick H Nachman, Barbara S Gillespie, Laurence H Beck, Aliza M Thompson, Austin H Hu, Elizabeth A Stafford, Josh M Tarnoff, Brad H Rovin
{"title":"Designing Clinical Trials for the Treatment of Membranous Nephropathy in the Anti-Phospholipase A2 Receptor 1 Era: Results of a NephCure Membranous Nephropathy Workshop.","authors":"Marco Prunotto, Patrick H Nachman, Barbara S Gillespie, Laurence H Beck, Aliza M Thompson, Austin H Hu, Elizabeth A Stafford, Josh M Tarnoff, Brad H Rovin","doi":"10.1159/000544808","DOIUrl":"https://doi.org/10.1159/000544808","url":null,"abstract":"<p><p>Primary membranous nephropathy is a common cause of adult-onset nephrotic syndrome, with an overall incidence of 12 cases per million per year. Primary membranous nephropathy is an autoimmune kidney disease; however, primary membranous nephropathy autoantigens remained elusive until 2009, when the M-type phospholipase A2 receptor 1 (PLA2R) was identified as a disease autoantigen. This was followed relatively rapidly by the identification of several other autoantigens. Autoantibodies against PLA2R are detectable in ≈75% of patients with primary membranous nephropathy. The discovery of circulating and deposited autoantibodies against PLA2R offers an opportunity in nephrology to personalize disease management. On January 14, 2023, NephCure Kidney International convened a scientific workshop in Arlington, Virginia, to discuss the state of the science on autoantibodies against PLA2R and considerations related to the incorporation of autoantibodies against PLA2R in drug development programs for membranous nephropathy. The present report captures the discussion that occurred at the Membranous Nephropathy Scientific Workshop.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"133-141"},"PeriodicalIF":0.0,"publicationDate":"2025-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11908811/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652373","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Case Report and Literature Review of ANCA-Associated Vasculitis and Lupus Nephritis Overlap: Lessons in Management. anca相关性血管炎与狼疮性肾炎重叠1例报告及文献回顾:治疗经验。
Glomerular diseases Pub Date : 2025-03-14 eCollection Date: 2025-01-01 DOI: 10.1159/000543014
Gabriel Dardik, Anna Krieger, Maya K Rao, Michael B Stokes, Benjamin Wooden, Andrew S Bomback
{"title":"A Case Report and Literature Review of ANCA-Associated Vasculitis and Lupus Nephritis Overlap: Lessons in Management.","authors":"Gabriel Dardik, Anna Krieger, Maya K Rao, Michael B Stokes, Benjamin Wooden, Andrew S Bomback","doi":"10.1159/000543014","DOIUrl":"https://doi.org/10.1159/000543014","url":null,"abstract":"<p><strong>Introduction: </strong>ANCA-associated vasculitis (AAV) and lupus nephritis (LN) are distinct disease processes that both cause a nephritic picture with acute kidney injury but have different pathophysiologies and thus different treatment strategies. They also have different characteristic phenotypes on kidney biopsy. Rarely, the two processes can coincide in one patient, known as AAV-LN overlap syndrome.</p><p><strong>Case presentation: </strong>We present the case of a 78-year-old woman who presented with a rapidly progressive kidney injury and nephritic syndrome. Initial workup suggested AAV, but further serologies and ultimately a kidney biopsy demonstrated an overlap of both AAV and LN. Management was changed after discovery of this rare syndrome.</p><p><strong>Conclusion: </strong>A low threshold for ordering serologies and early kidney biopsy are warranted in both AAV and LN as AAV-LN overlap has a unique management strategy that may respond to tailored treatment.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"168-175"},"PeriodicalIF":0.0,"publicationDate":"2025-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11984937/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144013779","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Efficacy of Induction Treatment with a Cyclophosphamide- or Mycophenolate Mofetil-Based Regimen for Active Lupus Nephritis in Thailand: A Retrospective Cohort Study. 泰国以环磷酰胺或霉酚酸酯为基础的方案诱导治疗活动性狼疮肾炎的疗效:一项回顾性队列研究。
Glomerular diseases Pub Date : 2025-02-28 eCollection Date: 2025-01-01 DOI: 10.1159/000545014
Panuvich Poungsuwan, Ouppatham Supasyndh, Bancha Satirapoj
