Glomerular diseases最新文献

{"title":"Global Treatment Patterns for Immunoglobulin A Nephropathy in Adults: Results from a Real-World Survey.","authors":"Sydney C W Tang, Dario Roccatello, Carolina Aldworth, Briana Ndife, Suparna Mallik, Aneesh Thomas George, Julia Kattlun, Mark Pletscher, Ceri Hirst, Michel Anton Kroes, Jade Garratt-Wheeldon, Emma Chatterton, Jonathan de Courcy, Richard A Lafayette","doi":"10.1159/000551131","DOIUrl":"10.1159/000551131","url":null,"abstract":"<p><strong>Introduction: </strong>Data on global treatment patterns for immunoglobulin A nephropathy (IgAN) are limited. Herein, we describe real-world treatment in patients with IgAN in clinical practice across distinct global regions.</p><p><strong>Methods: </strong>This was a retrospective analysis of data from a cross-sectional, survey-based study of nephrologists actively managing patients with IgAN (<i>N</i> = 174) and their patients receiving treatment (<i>N</i> = 869) in the USA, China, Japan, France, Germany, Italy, Spain, and the UK in 2021. Nephrologists completed patient record forms and patients completed voluntary patient self-completion forms. Outcomes included patient demographics, clinical characteristics, treatment patterns, and remaining unmet treatment needs. Results were reported using descriptive statistics.</p><p><strong>Results: </strong>At survey completion, mean (±standard deviation [SD]) patient age was 41.6 years (±13.7), and 57.4% were male. At diagnosis, median proteinuria was 1.8 g/day (range: 0.0-22.0), and mean (±SD) estimated glomerular filtration rate was 76.9 mL/min/1.73 m² (±29.1). Median time from diagnosis to first IgAN treatment was 0.0 months (range: -12.8 to 111.4), and mean (±SD) number of treatment lines was 1.6 (±0.9). The most frequently prescribed treatments at survey completion were renin-angiotensin system inhibitors (RASi; 76.3%) and corticosteroids (42.8%). For treatments prescribed first, second, and third, RASi were received by 76.2% (<i>n</i> = 611/802), 79.7% (<i>n</i> = 247/310), and 86.0% (<i>n</i> = 98/114), and corticosteroids were received by 45.8% (<i>n</i> = 367/802), 45.2% (<i>n</i> = 140/310), and 57.9% (<i>n</i> = 66/114), respectively. At survey completion, 17.5% of nephrologists thought that patients' prescribed treatment was not achieving the best possible disease control. High levels of proteinuria (≥1 g/day) were observed regardless of region or treatment line.</p><p><strong>Conclusion: </strong>This survey found that despite high levels of proteinuria among patients with IgAN, nephrologists generally adhered to conservative treatment approaches, in line with international guidelines at survey completion. These findings highlight an opportunity to improve patient outcomes and quality of life through the broader adoption of emerging treatments.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"157-170"},"PeriodicalIF":0.0,"publicationDate":"2026-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13221177/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148139963","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":"Health Care Resource Utilization and Productivity Impairment in Adults with Immunoglobulin A Nephropathy: A Real-World, Observational, Global Analysis.","authors":"Sydney C W Tang, Dario Roccatello, Carolina Aldworth, Briana Ndife, Suparna Mallik, Aneesh Thomas George, Julia Kattlun, Mark Pletscher, Ceri Hirst, Michel Anton Kroes, Jade Garratt-Wheeldon, Emma Chatterton, Jonathan de Courcy, Richard A Lafayette","doi":"10.1159/000550904","DOIUrl":"10.1159/000550904","url":null,"abstract":"<p><strong>Introduction: </strong>There are limited data characterizing the health care resource utilization (HCRU) associated with immunoglobulin A nephropathy (IgAN) and its burden on patients and health care systems. Here, we describe the impact of IgAN on HCRU and productivity using nephrologist- and patient-reported data collected across distinct global geographic regions.</p><p><strong>Methods: </strong>This was a retrospective, cross-sectional survey of nephrologists managing patients with IgAN (<i>N</i> = 295) and their consulting patients with IgAN (<i>N</i> = 1,792) in the USA, China, and Japan, and France, Germany, Italy, Spain, and the UK (EU5) between June and October 2021. Data on patient demographics, clinical characteristics, tests/investigations used to diagnose and manage patients, hospitalizations, dialysis, kidney transplantation, and productivity impairment were collected from nephrologist-completed patient record forms and patient self-completion forms. Results were reported using descriptive statistics.