Glomerular diseases最新文献

{"title":"Cardiometabolic Risk, Evaluation, and Control in Lupus Nephritis.","authors":"Dawn Se Teng Lim, Julia Andres, Hui Zhuan Tan, Tung Lin Lee, Felice Fangie Leong, Irene Yanjia Mok, Jason C J Choo, Cynthia Ciwei Lim","doi":"10.1159/000553591","DOIUrl":"10.1159/000553591","url":null,"abstract":"<p><strong>Introduction: </strong>Lupus nephritis (LN) is associated with atherosclerotic cardiovascular disease but early recognition of cardiometabolic risk in patients with LN can facilitate risk optimization with disease-modifying strategies. To identify potential gaps in cardiometabolic risk management in LN in real-world practice, we aimed to evaluate the prevalence and trend in cardiometabolic risk, evaluation, and control.</p><p><strong>Methods: </strong>We performed a single-center, cross-sectional study of all adults with biopsy-confirmed LN between 2011 and 2022 to (1) assess the prevalence of cardiometabolic risks (age, sex, diabetes mellitus, hypertension, hyperlipidemia, ischemic heart disease [IHD], reduced kidney function [estimated glomerular filtration rate, estimated glomerular filtration rate <60 mL/min/1.73 m²], urine protein-to-creatinine ratio [UPCR] >0.15 g/g, glucocorticoid use), evaluation (office blood pressure [BP], fasting glucose and lipid and HbA1c), and control at the time of diagnosis; and then (2) evaluate the association between time periods (2019-2022 compared to 2015-2018) and cardiometabolic evaluation and control, adjusting for traditional and systemic lupus erythematosus disease-specific cardiometabolic risks in multivariable logistic regression models.</p><p><strong>Results: </strong>We evaluated 326 adults with LN (median age 42.0 years [interquartile range: 30.9, 52.7]). Across the 3 time periods, reduced kidney function was most frequent in 2011-2014. Hypertension and renin-angiotensin system blocker use were lowest in 2019-2022 (23.4% and 30.9%, respectively), while 38.0% achieved BP <130/80 mm Hg without significant change across time periods. Glycemic evaluation was least frequent in 2019-2022 (78.7%) compared to earlier time periods. Achievement of glycemic targets of fasting glucose <6.1 mmol/L and HbA1c ≤7% were 70.0% and 95.1%, respectively, without significant change across the time periods. Lipid evaluation was performed in 75.8% but only 32.0% achieved low-density lipoprotein-cholesterol <2.6 mmol/L. There was no significant change in the prevalence of lipid evaluation or control over time.</p><p><strong>Conclusion: </strong>Glycemic assessment and optimization of BP and lipid treatment to achieve guideline-recommended targets represent opportunities to improve cardiometabolic risk management in LN.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"291-302"},"PeriodicalIF":0.0,"publicationDate":"2026-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13577658/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148978888","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":"Hypokalemic Distal Tubulopathy Secondary to Anti-Phospholipase A2 Receptor-Positive Membranous Nephropathy: An Exceptional Case.","authors":"Thanawat Vongchaiudomchoke, Boonyarit Cheunsuchon, Nuttaya Wachiraphansakul","doi":"10.1159/000553335","DOIUrl":"10.1159/000553335","url":null,"abstract":"<p><strong>Introduction: </strong>Primary membranous nephropathy, one of the most common primary nephrotic syndromes in adults, rarely has tubular involvement. Two case reports have previously illustrated atypical tubular manifestations, including Liddle-like syndrome and proximal tubulopathy. Hypokalemic distal tubulopathy in anti-phospholipase A2 receptor antibody-positive membranous nephropathy has not yet been recognized.</p><p><strong>Case presentation: </strong>We report a case of a 37-year-old Thai male presenting with nephrotic syndrome and rhabdomyolysis secondary to severe hypokalemia. Kidney biopsy and serologic testing confirmed anti-phospholipase A2 receptor antibody-positive membranous nephropathy. Urinary indices showed a distal tubulopathy pattern. Hormonal studies, including plasma renin, plasma aldosterone, and a dexamethasone suppression test, were all negative. The patient responded well to prednisolone and oral cyclophosphamide. With nephrotic syndrome remission, hypokalemia was alleviated simultaneously.