Glomerular diseases最新文献

{"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 m²/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 m² (3.90 ± 11.42 vs. -9.31 ± 5.08 mL/min/1.73 m²/year, <i>p</i> = 0.011), mesangial hypercellularity M0 score (4.69 ± 11.37 vs. -2.63 ± 6.42 mL/min/1.73 m²/year, <i>p</i> = 0.049), and C0 score (2.48 ± 12.63 vs. -5.58 ± 8.4 mL/min/1.73 m²/year, <i>p</i> = 0.026). Additionally, rapid GFR decline (>5 mL/min/1.73 m²/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 m² 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}

{"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}

{"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}

{"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}

{"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/m² 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}

{"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}

{"title":"Minimal Change Podocytopathy with Coexistent Thin Glomerular Basement Membrane following Exposure to Semaglutide.","authors":"Karthik Kovvuru, Swetha Rani Kanduri, Johnathon Phillips, Juan Carlos Velez","doi":"10.1159/000543357","DOIUrl":"10.1159/000543357","url":null,"abstract":"<p><strong>Introduction: </strong>Glucagon-like peptide-1 receptor agonists (GLP-1RAs) are FDA-approved for weight loss and are increasingly prescribed for patients with obesity and type 2 diabetes mellitus. Multiple studies have demonstrated desirable renal and cardiovascular benefits from these novel agents. So far, a few case reports of acute tubular injury and acute interstitial nephritis have been reported with GLP-1RA. Podocytopathies in association with semaglutide are rare. In this case report, we present a case of nephrotic syndrome that developed after exposure to semaglutide and propose potential pathophysiological mechanisms underlying this rare renal complication.</p><p><strong>Case presentation: </strong>Herein, we report a novel case of a 43-year-old female who was evaluated in the nephrology clinic for abrupt onset of bilateral lower extremity edema and foamy urine, a few weeks after exposure to semaglutide. She was diagnosed with nephrotic syndrome and subsequently underwent a kidney biopsy, which revealed features suggestive of minimal change disease, along with coexistent thin basement membrane disease.</p><p><strong>Conclusion: </strong>GLP-1RAs have been increasingly prescribed due to their proven pleiotropic benefits, including improvements in albuminuria, glycemic control, weight loss, and cardioprotective effects. Despite the considerable benefits of GLP-1RAs, it is essential to recognize novel side effects.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"103-108"},"PeriodicalIF":0.0,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11875556/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143544779","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}

{"title":"Sodium-Glucose Cotransporter 2 Inhibitors in Diabetic Kidney Disease and beyond.","authors":"Zein Alabdin Hannouneh, C Elena Cervantes, Mohamad Hanouneh, Mohamed G Atta","doi":"10.1159/000543685","DOIUrl":"10.1159/000543685","url":null,"abstract":"<p><strong>Background: </strong>Sodium-glucose cotransporter 2 inhibitors (SGLT2is) have significantly impacted the management of diabetic kidney disease (DKD) and heart failure (HF), providing benefits beyond glycemic control. This review examines the mechanisms through which SGLT2is provide renal and cardiovascular protection and assesses their clinical efficacy.</p><p><strong>Summary: </strong>By inducing glucosuria and natriuresis, SGLT2is alleviate multiple complications induced by chronic hyperglycemia. Moreover, SGLT2is reduce albuminuria, improve tubular function, and modulate erythropoiesis. Additionally, they mitigate inflammation and fibrosis by decreasing oxidative stress and downregulating proinflammatory pathways. Clinical trials have demonstrated significant reductions in renal and cardiovascular events among patients with type 2 diabetes mellitus. A comprehensive review of the literature was conducted through PubMed, highlighting the effects of SGLT2is and the results of major clinical trials involving SGLT2is.</p><p><strong>Key messages: </strong>SGLT2is play a crucial role in the management of DKD and HF by addressing multiple pathogenic pathways. Currently, SGLT2is are included in clinical guidelines for DKD and HF management, and their benefits extend to nondiabetic populations. Further research is needed to explore SGLT2is' multifaceted mechanisms and potential applications across diverse patient populations and different disease etiologies.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"119-132"},"PeriodicalIF":0.0,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11906174/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143627027","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}

{"title":"Genetic and Iatrogenic Defects in Peripheral Tolerance Associated with Anti-Nephrin Antibody-Associated Minimal Change Disease.","authors":"J Ashley Jefferson, Karin Chen, Sangeeta Hingorani, A Bilal Malik, Scott S Tykodi, Keith H Keller, Yuan Huang, Kelly D Smith, Robyn C Reed, Astrid Weins, Shreeram Akilesh","doi":"10.1159/000543334","DOIUrl":"10.1159/000543334","url":null,"abstract":"<p><strong>Introduction: </strong>Minimal change disease (MCD) is a common cause of nephrotic syndrome in children and adults. Immune dysregulation is a contributor, but the relative roles of individual components of the immune system in MCD pathogenesis remain unclear.</p><p><strong>Case presentation: </strong>Here, we present 2 patients with defects in immune tolerance mechanisms that developed MCD associated with anti-nephrin antibodies. The first patient had a pathogenic deletion in <i>FOXP3</i>, leading to reduced regulatory T cells. Serum could not be obtained from this patient during the active phase of MCD to directly establish the presence of anti-nephrin antibodies. However, this patient demonstrated IgG dusting over podocyte cell bodies by immunofluorescence microscopy, as well as colocalization of IgG with nephrin in confocal microscopy. The second patient developed MCD in the context of immune checkpoint inhibitor treatment for metastatic carcinoma. Anti-nephrin antibodies were detected in this patient during active disease. The patient's kidney biopsy also showed evidence of binding of anti-nephrin antibodies within the glomeruli.</p><p><strong>Conclusion: </strong>These cases demonstrate that genetic and iatrogenic mechanisms of breakdown in peripheral tolerance can lead to MCD.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"74-83"},"PeriodicalIF":0.0,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11845169/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484308","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}

{"title":"5th International Renal Pathology Conference in Zagreb, Croatia: Meeting Report.","authors":"Petar Šenjug, Danica Galešić Ljubanović","doi":"10.1159/000542726","DOIUrl":"10.1159/000542726","url":null,"abstract":"","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"19-25"},"PeriodicalIF":0.0,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11842037/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484965","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}