BACKGROUNDPeritoneal fibrosis is a serious complication of long-term peritoneal dialysis (PD) and abdominal surgeries, yet effective treatments remain elusive. Given the known roles of mucosal-associated invariant T (MAIT) cells in immune responses and fibrotic diseases, we investigated their involvement in PD-induced peritoneal fibrosis to identify potential therapeutic targets.METHODSWe employed single-cell RNA sequencing (scRNA-seq) and flow cytometry to characterize the activation and function of peritoneal MAIT cells in patients undergoing long-term PD. Our investigation focused on the molecular pathways activated by these cells, particularly the MHC class I-related protein 1 (MR1)-mediated interaction with mesothelial cells and subsequent activation of the mTORC1 signaling pathway. We further assessed the impact of inhibiting MAIT cells on fibrogenesis using both in vitro models and Mr1 knockout mice.RESULTSOur study revealed that long-term PD significantly enhanced the activation of MAIT cells, particularly the pro-inflammatory MAIT17 subtype. These activated cells contributed to peritoneal fibrogenesis by binding to the MR1 receptor on mesothelial cells, which triggered hyperglycolysis through the mTORC1 pathway, ultimately leading to fibrogenesis. Notably, we demonstrated that blocking the MR1-MAIT interaction, either through genetic knockout or pharmacological inhibition with acetyl-6-formylpterin (Ac-6-FP), effectively mitigated fibrosis.CONCLUSIONSThis study identified MAIT cells as crucial drivers of PD-induced peritoneal fibrosis.
{"title":"MAIT Cell-Mediated Immune Mechanisms of Dialysis-Induced Peritoneal Fibrosis and Therapeutic Targeting.","authors":"Yuxiang Sun,Qiang Huang,Juan Sun,Hu Zhou,Dandan Guo,Long Peng,Hongchun Lin,Canming Li,Hongli Shang,Tongtong Wang,Yanxu Chen,Yong Huang,Cheng Hu,Zhaoyong Hu,Yan Lu,Hui Peng","doi":"10.1681/asn.0000000627","DOIUrl":"https://doi.org/10.1681/asn.0000000627","url":null,"abstract":"BACKGROUNDPeritoneal fibrosis is a serious complication of long-term peritoneal dialysis (PD) and abdominal surgeries, yet effective treatments remain elusive. Given the known roles of mucosal-associated invariant T (MAIT) cells in immune responses and fibrotic diseases, we investigated their involvement in PD-induced peritoneal fibrosis to identify potential therapeutic targets.METHODSWe employed single-cell RNA sequencing (scRNA-seq) and flow cytometry to characterize the activation and function of peritoneal MAIT cells in patients undergoing long-term PD. Our investigation focused on the molecular pathways activated by these cells, particularly the MHC class I-related protein 1 (MR1)-mediated interaction with mesothelial cells and subsequent activation of the mTORC1 signaling pathway. We further assessed the impact of inhibiting MAIT cells on fibrogenesis using both in vitro models and Mr1 knockout mice.RESULTSOur study revealed that long-term PD significantly enhanced the activation of MAIT cells, particularly the pro-inflammatory MAIT17 subtype. These activated cells contributed to peritoneal fibrogenesis by binding to the MR1 receptor on mesothelial cells, which triggered hyperglycolysis through the mTORC1 pathway, ultimately leading to fibrogenesis. Notably, we demonstrated that blocking the MR1-MAIT interaction, either through genetic knockout or pharmacological inhibition with acetyl-6-formylpterin (Ac-6-FP), effectively mitigated fibrosis.CONCLUSIONSThis study identified MAIT cells as crucial drivers of PD-induced peritoneal fibrosis.","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":"62 1","pages":""},"PeriodicalIF":13.6,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143056897","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}
Ning Shi, Ji-Wen Wang, Gengchen Su, Gaoxiang Ma, Feng-Qing Huang, Si-Jia Jin, Hua-Mei Xie, Wen-Xin Ge, Jiang-Ping Song, Xiaodong Luan, Lei Zhang, Lian-Wen Qi
{"title":"N -Acetyl-Tryptophan in Acute Kidney Injury after Cardiac Surgery.","authors":"Ning Shi, Ji-Wen Wang, Gengchen Su, Gaoxiang Ma, Feng-Qing Huang, Si-Jia Jin, Hua-Mei Xie, Wen-Xin Ge, Jiang-Ping Song, Xiaodong Luan, Lei Zhang, Lian-Wen Qi","doi":"10.1681/ASN.0000000626","DOIUrl":"10.1681/ASN.0000000626","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":10.3,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143023941","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}
Kimberly R Schildknecht, Molly Deutsch-Feldman, Jason Cummins, Divia P Forbes, Maryam B Haddad, Ibironke W Apata, Jonathan M Wortham
{"title":"Tuberculosis in the US Kidney Failure Population.","authors":"Kimberly R Schildknecht, Molly Deutsch-Feldman, Jason Cummins, Divia P Forbes, Maryam B Haddad, Ibironke W Apata, Jonathan M Wortham","doi":"10.1681/ASN.0000000621","DOIUrl":"10.1681/ASN.0000000621","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":10.3,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143023942","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}
Nina Himmerkus, Catarina Quintanova, Harneet Bhullar, Wouter H van Megen, Amanda Lima Deluque, Karsten Skjødt, Milos Bogdanovic, Markus Bleich, R Todd Alexander, Henrik Dimke