</p><p><strong>Results: </strong>At survey completion, mean (± standard deviation [SD]) patient age was 43.6 (±15.0) years and 58.7% of patients were male. Mean (±SD) estimated glomerular filtration rate was 67.6 (±30.7) mL/min/1.73 m², and 4.2% of patients were receiving regular dialysis. IgAN was diagnosed by kidney biopsy in 85.8% of patients (range: 79.9 [EU5]-98.2% [Japan]). In the 12 months preceding the survey, patients had a mean (±SD) of 7.1 (±7.7) visits with any health care practitioner and 0.4 (±1.1) IgAN-related hospitalizations. In the 7 days prior to the survey, patients missed 11.8% of their work time, 24.3% of their work was impaired while working, 30.0% of their overall work was impaired, and 32.3% of their daily activities were impaired due to IgAN.</p><p><strong>Conclusion: </strong>The diagnosis and management of IgAN are associated with a substantial impact on HCRU and productivity across distinct regions. This highlights the unmet need for novel therapies that target the underlying disease pathophysiology to reduce the ongoing HCRU and humanistic burden associated with IgAN.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"69-83"},"PeriodicalIF":0.0,"publicationDate":"2026-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13082774/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147700889","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":"Crescentic IgM-κ Monoclonal Immunoglobulin Deposition Disease with Thrombotic Microangiopathy in Waldenström's Macroglobulinemia: A Rare and Aggressive Monoclonal Gammopathy of Renal Significance Presentation - A Case Report and Literature Review.","authors":"Chalermchon Suttaluang, Pisut Katavetin, Jerasit Surintrspanont, Talerngsak Kanjanabuch","doi":"10.1159/000550721","DOIUrl":"10.1159/000550721","url":null,"abstract":"<p><strong>Introduction: </strong>Monoclonal immunoglobulin deposition disease (MIDD) is a rare monoclonal gammopathy of renal significance (MGRS) characterized by linear, non-organized deposition of immunoglobulin fragments along glomerular, tubular, and vascular basement membranes. While light and heavy chain MIDDs are more frequently reported, IgM-associated MIDD is exceedingly rare, with only a few cases documented.</p><p><strong>Case presentation: </strong>We report a 71-year-old man who presented with seronegative rapidly progressive glomerulonephritis (RPGN) and sub-nephrotic range proteinuria. Initial serologies were negative for autoimmune and infectious causes. A monoclonal workup revealed an IgM-κ paraprotein, prompting further evaluation. Kidney biopsy demonstrated diffuse crescentic glomerulonephritis (100%), linear IgM-κ deposits, and arterial thrombotic microangiopathy (TMA). Bone marrow studies confirmed lymphoplasmacytic lymphoma with an <i>MYD88</i> c.794T>C (p.Leu265Pro) mutation, consistent with Waldenström's macroglobulinemia (WM).</p><p><strong>Conclusion: </strong>This case represents the first reported instance of IgM-κ MIDD presenting with both crescentic GN and TMA in the setting of WM, defining a uniquely aggressive dual-injury kidney phenotype. It highlights the diagnostic challenge of seronegative RPGN, in which MIDD should be included in the differential diagnosis. The case underscores the importance of integrated histopathology and hematologic genomics in identifying rare MGRS entities, recognizing that severe kidney injury may evolve rapidly despite appropriate management.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"171-177"},"PeriodicalIF":0.0,"publicationDate":"2026-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13241080/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148201261","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":"Beyond Ingestion: Nasal Inhalation-Induced Aristolochic Acid Nephropathy from a Traditional Herbal Remedy - A Case Report.","authors":"Talerngsak Kanjanabuch, Veerapat Wattanasatja, Patnarin Kanjanabuch, Suwasin Udomkarnjananun, Somchai Eiam-Ong","doi":"10.1159/000550695","DOIUrl":"10.1159/000550695","url":null,"abstract":"<p><strong>Introduction: </strong>Aristolochic acid nephropathy (AAN), a progressive form of interstitial nephritis, is predominantly associated with the oral ingestion of herbal remedies containing aristolochic acid (AA).</p><p><strong>Case presentation: </strong>This report details a rare case of AAN linked to nasal inhalation of a traditional herbal preparation. An 80-year-old monk with chronic kidney disease and rapidly declining kidney function underwent kidney biopsy, revealing severe interstitial fibrosis, tubular atrophy, and the presence of aristolochic acid-DNA adducts, confirming the diagnosis. Further investigation uncovered prolonged use of a nasal inhalation remedy for alertness and nasal decongestion. Analysis of the preparation using liquid chromatography-diode array detection-quadrupole time-of-flight mass spectrometry identified aristolochic acid I, directly linking the remedy to the renal pathology.</p><p><strong>Conclusion: </strong>This case highlights a previously underrecognized route of AA exposure and underscores the critical importance of comprehensive history-taking, increased regulation of alternative medicines, and public awareness to prevent similar cases of nephrotoxicity.