Glomerular diseasesPub Date : 2025-11-12eCollection Date: 2025-01-01DOI: 10.1159/000549351
Basheer Ahamad Kummangal, Oliver Padgett, Lisa Kumasaka, Nicole Andeen, Rupali S Avasare
{"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}
Glomerular diseasesPub Date : 2025-11-07eCollection Date: 2025-01-01DOI: 10.1159/000549367
Basil Alnasrallah, Manaf Aljishi, Shatha Alfaraj, Abduallah Alqawain, Zainab Alkhuraidah, Eman Alabbad, Mohammad M Aljishi, Ahmed Ali Alnasser, Jafar Alrebh, Amein K Al-Ali, Husam Alzayer
{"title":"Histopathological Features of Sickle Cell Nephropathy in the Arab-Indian Haplotype.","authors":"Basil Alnasrallah, Manaf Aljishi, Shatha Alfaraj, Abduallah Alqawain, Zainab Alkhuraidah, Eman Alabbad, Mohammad M Aljishi, Ahmed Ali Alnasser, Jafar Alrebh, Amein K Al-Ali, Husam Alzayer","doi":"10.1159/000549367","DOIUrl":"10.1159/000549367","url":null,"abstract":"<p><strong>Introduction: </strong>Sickle cell disease (SCD), an autosomal recessive disorder caused by hemoglobin S, leads to red blood cell sickling and multiorgan damage, including sickle cell nephropathy (SCN). While SCN histopathological changes are well described, data from the Middle East region, where the Arab-Indian (AI) haplotype is common, are limited. We hypothesized that SCN in patients in Saudi Arabia shows histopathological features similar to those in other regions, reflecting a shared renal injury mechanism.</p><p><strong>Methods: </strong>This single-center retrospective study analyzed kidney biopsies from adults with SCD in the Eastern Province of Saudi Arabia from 2012 to 2023. Histological specimens were examined using hematoxylin and eosin, periodic acid-Schiff stain, Masson trichrome stain, Jones silver stain, and immunofluorescence. Haplotype genotyping was performed using a nuclease allelic discrimination assay with target-specific primers and TaqMan probes labeled with VIC and FAM. Clinical and biochemical data were collected at the time of biopsy and at the last follow-up.</p><p><strong>Results: </strong>Twelve biopsies were included in the study (median age 44 years, 50% female). Median serum creatinine was 91.5 µmol/L (interquartile range [IQR] 59-130), and 24-h urinary protein was 3,300 mg (IQR 1,181-5,725). Of 9 patients tested, seven had homozygous AI haplotype, one had heterozygous AI, and one had BEN/CAR/CAM. Histopathology showed tubular hemosiderosis (92%), global sclerosis (75%), glomerular hypertrophy (75%), sickled red blood cells (58%), focal segmental glomerulosclerosis (42%), and glomerular basement membrane duplication (33%). These findings mirror SCN patterns in other haplotypes.</p><p><strong>Conclusions: </strong>In Saudi patients with SCD with predominant AI haplotype, SCN histopathological features align with those reported globally, suggesting a consistent renal pathogenesis across haplotypes. This study improves our understanding of SCN in the AI haplotype, supporting standardized treatment methods and further research into preventing progression.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"438-447"},"PeriodicalIF":0.0,"publicationDate":"2025-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12680401/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145703020","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}
Glomerular diseasesPub Date : 2025-10-23eCollection Date: 2025-01-01DOI: 10.1159/000549196
Alisha N Mehta, Ramya R Malchira, Kalyani Mehta, Cynthia C Nast
{"title":"Membranoproliferative Glomerulonephritis due to <i>Haemophilus, Aggregatibacter, Cardiobacterium, Eikenella, Kingella</i> Endocarditis in a Patient with Cirrhosis: A Case Report.","authors":"Alisha N Mehta, Ramya R Malchira, Kalyani Mehta, Cynthia C Nast","doi":"10.1159/000549196","DOIUrl":"10.1159/000549196","url":null,"abstract":"<p><strong>Introduction: </strong>Membranoproliferative glomerulonephritis (MPGN) is a rare pattern of glomerular injury that may be immune complex-mediated (ICM), most often secondary to infections, autoimmune disorders, or hematologic or less often solid tumor malignancies. Infections with <i>Haemophilus, Aggregatibacter, Cardiobacterium, Eikenella, Kingella</i> (HACEK) organisms are a well-known but rare cause of infective endocarditis (IE), which may be difficult to detect. Bacterial infections are more common in patients with cirrhosis, which may complicate the interpretation of clinical findings.