</p><p><strong>Conclusion: </strong>This case suggests a recognition and expansion of the knowledge of an extremely rare tubular involvement in relation to anti-phospholipase A2 receptor antibody-positive membranous nephropathy.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"285-290"},"PeriodicalIF":0.0,"publicationDate":"2026-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13461131/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148714804","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":"How Late Is Too Late? Successful Use of Targeted-Release Budesonide in IgA Nephropathy with Estimated Glomerular Filtration Rate <30 mL/min/1.73 m².","authors":"Abhinav Sharma, Adnan Fatfat, Ishaan Batish, Md Shahrier Amin, LaTonya J Hickson, Nabeel Aslam, Shane A Bobart","doi":"10.1159/000553107","DOIUrl":"10.1159/000553107","url":null,"abstract":"<p><strong>Introduction: </strong>IgA nephropathy (IgAN) is a common cause of chronic kidney disease (CKD) worldwide. However, patients with advanced CKD and significant fibrosis on biopsy have limited treatment options, and clinical trials studying targeted-release budesonide have excluded individuals with low estimated glomerular filtration rate (eGFR).</p><p><strong>Case presentation: </strong>We report a case of a 36-year-old male with biopsy-proven crescentic IgAN, initially diagnosed 3 years prior, who presented with progressive renal dysfunction and persistent proteinuria despite prior treatment with immunosuppression, including corticosteroids and rituximab. At evaluation, serum creatinine was 2.86 mg/dL with an eGFR of 28 mL/min/1.73 m², urine protein-to-creatinine ratio was 1.64 grams/day, and microscopic hematuria was present. Repeat kidney biopsy demonstrated significant chronic changes with severe interstitial fibrosis and tubular atrophy. The Oxford classification score was M1, E1, S1, T2, C1. Targeted-release budesonide was initiated, after which hematuria resolved within 1 month, proteinuria decreased significantly reaching 0.29 g/day, and kidney function improved with a serum creatinine of 2.24 mg/dL and eGFR of 38 mL/min/1.73 m² at 8 months into treatment. The patient tolerated therapy without major adverse events.</p><p><strong>Conclusion: </strong>This case demonstrates clinically meaningful improvement in both proteinuria and renal function following treatment with targeted-release budesonide, despite advanced chronicity but with disease activity and low eGFR, a population that remains underrepresented in clinical trials and prospective studies. Our findings highlight the importance of repeat biopsy and future studies addressing the potential benefit of targeted-release budesonide in select patients with advanced IgAN, who may retain a therapeutically targetable inflammatory component.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"279-284"},"PeriodicalIF":0.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13441277/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148681245","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":"Acute Care Visits and Subsequent Health-Related Quality of Life among Children and Adults with Glomerular Disease in the Cure Glomerulonephropathy Cohort.","authors":"Chien-Wen Yang, Shalini S Ramachandra, Qian Liu, Dorey A Glenn, Michelle Denburg, Todd Miano, Jarcy Zee","doi":"10.1159/000552975","DOIUrl":"10.1159/000552975","url":null,"abstract":"<p><strong>Introduction: </strong>Health-related quality of life (HRQOL) is an important patient-reported aspect of overall health and is understudied in patients with glomerular disease (GD). Conflicting data have emerged regarding whether acute care visits (ACVs) are associated with HRQOL. This report describes types of ACVs and their short-term impact on HRQOL in patients with minimal change disease (MCD), IgA nephropathy/vasculitis (IgAN/IgAV), focal segmental glomerulosclerosis (FSGS), and membranous nephropathy (MN).</p><p><strong>Methods: </strong>The Cure Glomerulonephropathy (CureGN) network is an observational prospective cohort study that has enrolled children and adults diagnosed with MCD, FSGS, MN, or IgAN/IgAV from 71 clinical centers across the USA, Canada, and Europe since December 2014. We used linear mixed-effects models to examine the association between frequency of ACVs over a 3-month period and subsequent Patient-Reported Outcomes Measurement Information System (PROMIS) scores, accounting for previous PROMIS scores, clinical characteristics, medication use, comorbid conditions, and socioeconomic indicators.