The renal tubule and collecting duct express a large number of proteins, all having putative immunoreactive motives. Therefore, all can be the target of pathogenic autoantibodies. However, autoimmune tubulopathies seem to be rare, and we hypothesize that they are underdiagnosed. This review summarizes the current knowledge on autoimmune tubulopathies. We elected to classify tubulopathies according to the segment that is targeted because this determines, at least in part, the phenotypic presentation. In the proximal tubule, autoantibodies can cause anti-brush border antibody disease, renal Fanconi syndrome, renal proximal tubular acidosis, or tubulointerstitial nephritis and uveitis syndrome. Autoantibodies targeting the thick ascending limb of the loop of Henle can cause either acquired Bartter syndrome or hypomagnesemia with hypercalciuria, whereas autoantibodies targeting the distal convoluted tubule can cause acquired Gitelman syndrome. Finally, renal distal tubular acidosis or nephrogenic diabetes insipidus can be caused by autoantibodies targeting the collecting duct. In most instances, the characterization of the autoantibodies remains incomplete and the pathogenesis of the disease obscure. We believe it is important to increase the awareness of physicians regarding autoantibody-mediated tubular diseases to have a better estimation of the prevalence and to improve the care to patients. A research effort to increase the understanding of the pathogenesis of autoantibodies-mediated tubular diseases is also hoped for.
{"title":"Autoimmune Tubulopathies.","authors":"Pascal Houillier, Caroline Prot-Bertoye","doi":"10.1681/ASN.0000000628","DOIUrl":"10.1681/ASN.0000000628","url":null,"abstract":"<p><p>The renal tubule and collecting duct express a large number of proteins, all having putative immunoreactive motives. Therefore, all can be the target of pathogenic autoantibodies. However, autoimmune tubulopathies seem to be rare, and we hypothesize that they are underdiagnosed. This review summarizes the current knowledge on autoimmune tubulopathies. We elected to classify tubulopathies according to the segment that is targeted because this determines, at least in part, the phenotypic presentation. In the proximal tubule, autoantibodies can cause anti-brush border antibody disease, renal Fanconi syndrome, renal proximal tubular acidosis, or tubulointerstitial nephritis and uveitis syndrome. Autoantibodies targeting the thick ascending limb of the loop of Henle can cause either acquired Bartter syndrome or hypomagnesemia with hypercalciuria, whereas autoantibodies targeting the distal convoluted tubule can cause acquired Gitelman syndrome. Finally, renal distal tubular acidosis or nephrogenic diabetes insipidus can be caused by autoantibodies targeting the collecting duct. In most instances, the characterization of the autoantibodies remains incomplete and the pathogenesis of the disease obscure. We believe it is important to increase the awareness of physicians regarding autoantibody-mediated tubular diseases to have a better estimation of the prevalence and to improve the care to patients. A research effort to increase the understanding of the pathogenesis of autoantibodies-mediated tubular diseases is also hoped for.</p>","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":10.3,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142950716","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Exon Skipping Therapies for Rare Kidney Diseases.","authors":"Tomohiko Yamamura, Kandai Nozu","doi":"10.1681/ASN.0000000623","DOIUrl":"10.1681/ASN.0000000623","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":10.3,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142950718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Advancing Understanding of Cardiovascular Risk in CKD.","authors":"Vikas S Sridhar, Ayodele Odutayo","doi":"10.1681/ASN.0000000606","DOIUrl":"https://doi.org/10.1681/ASN.0000000606","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":10.3,"publicationDate":"2025-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142962303","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mengyao Tang, Mario Luca Morieri, Sahir Kalim, Alessandro Doria
{"title":"Combination Therapy with SGLT2 Inhibitors and GLP-1 Receptor Agonists for Diabetic Kidney Disease.","authors":"Mengyao Tang, Mario Luca Morieri, Sahir Kalim, Alessandro Doria","doi":"10.1681/ASN.0000000620","DOIUrl":"10.1681/ASN.0000000620","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":10.3,"publicationDate":"2025-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142950717","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Absent, Small, or Homeotic 2-Like-Mediated H3K4 Methylation and Nephrogenesis.","authors":"Ziyi Zhao, Xuantong Dai, Gengru Jiang, Fujun Lin","doi":"10.1681/ASN.0000000600","DOIUrl":"10.1681/ASN.0000000600","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":10.3,"publicationDate":"2025-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142950715","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-01Epub Date: 2024-12-03DOI: 10.1681/ASN.0000000578
Jenny I Shen, Jeffrey Perl
{"title":"Disparities in Care: Desperate for Modality Education.","authors":"Jenny I Shen, Jeffrey Perl","doi":"10.1681/ASN.0000000578","DOIUrl":"10.1681/ASN.0000000578","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":"16-18"},"PeriodicalIF":10.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11706551/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142770098","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-01Epub Date: 2024-08-26DOI: 10.1681/ASN.0000000000000477
L Parker Gregg, Peter A Richardson, Vijay Nambi, Laura A Petersen, Michael E Matheny, Salim S Virani, Sankar D Navaneethan
{"title":"Sodium-Glucose Cotransporter-2 Inhibitor and Glucagon-Like Peptide-1 Receptor Agonist Discontinuation in Patients with CKD.","authors":"L Parker Gregg, Peter A Richardson, Vijay Nambi, Laura A Petersen, Michael E Matheny, Salim S Virani, Sankar D Navaneethan","doi":"10.1681/ASN.0000000000000477","DOIUrl":"10.1681/ASN.0000000000000477","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":"87-98"},"PeriodicalIF":10.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11706553/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142073167","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"ST2 + T-Regulatory Cells in Renal Inflammation and Fibrosis after Ischemic Kidney Injury.","authors":"Vikram Sabapathy, Airi Price, Nardos Tesfaye Cheru, Rajkumar Venkatadri, Murat Dogan, Gabrielle Costlow, Saleh Mohammad, Rahul Sharma","doi":"10.1681/ASN.0000000000000471","DOIUrl":"10.1681/ASN.0000000000000471","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":"73-86"},"PeriodicalIF":10.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11706559/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142073168","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-01Epub Date: 2024-11-05DOI: 10.1681/ASN.0000000569
Christopher J Pino, Kimberly A Johnston, Angela J Westover, H David Humes
{"title":"Selective Cytopheretic Device (QUELimmune): A Leukocyte Processing, Immunomodulatory Device.","authors":"Christopher J Pino, Kimberly A Johnston, Angela J Westover, H David Humes","doi":"10.1681/ASN.0000000569","DOIUrl":"10.1681/ASN.0000000569","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":"144-146"},"PeriodicalIF":10.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11706555/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142583676","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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