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"52-55"},"PeriodicalIF":0.0,"publicationDate":"2026-01-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12959914/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147367441","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":"Examining Referral Pathways for Patients with Hematuria: A Real-World Retrospective Analysis.","authors":"Alexander R Chang, Yirui Hu, Lauren Brubaker, Jamie Green, Waleed Zafar, Briana Ndife, Carolina Aldworth","doi":"10.1159/000550577","DOIUrl":"10.1159/000550577","url":null,"abstract":"<p><strong>Introduction: </strong>Follow-up for hematuria, often found incidentally on urinalysis, is critical as it may be an early sign of glomerular disease or malignancy. We evaluated nephrology and urology referral patterns for outpatients with hematuria, and factors associated with nephrology and urology referrals.</p><p><strong>Methods: </strong>This retrospective, observational cohort study leveraged electronic health record data from a large, regional health care system in Pennsylvania, USA. Outpatients with hematuria (≥1+ blood) on dipstick urinalysis from January 1, 2022, to September 30, 2022, without nephrology/urology appointments in the prior 10 years or prior genitourinary cancer were enrolled. The primary objective evaluated the proportion of outpatients with hematuria who had nephrology or urology referrals or appointments within 6 months following initial presentation. Secondary objectives evaluated concurrent proteinuria and follow-up testing patterns.</p><p><strong>Results: </strong>The mean (standard deviation) age of the 5,475 outpatients with hematuria on urinalysis was 60.5 (20.8) years, and most were female (78.0%). Overall, 774/5,475 (14.1%) patients with hematuria had a nephrology or urology referral/appointment within 6 months following presentation. Urine microscopy was performed at the time of hematuria detection for 3,324/5,475 (60.7%) patients, of whom 2,388 (71.8%) had ≥3 red blood cells (RBCs) per high-power field (HPF). Repeat urinalysis was performed for 1,582/5,475 (28.9%) patients. Increased referral likelihood was associated with male sex (odds ratio: 2.3; 95% confidence interval [CI]: 1.9-2.7) and ≥30 RBCs/HPF on microscopy (odds ratio: 1.7; 95% CI: 1.3-2.1). Of 4,952 patients with proteinuria data, 2,375 (48.0%) had hematuria and ≥1+ protein on urinalysis, and 373/2,375 (15.7%) had nephrology (<i>n</i> = 58; 2.4%) and/or urology (<i>n</i> = 332; 14.0%) referrals within 6 months.</p><p><strong>Conclusion: </strong>This study provides insight into current outpatient hematuria management patterns. The low referral rates, particularly with concurrent hematuria and proteinuria, highlight opportunities to improve management moving forward.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"56-68"},"PeriodicalIF":0.0,"publicationDate":"2026-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13012779/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147517089","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 Cutting Edge: A Clinicopathology Minireview of Immunoglobulin A Nephropathy.","authors":"Farid Arman, Glen S Markowitz, Pietro A Canetta","doi":"10.1159/000550295","DOIUrl":"10.1159/000550295","url":null,"abstract":"<p><p>Recent years have seen an explosion of interest in the pathogenesis and therapy of immunoglobulin A nephropathy (IgAN). Large cohort studies have facilitated improvements in disease classification and prognosis, including the establishment and validation of a standard pathological classification, the Oxford classification. Landmark treatment studies published in the past several years have solidified a basic therapeutic approach, emphasizing the value of adding immunomodulatory drugs to supportive care for aggressive disease. There are currently a large number of therapies with diverse mechanisms of action in active development for IgAN, promising at least to enhance our understanding of key pathogenic pathways and, at best, to eventually prove useful in slowing or stopping the progression of IgAN. In this cutting-edge feature, we use a clinical case to review and discuss the current standard of care in the diagnosis and treatment of IgAN, as well as to highlight emerging approaches to treatment.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"26-35"},"PeriodicalIF":0.0,"publicationDate":"2025-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12858203/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146108992","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":"Shifting Sands or ANCA-ed in Place? The Epidemiology of ANCA-Associated Vasculitis over 2 Decades at a Large Tertiary Centre.","authors":"Joshua Storrar, Sawangchit Launiyomchon, Yousaf Amin, Sayyid Raza, Rajkumar Chinnadurai, Edmond O'Riordan, Helen Alderson, Nina Brown, Aine Maire DeBhailis, Smeeta Sinha, Philip A Kalra","doi":"10.1159/000549121","DOIUrl":"10.1159/000549121","url":null,"abstract":"<p><strong>Introduction: </strong>Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic condition usually affecting small blood vessels, commonly causing kidney disease. It has a reported incidence of around 20-25 per million per year. AAV includes both granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Here, we describe the epidemiology of this condition at our renal centre over a 23-year period, compare GPA and MPA and compare the overall cohort by era of diagnosis.