</p><p><strong>Case presentation: </strong>The patient is a 44-year-old male with alcoholic cirrhosis who presented with hematuria, proteinuria, and acute kidney injury (AKI), with negative blood cultures. Kidney biopsy revealed ICM-MPGN, with no underlying etiology identified. His clinical course was complicated by recurrent fluid overload with AKI requiring dialysis. Following corticosteroid therapy for ICM-MPGN, he developed <i>Aggregatibacter</i> bacteremia associated with worsening mitral regurgitation but without vegetations. After antibiotic therapy, the mitral valve was replaced and showed healed endocarditis. Following prolonged antibiotic therapy and successful surgical intervention, his kidney function improved and he remains dialysis independent.</p><p><strong>Conclusion: </strong>This case highlights the role of HACEK organisms causing subclinical and difficult to diagnose IE, the increased risk of infection in patients with cirrhosis, and the need for a thorough evaluation for infectious etiologies in patients with ICM-MPGN prior to immunosuppressive therapy.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"431-437"},"PeriodicalIF":0.0,"publicationDate":"2025-10-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12659674/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145649816","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}
Glomerular diseasesPub Date : 2025-10-16eCollection Date: 2025-01-01DOI: 10.1159/000548897
Abdelaziz M Abusal, Mohamed Ali, Khaled Murshed, Faten Aqeel
{"title":"Focal Crescentic Pauci-Immune Glomerulonephritis in Systemic Lupus Erythematosus: A Case Report.","authors":"Abdelaziz M Abusal, Mohamed Ali, Khaled Murshed, Faten Aqeel","doi":"10.1159/000548897","DOIUrl":"10.1159/000548897","url":null,"abstract":"<p><strong>Introduction: </strong>We report a rare case of class III lupus nephritis presenting as crescentic pauci-immune glomerulonephritis - an unusual biopsy finding not captured in the current International Society of Nephrology/Renal Pathology Society classification, highlighting a diagnostic and therapeutic challenge.</p><p><strong>Case presentation: </strong>A 24-year-old woman with systemic lupus erythematosus presented with preserved renal function, microscopic hematuria, nephritic-range proteinuria, elevated anti-dsDNA levels, negative antineutrophil cytoplasmic antibody serologies, and a kidney biopsy demonstrating focal crescentic pauci-immune glomerulonephritis with mesangial, subendothelial, and subepithelial electron-dense deposits, consistent with a rare histopathologic variant of class III LN.</p><p><strong>Conclusion: </strong>This case underscores the importance of kidney biopsy in atypical lupus presentations as histopathological findings may not always align with serological or clinical features, and can significantly influence diagnosis, prognosis, and management.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"425-430"},"PeriodicalIF":0.0,"publicationDate":"2025-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12659650/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145649779","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}
Glomerular diseasesPub Date : 2025-09-24eCollection Date: 2025-01-01DOI: 10.1159/000548415
Paulo Cesar Koch-Nogueira, Luciana de Santis Feltran, Maria Fernanda Carvalho de Camargo, Débora Villar Martins, João Bosco Pesquero, Patricia Varela, Andreia Watanabe, Mara Sanches Guaragna, Vera Maria Santoro Belangero, Luiz Fernando Onuchic
{"title":"Epidemiology of Pediatric Nephrotic Syndrome: Real-World Data from Brazil.","authors":"Paulo Cesar Koch-Nogueira, Luciana de Santis Feltran, Maria Fernanda Carvalho de Camargo, Débora Villar Martins, João Bosco Pesquero, Patricia Varela, Andreia Watanabe, Mara Sanches Guaragna, Vera Maria Santoro Belangero, Luiz Fernando Onuchic","doi":"10.1159/000548415","DOIUrl":"10.1159/000548415","url":null,"abstract":"<p><strong>Introduction: </strong>The present study aimed to describe the clinical and demographic characteristics of nephrotic syndrome in children from a large, highly genetically admixed Latin American country and to identify key challenges in managing pediatric nephrotic syndrome in Brazil.</p><p><strong>Methods: </strong>This observational, multicenter study included a patient sample from 21 pediatric nephrology centers. Descriptive statistics were employed to analyze the outcomes.</p><p><strong>Results: </strong>Data were collected from 597 patients; 333 (56%) were male, with a median age of 3.5 years (interquartile range [IQR], 2.2-6.0) at disease onset. There was no consensus among centers regarding initial treatment duration, criteria of response to treatment, although all patients received corticosteroids. After a median follow-up of 4.3 years (IQR, 2.0-7.4), 423 (71.0%) patients were classified as steroid-sensitive, and 172 (28.9%) as steroid-resistant. Progressive kidney dysfunction (chronic kidney disease [CKD] stages 4/5) was observed in 35 (5.9%) patients. Despite 224 patients undergoing kidney biopsy and 172 being classified as steroid-resistant, only 8 (1.3%) children underwent genetic testing. Electron microscopy was performed in only 19 kidney biopsies.