</p><p><strong>Results: </strong>The study included 2,393 patients, 1,282 of whom experienced at least 1 ACV with a total of 3,555 ACVs (33.3 per 100 person-years) observed during CureGN study follow-up. The most frequent ACV-related diagnoses were categorized as infectious, gastrointestinal, hypertensive, or cardiovascular in origin. Two additional ACVs were associated with clinically and statistically significantly worse general physical health among adults; self-reported fatigue, mobility, and global health in children; and parent-reported fatigue, mobility, and global health in children. Only one additional ACV was associated with clinically and significantly worse fatigue among patients with cyclophosphamide exposure and parent-reported mobility among children ages 8-<11.</p><p><strong>Conclusions: </strong>Increasing ACV burden was associated with lower HRQOL across multiple physical health domains for patients with GD. Our study reinforces the health care burden experienced by patients with GD and identifies risk factors for deterioration in HRQOL.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"260-267"},"PeriodicalIF":0.0,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13423334/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148632985","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":"Prognostic Value of Baseline and Early Proteinuria Changes in IgA Nephropathy: A US Cohort Study.","authors":"Scott G Silvey, Ambarish Athavale, Naman Gupta, Jason Kidd, Nilang Patel","doi":"10.1159/000552941","DOIUrl":"10.1159/000552941","url":null,"abstract":"<p><strong>Introduction: </strong>Proteinuria is a key marker in IgA nephropathy (IgAN). However, long-term thresholds and significance of early changes in proteinuria remains unclear in US populations.</p><p><strong>Methods: </strong>Retrospective cohort study of 745 adult patients with IgA nephropathy from the TriNetX US network (2015-2023). The baseline Time-average proteinuria (TAP) were calculated and categorized as <500, 500-1,000, 1,000-2,000, and >2,000 mg/g. Starting 1 year post diagnosis, the composite kidney failure outcome (sustained eGFR ≤15 mL/min/1.73 m², sustained >40% eGFR decline, initiation of dialysis, or all-cause mortality) was evaluated. Associations with baseline TAP and changes in TAP at first-year were evaluated using Kaplan-Meier analysis and multivariable Cox proportional hazard models.</p><p><strong>Results: </strong>Out of 745 patients (mean age 43.5 [±16] years, 58% male, 68% White), a composite outcome occurred in 175 (23.5%) of patients over a median follow-up of 5.4 years (IQR: 3.2-7.2). Survival analysis showed 13.8% of patients with TAP <500 mg/g remained at risk of kidney failure by 8 years. Compared to TAP <500 mg/g, adjusted hazard ratios were significantly higher for TAP 1,000-2,000 mg/g (aHR 2.84; 95% CI: 1.66-4.85) and TAP >2,000 mg/g (aHR 5.68; 95% CI: 3.45-9.36); whereas TAP 500-1,000 mg/g was not significant after adjustment (aHR 1.69; 95% CI: 0.96-2.97). Higher baseline eGFR and white race were associated with better outcomes. Among 389 patients with follow up TAP data at the first-year observation period, each one-category reduction in TAP was associated with ∼40% lower risk of composite outcome, and ≥30% and ≥50% proteinuria reductions were associated with 62% and 66% lower risk of composite outcome, respectively.</p><p><strong>Conclusions: </strong>In US adults with IgA nephropathy, baseline proteinuria exceeding 1,000 mg/g identifies a high-risk group. Early proteinuria reductions - either by category (TAP) or percentage (≥30-50%) - strongly predict improved outcomes. This study supports use of proteinuria as a modifiable, treat-to-target surrogate for prognosis and treatment monitoring.