</p><p><strong>Methods: </strong>We identified all patients with AAV with renal involvement between January 2000 and December 2022 through our biopsy database and electronic patient record. The cohort after exclusions totaled 278. We collected demographic data, baseline laboratory values, treatment received and outcomes including progression to renal replacement therapy (RRT), relapse rates and mortality. We performed subgroup analysis comparing those with GPA and MPA, and created 4 groups by era of diagnosis.</p><p><strong>Results: </strong>Median age of the cohort was 63.5 years (interquartile range [IQR] 52-72), 52.5% were male and 92.4% White. GPA was seen in 49.3% and MPA in 42.1%. There was advanced renal disease at presentation with median eGFR 18 mL/min/1.73 m² (IQR 9-38) and 54 (19.4%) required RRT at presentation. Relapse occurred in 23%, progression to RRT in 26.6% and 1-year mortality was 3.2%. Median follow-up duration was 54 months (IQR 22-98). Those with GPA were younger, had higher incidence of ENT disease at presentation, were more likely to relapse and had greater 1- and 5-year survival. Mortality rate per hundred person years improved from 2010 onwards.</p><p><strong>Conclusions: </strong>This is one of the largest retrospective observational studies conducted on AAV. We demonstrate that there were more cases of GPA (49.2%) compared to MPA (42.1%) presenting to our centre; comorbidity rates were high; there was advanced renal disease at presentation and mortality rate appeared to improve in the more recent eras.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"10-25"},"PeriodicalIF":0.0,"publicationDate":"2025-12-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12721725/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145822293","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":"Lived Experiences of Patients and Care Providers in Focal Segmental Glomerular Sclerosis and IgA Nephropathy.","authors":"Jai Radhakrishnan, Juan Carlos Julián Mauro, Emily Louise Thompson, Abbie Taylor, Pascaline Agathe Thébault, Kelly Helm, Meaghan Allain Malley, Jennifer Ann Wilson, Kathy Machuzak, Mark Eliot Bensink, Jurgen Floege","doi":"10.1159/000550113","DOIUrl":"10.1159/000550113","url":null,"abstract":"<p><strong>Introduction: </strong>Focal segmental glomerulosclerosis (FSGS) and IgA nephropathy (IgAN) are progressive kidney conditions associated with substantial clinical burden. However, limited research has explored the lived experiences of individuals affected by these conditions. The DEFINE study aimed to address this gap by examining the day-to-day impacts of FSGS and IgAN on personal and professional lives, emotional well-being, time, and finances.</p><p><strong>Methods: </strong>DEFINE was a multi-country qualitative study involving adults with primary FSGS or IgAN, and adult care providers of adults or children with these conditions. Participants were recruited from Germany, Spain, the UK, and the USA. All were invited to complete a pre-task survey and to join virtual focus groups. Thematic analysis was conducted to identify key themes and insights.</p><p><strong>Results: </strong>Thirty-two participants completed the pre-task survey and 26 attended the focus groups. Fatigue was the most burdensome symptom, affecting daily activities, social life, and employment, yet was often overlooked in clinical care. Dietary restrictions and reduced physical activity further limited quality of life, compounded by inconsistent guidance. Emotional impacts, such as sadness and anxiety, were commonly reported, driven by the condition's effect on daily life and fear of disease progression. Many patients reported reduced work hours or ceased employment due to symptoms or condition management. Care providers also made professional adjustments, although some reported greater workplace flexibility. Financial strain was reported across all regions, including medical costs, dietary expenses, medical-related travel, and lost income. Participants highlighted the value of peer support, self-advocacy, and access to specialist healthcare professionals (HCPs).</p><p><strong>Conclusion: </strong>DEFINE highlights the wide-ranging burdens of FSGS and IgAN on patients and care providers. Findings underscore the need for holistic, patient-centered care that recognizes lived experience and provides consistent lifestyle guidance and emotional support. Greater collaboration among HCPs, industry, and patient organizations is essential to improve self-management and quality of life.