</p><p><strong>Conclusion: </strong>Despite the inherent limitations of observational studies, this investigation presents valuable clinical and demographic data on nephrotic syndrome from a previously underrepresented region. It highlights significant unmet medical needs in Brazil, including the need for consensus on treatment protocols and standardized definitions of responses to treatment. In addition, the near absence of genetic testing in steroid-resistant nephrotic syndrome represents a critical gap that requires prompt intervention.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"403-413"},"PeriodicalIF":0.0,"publicationDate":"2025-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12659168/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145650360","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}
Glomerular diseasesPub Date : 2025-09-23eCollection Date: 2025-01-01DOI: 10.1159/000548609
Katherine E Guittari, Young-Hyun Moon, Virginia Pate, Elizabeth O Moreton, Amy Mottl, J Bradley Layton, Dorey A Glenn
{"title":"Pneumococcal Vaccine Use and Immunogenicity in Patients with Glomerular Disease: A Scoping Review and Analysis of Healthcare Claims Data.","authors":"Katherine E Guittari, Young-Hyun Moon, Virginia Pate, Elizabeth O Moreton, Amy Mottl, J Bradley Layton, Dorey A Glenn","doi":"10.1159/000548609","DOIUrl":"10.1159/000548609","url":null,"abstract":"<p><strong>Introduction: </strong><i>Streptococcus pneumoniae</i> infections are a preventable cause of morbidity and mortality in individuals with glomerular disease. There is limited immunogenicity data to inform the effectiveness of vaccination in this high-risk patient population.</p><p><strong>Methods: </strong>We conduct a scoping review of studies evaluating pneumococcal vaccine immunogenicity in adults and children with idiopathic nephrotic syndrome, focal segmental glomerulosclerosis, IgA vasculitis, or systemic lupus erythematosus with nephritis. We summarize patient characteristics, serotypes assessed, and rates of seroprotection and/or seroconversion. We then use the Merative MarketScan® Commercial Claims and Encounters database to describe rates of adequate, partial, and inadequate pneumococcal vaccination (PCV13 and/or PPSV23) within 12 months of incident glomerular disease diagnosis from 2010 to 2018.</p><p><strong>Results: </strong>A total of 879 citations were screened, of which 63 studies were reviewed for inclusion criteria. A final analytic set included 19 studies. Most studies reported clinically meaningful rates of seroprotection, though definitions varied widely across studies. In the analysis of commercial healthcare claims data, 1,343 children aged 2-11 years and 19,987 individuals aged 12 to <65 years with incident of GD were identified. Of those inadequately vaccinated prior to GD diagnosis, 4.0% (38/947) of the 2-11-year group and 6.8% (797/11,646) of those in the 12 to <65-year group were partially or adequately vaccinated within 12 months of diagnosis. Demographic and clinical factors more commonly identified in those with pneumococcal vaccination included sex, age, region, and prior influenza vaccination.</p><p><strong>Conclusion: </strong>There is limited literature describing pneumococcal vaccine immunogenicity in patients with glomerular disease. Metrics of reporting seroprotection and seroconversion to pneumococcal vaccines would benefit from standardization. Healthcare claims for pneumococcal vaccines following a diagnosis of glomerular disease suggest that efforts to improve timely vaccination are needed.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"414-424"},"PeriodicalIF":0.0,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12643526/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145607441","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}
Glomerular diseasesPub Date : 2025-08-22eCollection Date: 2025-01-01DOI: 10.1159/000548151
Martin Benjamin Yama Estrella, Mario Alamilla-Sanchez, Carolina Gonzalez-Fuentes, Nicte Alaide Ramos Garcia, Victor Manuel Ulloa Galván, Mayra Matias Carmona, José Cano Cervantes, Regina Canade Hernández Hernández