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"249-259"},"PeriodicalIF":0.0,"publicationDate":"2026-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13427026/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148655197","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":"Renal Histopathology and Outcomes of Anti-Neutrophil Cytoplasmic Antibody-Associated Glomerulonephritis: The Qatar Experience.","authors":"Faten Aqeel, Omar Fituri, Awais Nauman, Mohamad M Alkadi, Fatima Babiker Eltayeb, Eiman Mohamed Hamid, Essa Abdulla Abuhelaiqa, Khaled Murshed, Rajvir Singh, Muhammad Asim, Hassan Ali AlMalki","doi":"10.1159/000552845","DOIUrl":"10.1159/000552845","url":null,"abstract":"<p><strong>Introduction: </strong>Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe small-vessel vasculitis with substantial morbidity. Data from the Gulf region are limited, restricting understanding of disease patterns and outcomes. This study describes the epidemiology, clinical features, kidney pathology, and management of ANCA-associated glomerulonephritis (ANCA-GN) in Qatar.</p><p><strong>Methods: </strong>We performed a retrospective cohort study of adults diagnosed with ANCA-GN between 2015 and 2025 at Hamad Medical Corporation. Demographic, clinical, serologic, histopathologic, and therapeutic data were extracted from electronic records. Primary outcomes were end-stage kidney disease (ESKD) and death; secondary outcomes included kidney function trends, proteinuria, ANCA titers, relapse, and infections. Statistical analyses included Student's t test, chi-square, Fisher's exact test, and Kaplan-Meier survival.</p><p><strong>Results: </strong>Twenty-eight patients were followed for a median of 16 months (IQR 16-43). Median age was 49 years, 71% were male, and 54% were of South Asian origin. Seasonal peaks occurred in winter (43%) and summer (32%). GPA and MPA accounted for 54% and 43% of cases; 57% had pulmonary involvement. PR3-ANCA was positive in 43% and MPO-ANCA in 36%, and 18% had atypical ANCA. Mixed (50%) and crescentic (25%) histologic classes predominated. Despite 61% having low-to-moderate ANCA Kidney Risk Score (AKRiS), 39% developed ESKD and 14% died. Maintenance therapy was given to only 54%. Infection-related mortality accounted for most deaths. Lack of hematuria resolution, failure of early proteinuria reduction, and absence of maintenance therapy were significantly associated with ESKD (<i>p</i> < 0.05).</p><p><strong>Conclusion: </strong>ANCA-GN in Qatar demonstrates seasonal clustering and high ESKD rates, pointing to opportunities for improved monitoring, timely therapy, and more consistent care pathways.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"236-248"},"PeriodicalIF":0.0,"publicationDate":"2026-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399959/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148586312","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":"Transitions in Glomerular Disease.","authors":"Jessamyn S Carter, Marianne Kerski, Jessica Greco, Jacqueline A Madison","doi":"10.1159/000552819","DOIUrl":"10.1159/000552819","url":null,"abstract":"<p><strong>Background: </strong>The transition from pediatric to adult care is a complex and challenging issue for increasing numbers of adolescents and young adults (AYA). Despite increasing attention to the transition of AYA with chronic medical conditions, there remain difficulties which vary by local resources, disease processes, and individual factors. There are also poorly understood factors regarding transition involving rare diseases, including glomerular disease. Specialties and organizations are working to evaluate and enhance the transition process; however, the variability in resources limits formation of a true consensus.</p><p><strong>Summary: </strong>AYA with glomerular disease, broadly as chronic kidney disease and specifically with respect to diagnoses such as lupus nephritis, are vulnerable during the health care transition (HCT). Though young, many of these patients have severe disease with the loss of kidney function over their disease course and risk significant complications around the time of transition. Many facets make HCT challenging related to personal, caregiver, provider, and system factors. Despite difficulties, some groups have found success, largely in dedicated clinics designed to promote successful transition from pediatric to adult models of care. Society guidelines focus on a structured, intentional process providing continuity, with or without a dedicated transition clinic. Preparation, communication across the transition, and support for patients and providers with additional staff are all recommended aspects of transition from the International Society of Nephrology and International Pediatric Nephrology Association. Additionally, transition toolkits are available from several sources which can aid in developing a structured transition program for patients with glomerular disease.