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"36-51"},"PeriodicalIF":0.0,"publicationDate":"2025-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12885528/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146159454","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":"Evaluation of Biopsy-Based Molecular Risk Prediction in Crescentic Glomerulonephritis.","authors":"Benjamin A Adam, Kristalee Watson, Arashdeep Saini, Peter Dromparis, Ainslie Eberhart, Wirongrong Churngchow, Sridevi Koduru, Maziar Riazy, Sean J Barbour, Michael Mengel","doi":"10.1159/000549541","DOIUrl":"10.1159/000549541","url":null,"abstract":"<p><strong>Introduction: </strong>Novel molecular tools have the potential to improve current clinical and histology-based risk classification systems for various medical renal diseases including glomerulonephritis (GN). We aimed to assess the utility of gene expression for improving biopsy-based risk prediction in patients with GN with and without crescent formation.</p><p><strong>Methods: </strong>This retrospective case-control study used NanoString nCounter to measure the expression of 54 previously described inflammation, nephron injury, endothelium, and crescent-related genes in 335 archival, formalin-fixed paraffin-embedded native kidney biopsies, including a 288-biopsy discovery cohort representing a broad spectrum of crescentic GN subtypes, and an independent 47-biopsy validation cohort focused on ANCA-associated crescentic GN. Clinical, histologic, and gene expression data were compared.</p><p><strong>Results: </strong>Discovery cohort analysis demonstrated increased expression of 13 genes in crescentic GN cases that developed end-stage renal disease (ESRD) versus those that did not (false discovery rate <0.05). Within the 75-biopsy subset of ANCA-associated crescentic GN cases in the discovery cohort, this 13-gene set was found to be independently predictive of ESRD in multivariate Cox proportional hazards regression analysis (<i>p</i> = 0.015), with significant differentiation of high and low risk patients in the Kaplan-Meier renal survival analysis (log-rank test, <i>p</i> = 0.002). However, validation cohort analysis did not demonstrate significant improvement in risk stratification with the 13-gene set when compared with established clinicopathologic models.</p><p><strong>Conclusion: </strong>These results suggest that biopsy-based gene expression may provide the opportunity for improved risk stratification in crescentic GN; however, the genes evaluated in this study appear to have limited added clinical utility over existing risk scores.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"454-469"},"PeriodicalIF":0.0,"publicationDate":"2025-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12695120/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145745861","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":"NELL1-Associated Membranous Nephropathy in a Patient with Papillary Thyroid Carcinoma: A Case Report and Literature Review.","authors":"Basheer Ahamad Kummangal, Oliver Padgett, Lisa Kumasaka, Nicole Andeen, Rupali S Avasare","doi":"10.1159/000549351","DOIUrl":"10.1159/000549351","url":null,"abstract":"<p><strong>Introduction: </strong>Membranous nephropathy (MN) is a leading cause of nephrotic syndrome in adults. NELL1 accounts for approximately 10% of MN cases and is emerging as a distinct subtype often linked to secondary conditions, such as malignancies and drugs. Here, we describe a case of MN in a patient with metastatic papillary thyroid carcinoma.</p><p><strong>Case presentation: </strong>A 78-year-old man with metastatic papillary thyroid carcinoma and a distant history of treated syphilis presented with nephrotic syndrome. A renal biopsy revealed segmental MN with positive NELL1 staining, while the thyroid carcinoma tissue also demonstrated NELL1 staining at weak to moderate intensity. The patient was managed conservatively without immunosuppression due to the lack of oncologic treatment options and syphilis was treated with doxycycline due to positive serologies, though active infection was not suspected. Follow-up showed stabilization of renal function and marked reduction in proteinuria.</p><p><strong>Conclusion: </strong>NELL1-positive MN is increasingly recognized for its association with malignancy. The patient achieved partial remission with supportive, non-immunosuppressive therapy. Clinicians should maintain a high index of suspicion for secondary conditions when NELL1-positive MN is encountered. This case report reviews secondary NELL1 associations and underscores the diagnostic challenges in MN when multiple potential secondary causes exist.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"448-453"},"PeriodicalIF":0.0,"publicationDate":"2025-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12685352/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145716622","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}