{"title":"Collapsing Glomerulopathy during Pregnancy: A Case Series.","authors":"Martin Benjamin Yama Estrella, Mario Alamilla-Sanchez, Carolina Gonzalez-Fuentes, Nicte Alaide Ramos Garcia, Victor Manuel Ulloa Galván, Mayra Matias Carmona, José Cano Cervantes, Regina Canade Hernández Hernández","doi":"10.1159/000548151","DOIUrl":"10.1159/000548151","url":null,"abstract":"<p><strong>Introduction: </strong>Preeclampsia, a leading cause of morbidity during pregnancy, is associated with glomerular endotheliosis, fibrin deposition, and thrombotic microangiopathy and is characterized by edema, proteinuria, and acute kidney injury. Preeclampsia has been described on a background of glomerular disease membranous nephropathy, IgA nephropathy, and focal segmental glomerulosclerosis, but biopsy studies have also described the de novo diagnosis of glomerulopathy as thrombotic microangiopathy, endotheliosis or collapsing glomerulopathy in the setting of preeclampsia.</p><p><strong>Case presentations: </strong>We report 3 cases of preeclampsia-associated collapsing focal and segmental glomerulosclerosis in the third trimester of gestation, two of which were previously healthy and one with a history of chronic hypertension that presented with nephrotic-range proteinuria without secondary causes detected. It was decided to begin with antiproteinuric treatment after delivery, resulting in a complete response without the need for immunosuppressant drugs. The outcomes of these cases suggest that a favorable evolution is expected once preeclampsia had resolved and therefore the glomerular changes had been reversed.</p><p><strong>Conclusion: </strong>A subgroup of pregnant patients can be managed without exposing the mother-child pair to adverse effects related to immunosuppression when preeclampsia is detected in the third trimester of gestation.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"395-402"},"PeriodicalIF":0.0,"publicationDate":"2025-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12503771/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145253867","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}
Glomerular diseasesPub Date : 2025-08-19eCollection Date: 2025-01-01DOI: 10.1159/000548082
Kruti Gitesh Shah, Sharon G Adler, Tiane Dai, Cynthia C Nast
{"title":"Calcific Changes in an End-Stage Kidney following Long-Term Dialysis, Tertiary Hyperparathyroidism, and Treatment for Complement-Mediated Thrombotic Microangiopathy: A Case Report.","authors":"Kruti Gitesh Shah, Sharon G Adler, Tiane Dai, Cynthia C Nast","doi":"10.1159/000548082","DOIUrl":"10.1159/000548082","url":null,"abstract":"<p><strong>Introduction: </strong>There are few descriptions of glomerular calcification in patients with advanced or end-stage kidney disease (ESKD). There also are limited data on long-term outcomes for patients receiving complement factor 5 inhibitor (C5i) treatment for complement-mediated thrombotic microangiopathy (CM-TMA), previously termed atypical hemolytic uremic syndrome, associated with a complement factor I (CFI) mutation.</p><p><strong>Case presentation: </strong>Here we report a case of ESKD from CM-TMA in a patient who developed tertiary hyperparathyroidism. Due to cerebral symptoms (focal paresthesias) of TMA, he received long-term treatment with a C5i. A nephrectomy subsequently was performed for renal cell carcinoma and showed diffuse glomerular, in addition to focal arterial and tubular basement membrane, calcification. There also was chronic TMA associated with continued C5i treatment, with no evidence of recurrent thrombosis consistent with quiescent systemic TMA activity.</p><p><strong>Conclusion: </strong>Glomerular calcification is rare, and it is unknown if this is related to the treated hyperparathyroidism or other pathogenetic mechanisms. The nephrectomy findings also suggest that patients with pathogenic mutations in CFI may benefit from long-term, likely lifelong, complement inhibitory treatment.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"374-379"},"PeriodicalIF":0.0,"publicationDate":"2025-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12503778/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145253695","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}
Glomerular diseasesPub Date : 2025-08-07eCollection Date: 2025-01-01DOI: 10.1159/000547744
Smeeta Sinha, Jonathan de Courcy, Susanna Libby, Alice Simons, Briana Ndife, Katharina Pannagl, Clare Proudfoot, Raymond Przybysz, Raisa Sidhu, Serge Smeets, Richard A Lafayette
{"title":"Treatment and Disease Burden in Patients with Complement 3 Glomerulopathy: Multinational Real-World Study Results.","authors":"Smeeta Sinha, Jonathan de Courcy, Susanna Libby, Alice Simons, Briana Ndife, Katharina Pannagl, Clare Proudfoot, Raymond Przybysz, Raisa Sidhu, Serge Smeets, Richard A Lafayette","doi":"10.1159/000547744","DOIUrl":"10.1159/000547744","url":null,"abstract":"<p><strong>Introduction: </strong>Patients with complement 3 glomerulopathy (C3G) have poor clinical outcomes, although new targeted therapies have very recently been approved. Here, we describe the burden and management of C3G in global clinical practice.