</p><p><strong>Key messages: </strong>(1) There are many challenges in transitioning AYA patients with glomerular disease from pediatric to adult care. (2) HCT is a vulnerable period with increased risk of poor outcomes. (3) A successful HCT may be promoted by developing a structured process and/or program focused on preparation, communication, and support.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"268-278"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13427328/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148654874","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":"Glomerular Structure and Function in Type 2 Diabetes Stratified by Glomerular Basement Membrane Thickening.","authors":"Amy K Mottl, Elif I Ekinci, John M Basgen, J Charles Jennette, Yichun Hu, Susan L Hogan, David Power, Alison Skene, Susanne B Nicholas, Michael Mauer","doi":"10.1159/000552638","DOIUrl":"10.1159/000552638","url":null,"abstract":"<p><strong>Introduction: </strong>Glomerular structural-functional relationships in diabetes were tested, stratifying by glomerular basement membrane (GBM) thickness, an approach not previously explored.</p><p><strong>Methods: </strong>Research kidney biopsies were performed in 30 people with type 2 diabetes (T2D) and any of the following baseline characteristics: moderate/severe albuminuria (urine albumin:creatinine ratio [UACR]: >30 µg/mg), low estimated glomerular filtration rate (eGFR) (45-60 mL/min/1.73 m², with and without albuminuria), or severe/proliferative retinopathy. Stereologic analyses were performed, and participants were stratified by the presence of GBM thickening. Standard light microscopy (LM) for glomerular, vascular, and tubulointerstitial lesions was performed in a subset (<i>N</i> = 19) with available paraffin-embedded tissue. Creatinine values were collected, and the eGFR slope was calculated.</p><p><strong>Results: </strong>Thickening of the GBM was present in 21 of 30 participants. Clinically, the GBM-thickened group differed numerically from the normal GBM group only in having a higher HbA1c (7.9% vs. 6.6%) and greater prevalence of albuminuria (67% vs. 22%). Correlations with eGFR slope were not present in the full cohort, but were present in the group with GBM thickening: fractional volume of mesangium per glomerulus (<i>R</i> = -0.4; <i>p</i> = 0.03), fractional volume of mesangial matrix per glomerulus (<i>R</i> = -0.5; <i>p</i> = 0.04), and podocyte foot process width (<i>R</i> = -0.5; <i>p</i> = 0.03). Results were unchanged after excluding two participants with normal UACR and eGFR. Of the six members of the subgroup without GBM thickening who had available LM, two had hypertensive nephrosclerosis, three had significant vascular lesions but no interstitial fibrosis, and one had mild vascular and interstitial lesions.</p><p><strong>Conclusion: </strong>This study highlights the need for further work to better categorize renal structural phenotypes in patients with T2D to facilitate kidney disease prognostication, novel biomolecular target discovery and therapies, and renal structural endpoints for clinical trials.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"228-235"},"PeriodicalIF":0.0,"publicationDate":"2026-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13363132/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148439216","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":"Interpretative Challenges of Variants of Uncertain Significance in Severe Treatment-Refractory Focal Segmental Glomerulosclerosis.","authors":"Theerachai Thammathiwat, Noppakao Kongtal, Laor Chompuk, Suphakit Khutanthong, Banpot Suwatronnakorn, Talerngsak Kanjanabuch","doi":"10.1159/000552476","DOIUrl":"10.1159/000552476","url":null,"abstract":"<p><strong>Introduction: </strong>Steroid-resistant nephrotic syndrome (SRNS) frequently reflects an underlying monogenic podocytopathy; however, the increasing use of genomic testing has also led to frequent identification of variants of uncertain significance (VUS), complicating clinical interpretation and creating a risk of overattribution of causality.