{"title":"The Efficacy of Induction Treatment with a Cyclophosphamide- or Mycophenolate Mofetil-Based Regimen for Active Lupus Nephritis in Thailand: A Retrospective Cohort Study.","authors":"Panuvich Poungsuwan, Ouppatham Supasyndh, Bancha Satirapoj","doi":"10.1159/000545014","DOIUrl":"10.1159/000545014","url":null,"abstract":"<p><strong>Introduction: </strong>Treating lupus nephritis is vital to reducing morbidity and mortality. In Thailand, comparative data on intravenous cyclophosphamide- and mycophenolate mofetil (MMF)-based induction therapies are limited. This study assessed renal remission outcomes for these regimens.</p><p><strong>Methods: </strong>We analyzed renal and patient outcomes in 89 individuals with biopsy-proven active lupus nephritis treated at Phramongkutklao Hospital between 2020 and 2023. Among the cohort, 55 patients received a cyclophosphamide regimen, and 34 were treated with an MMF regimen.</p><p><strong>Results: </strong>Baseline clinical characteristics were comparable between the two groups, except for higher hematuria and renal activity index, as well as lower C3 complement levels and renal chronicity in the cyclophosphamide group. The average doses administered were 0.55 ± 0.12 g/m<sup>2</sup> per dose for intravenous cyclophosphamide and 2.15 ± 0.31 g/day for MMF. By the 24th week, the overall remission rate (complete and partial remission) was 81.8% (45 patients) in the cyclophosphamide group and 85.3% (29 patients) in the MMF group (relative risk 1.01, 95% CI 0.82-1.23, <i>p</i> = 0.949). Proteinuria reduction from baseline at the 24th week was -54.1 ± 93.3% in the cyclophosphamide group and -69.4 ± 22.2% in the MMF group (relative risk 1.01, 95% CI 0.99-1.01, <i>p</i> = 0.465). Adverse events were similar across the two regimens. However, 1 patient in the cyclophosphamide group died, and three required dialysis.</p><p><strong>Conclusion: </strong>Induction therapy with cyclophosphamide- and MMF-based regimens demonstrated comparable efficacy and safety in achieving renal remission in patients with active lupus nephritis.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"151-157"},"PeriodicalIF":0.0,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11961155/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143765919","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Clinical and Pathological Characteristics of Patients with Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits. 伴有单克隆免疫球蛋白沉积的增生性肾小球肾炎的临床和病理特征。
Glomerular diseases Pub Date : 2025-02-20 eCollection Date: 2025-01-01 DOI: 10.1159/000544864
Tariku T Gudura, Hanny Sawaf, Randy Ogbenna, Ali Mehdi, Leal Herlitz, Surafel K Gebreselassie, Shane A Bobart
{"title":"The Clinical and Pathological Characteristics of Patients with Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits.","authors":"Tariku T Gudura, Hanny Sawaf, Randy Ogbenna, Ali Mehdi, Leal Herlitz, Surafel K Gebreselassie, Shane A Bobart","doi":"10.1159/000544864","DOIUrl":"10.1159/000544864","url":null,"abstract":"<p><strong>Introduction: </strong>Proliferative glomerulonephritis with monoclonal immunoglobin (Ig) deposits (PGNMID) is a rare form of kidney disease associated with low monoclonal protein detection rates and poor renal outcomes. The lack of effective therapy is partly due to limited data and understanding of its pathogenesis.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of 18 patients with PGNMID from the Cleveland Clinic Kidney Biopsy Epidemiology Project from January 2015 to March 2023.</p><p><strong>Results: </strong>PGNMID was more predominant among males (67%), and whites (78%), with median age of 60 years. Over 2/3rd of patients presented with hypertension, renal insufficiency, and hematuria, while 26% of patients had nephrotic syndrome. Mean serum creatinine and proteinuria at biopsy were 3.2 mg/dL and 4.3 g/g, respectively. A detectable monoclonal (M) protein was detected in 28% of cases; however, only 3 patients had underlying hematologic disorders (multiple myeloma, CLL, and B-cell lymphoma). An endocapillary hypercellularity/membranoproliferative pattern (72%), IgG/kappa (83%), and IgG3 (56%) were predominant findings on kidney pathology. Eight patients (44%) progressed to end-stage kidney disease (ESKD), with a median onset of 7.5 months after the initial kidney biopsy. While 6 patients achieved complete remission primarily with clone-directed therapy.</p><p><strong>Conclusion: </strong>Despite monoclonal protein deposition on kidney biopsy, 28% patients with PGNMID had a detectable monoclonal protein. Unlike other forms of paraproteinemic kidney diseases, renal outcomes for patients with PGNMID are poor, with 44% progressing to ESKD (median time 7.5 months) after kidney biopsy in our cohort. Clone-directed therapy to improve outcomes remains the mainstay of treatment, despite the absence of detectable clone in most cases. Thus, further investigation into the pathogenesis of PGNMID is warranted to guide future treatment discovery and clinical trials.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"142-150"},"PeriodicalIF":0.0,"publicationDate":"2025-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11936450/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712458","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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