</p><p><strong>Methods: </strong>Retrospective analysis of data obtained from a cross-sectional survey (Adelphi Real World C3G Disease Specific Programme™) of nephrologists actively managing C3G (<i>N</i> = 195) and their patients (<i>N</i> = 385) in France, Germany, Italy, Spain, the UK (EU5), the USA, China, and Japan (July 2022-April 2023). Information on patient demographics, clinical characteristics, diagnosis journey, treatment patterns, dialysis and kidney transplant information, and patient-reported outcomes was collected from nephrologist-completed patient record forms (PRFs) and patient self-completion forms (PSCFs). Results were reported using descriptive statistics.</p><p><strong>Results: </strong>Records for 385 patients with C3G were completed by 129 nephrologists. Most patients had moderate to severe disease at the time of diagnosis (85.1%), based on individual nephrologist assessment. At the time of PRF completion, 83.4% of patients were receiving pharmacological treatment, which included angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers (70.4%) and corticosteroids (48.6%). In patients receiving treatment, proteinuria remained the most common nephrologist-reported sign of C3G (66.0%), irrespective of treatment duration; of patients with available data, 68.9% had proteinuria ≥1 g/day. At the time of PRF completion, 8.3% of patients were on dialysis, 41.6% of patients were considered eligible for a kidney transplant, and 6.5% were transplant recipients. Patients experienced anxiety/depression (73.7%), pain/discomfort (65.3%), fatigue (90.7%), and problems doing usual activities (62.7%) at the time of PSCF completion.</p><p><strong>Conclusion: </strong>Despite most patients receiving current guideline-recommended standard of care, the burden of C3G remains high worldwide, demonstrating the need for more effective treatment options. In addition, most patients presented with advanced disease by the time of diagnosis, warranting a need to address significant diagnostic delays to facilitate earlier therapeutic intervention.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"380-394"},"PeriodicalIF":0.0,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12503786/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145253871","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}
Glomerular diseasesPub Date : 2025-07-23eCollection Date: 2025-01-01DOI: 10.1159/000547588
Mythri Shankar, Anaghashree Udayashankar
{"title":"Heat, Humidity, and Hematuria: Glomerular Complications of Tropical Infections.","authors":"Mythri Shankar, Anaghashree Udayashankar","doi":"10.1159/000547588","DOIUrl":"10.1159/000547588","url":null,"abstract":"<p><strong>Background: </strong>Tropical regions, home to nearly 40% of the world's population, face a high burden of infectious diseases due to climate, socioeconomic factors, and limited healthcare access. Many tropical infections - including malaria, dengue, leptospirosis, schistosomiasis, filariasis, scrub typhus, and human immunodeficiency virus (HIV) - have been increasingly recognized as causes of glomerular disease. These infections can directly or indirectly affect the kidney, resulting in a diverse spectrum of glomerular pathologies.</p><p><strong>Summary: </strong>This review highlights the epidemiology, clinical manifestations, pathophysiology, and renal histopathological findings associated with major tropical infections that involve the glomeruli. It describes how parasitic, bacterial, and viral pathogens trigger immune-mediated glomerular injury, contribute to acute kidney injury, or lead to chronic kidney disease. Specific glomerular lesions, including mesangioproliferative glomerulonephritis (GN), membranoproliferative GN, focal segmental glomerulosclerosis, and HIV-associated nephropathy, are detailed with mechanistic insights. The article also discusses diagnostic challenges; therapeutic approaches, including antiparasitic and antiretroviral therapy; and the role of preventive strategies such as vaccination, vector control, and mass drug administration.</p><p><strong>Key message: </strong>Tropical infections are increasingly recognized as important yet underappreciated contributors to glomerular disease, particularly in low-resource settings. Early recognition and timely targeted treatment of infection-related GN can significantly reduce the risk of long-term kidney damage. To effectively mitigate the kidney disease burden, comprehensive public health measures - including enhanced surveillance, vaccination initiatives, and integrated vector control strategies - are essential.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"5 1","pages":"352-373"},"PeriodicalIF":0.0,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12503647/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145253894","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}