</p><p><strong>Case presentations: </strong>We report a 22-year-old woman with childhood-onset nephrotic syndrome evolving along the focal segmental glomerulosclerosis spectrum and refractory to multiple immunosuppressive agents, including corticosteroids, calcineurin inhibitors, mycophenolate mofetil, and rituximab. Despite prolonged therapy, she had persistent nephrotic-range proteinuria and severe hypoalbuminemia. Her clinical course was complicated by recurrent infections, necrotizing pancreatitis attributed to calcineurin inhibitor exposure, and thromboembolic events - including deep vein thrombosis and ischemic stroke - in the setting of nephrotic hypercoagulability. She subsequently developed a spontaneous hydropneumothorax during a period of severe disease activity. Kidney function remained preserved throughout follow-up. Comprehensive genomic testing identified heterozygous VUS in <i>ARHGAP24</i> and <i>NUP133</i>.</p><p><strong>Conclusion: </strong>This case illustrates the interpretative challenges posed by VUS in complex glomerular disease and underscores the need for disciplined variant classification, careful gene-disease validation, and rigorous clinical-genetic correlation to avoid misclassification and inappropriate management decisions in SRNS.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"222-227"},"PeriodicalIF":0.0,"publicationDate":"2026-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13345616/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148413980","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":"An \"All-in-One\" Kidney Biopsy in AIDS: Crescentic Immune Complex-Mediated Glomerulonephritis, CD8-Driven Interstitial Nephritis, and Superimposed Thrombotic Microangiopathy.","authors":"Thanawat Sevaphai, Jerasit Surintrspanont, Theerachai Thammathiwat, Thana Thongsricome, Talerngsak Kanjanabuch","doi":"10.1159/000552365","DOIUrl":"10.1159/000552365","url":null,"abstract":"<p><strong>Introduction: </strong>Human immunodeficiency virus (HIV)-associated kidney disease encompasses a diverse spectrum of glomerular, tubulointerstitial, and vascular pathologies. While HIV-associated nephropathy and immune complex-mediated glomerular disease in the setting of HIV are well-recognized entities, their simultaneous presentation with thrombotic microangiopathy (TMA) and diffuse infiltrative lymphocytosis syndrome (DILS) has not been previously documented. We report a novel case of advanced HIV infection manifesting as crescentic lupus-like glomerulonephritis (GN), CD8-predominant tubulointerstitial nephritis, and acute TMA.</p><p><strong>Case presentation: </strong>A 43-year-old man presented with rapidly progressive kidney failure, nephrotic-range proteinuria, hematuria, and hypertensive emergency. Laboratory workup revealed newly diagnosed HIV infection confirmed by fourth-generation antibody assay with a CD4 count of 53 cells/µL. Kidney biopsy demonstrated a \"full-house\" immune complex-mediated GN with cellular and fibrous crescents involving 5 of 8 viable glomeruli, chronic active CD8+ lymphocytic interstitial nephritis, and arteriolar fibrin thrombus with endothelial injury. Electron microscopy revealed mesangial and subendothelial electron-dense deposits and tubuloreticular inclusions. No evidence of systemic lupus erythematosus, paraproteinemia, or other active infections was found. A diagnosis of crescentic immune complex-mediated GN in the setting of HIV with DILS-like interstitial nephritis and superimposed acute TMA was established. Despite antiretroviral therapy, corticosteroids, cyclophosphamide, and plasma exchange, kidney function recovery was incomplete and the patient remained dialysis-dependent.</p><p><strong>Conclusion: </strong>This case represents a rare \"all-in-one\" manifestation involving glomerular, interstitial, and vascular injury. These findings underscore profound immune dysregulation in acquired immunodeficiency syndrome (AIDS), where immune deficiency coexists with compartmentalized immune activation. Recognition of overlapping pathology is essential for accurate diagnosis and individualized, risk-adapted management.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"6 1","pages":"216-221"},"PeriodicalIF":0.0,"publicationDate":"2026-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13331470/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148392635","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}