{"title":"Calcium-Sensing Receptor in the Thick Ascending Limb and Renal Response to Hypercalcemia.","authors":"Nina Himmerkus, Catarina Quintanova, Harneet Bhullar, Wouter H van Megen, Amanda Lima Deluque, Karsten Skjødt, Milos Bogdanovic, Markus Bleich, R Todd Alexander, Henrik Dimke","doi":"10.1681/ASN.0000000612","DOIUrl":"10.1681/ASN.0000000612","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":10.3,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143007455","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":"Finding and Profiling Renal Cells with Spatial Transcriptomics.","authors":"Katherine R Bull","doi":"10.1681/ASN.0000000632","DOIUrl":"10.1681/ASN.0000000632","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":10.3,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143007449","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}
Lilia Cervantes,Katherine Rizzolo,Sri Lekha Tummalapalli,Marina Wainstein,Russell E Glasgow,Mónica Pérez Jolles
{"title":"Use of a Context- and Equity-Focused Implementation Science Framework to Aid the Design of Clinical Trials.","authors":"Lilia Cervantes,Katherine Rizzolo,Sri Lekha Tummalapalli,Marina Wainstein,Russell E Glasgow,Mónica Pérez Jolles","doi":"10.1681/asn.0000000633","DOIUrl":"https://doi.org/10.1681/asn.0000000633","url":null,"abstract":"","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":"14 1","pages":""},"PeriodicalIF":13.6,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142988790","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}
Jason H Greenberg,Alison G Abraham,Yunwen Xu,Jeffrey R Schelling,Steven G Coca,Sarah J Schrauben,F Perry Wilson,Sushrut S Waikar,Ramachandran S Vasan,Orlando M Gutierrez,Michael G Shlipak,Joachim H Ix,Bradley A Warady,Paul L Kimmel,Joseph V Bonventre,Chirag R Parikh,Michelle Denburg,Susan Furth,
BACKGROUNDWe have previously studied biomarkers of tubular health (EGF), injury (KIM-1), dysfunction (alpha-1 microglobulin), and inflammation (TNFR-1, TNFR-2, MCP-1, YKL-40, suPAR), and demonstrated that plasma KIM-1, TNFR-1, TNFR-2 and urine KIM-1, EGF, MCP-1, urine alpha-1 microglobulin are each independently associated with CKD progression in children. In this study, we used bootstrapped survival trees to identify a combination of biomarkers to predict CKD progression in children.METHODSThe CKiD Cohort Study prospectively enrolled children 6 months to 16 years old with an eGFR of 30-90 ml/min/1.73m2. We measured biomarkers in stored plasma and urine collected 5 months after study enrollment. The primary outcome of CKD progression was a composite of 50% eGFR decline or kidney failure. We constructed a regression tree-based model for predicting the time to the composite event, using a panel of clinically relevant biomarkers with empirically derived thresholds, in addition to conventional risk factors.RESULTSOf the 599 children included, the median age was 12 years [IQR, 8 - 15], 371 (62%) were male, baseline urine protein to creatinine ratio was 0.33 [IQR: 0.12 - 0.95] mg/mg, and baseline eGFR was 53 [IQR, 40 - 66] ml/min/1.73m2. Overall, 205 (34%) children reached the primary outcome of CKD. A single regression tree-based model using the most informative predictors with data driven biomarker thresholds suggested a final set of 4 prognosis groups. In the final model, urine albumin/creatinine was the variable with the highest importance, and along with urine EGF/creatinine identified the highest risk group of 24 children, 100% of whom developed CKD progression at a median time of 1.3 years [95% CI: 1.0, 1.7]. When the regression tree-derived risk group classifications were added to prediction models including the clinical risk factors, the C-statistic increased from 0.76 [95%CI: 0.71 - 0.80] to 0.85 [95%CI: 0.81 - 0.88].CONCLUSIONSUsing regression tree-based methods, we identified a biomarker panel of urine albumin/creatinine, urine EGF/creatinine, plasma KIM-1, and eGFR which significantly improved discrimination for CKD progression.
{"title":"Biomarker Panels for Discriminating Risk of CKD Progression in Children.","authors":"Jason H Greenberg,Alison G Abraham,Yunwen Xu,Jeffrey R Schelling,Steven G Coca,Sarah J Schrauben,F Perry Wilson,Sushrut S Waikar,Ramachandran S Vasan,Orlando M Gutierrez,Michael G Shlipak,Joachim H Ix,Bradley A Warady,Paul L Kimmel,Joseph V Bonventre,Chirag R Parikh,Michelle Denburg,Susan Furth,","doi":"10.1681/asn.0000000602","DOIUrl":"https://doi.org/10.1681/asn.0000000602","url":null,"abstract":"BACKGROUNDWe have previously studied biomarkers of tubular health (EGF), injury (KIM-1), dysfunction (alpha-1 microglobulin), and inflammation (TNFR-1, TNFR-2, MCP-1, YKL-40, suPAR), and demonstrated that plasma KIM-1, TNFR-1, TNFR-2 and urine KIM-1, EGF, MCP-1, urine alpha-1 microglobulin are each independently associated with CKD progression in children. In this study, we used bootstrapped survival trees to identify a combination of biomarkers to predict CKD progression in children.METHODSThe CKiD Cohort Study prospectively enrolled children 6 months to 16 years old with an eGFR of 30-90 ml/min/1.73m2. We measured biomarkers in stored plasma and urine collected 5 months after study enrollment. The primary outcome of CKD progression was a composite of 50% eGFR decline or kidney failure. We constructed a regression tree-based model for predicting the time to the composite event, using a panel of clinically relevant biomarkers with empirically derived thresholds, in addition to conventional risk factors.RESULTSOf the 599 children included, the median age was 12 years [IQR, 8 - 15], 371 (62%) were male, baseline urine protein to creatinine ratio was 0.33 [IQR: 0.12 - 0.95] mg/mg, and baseline eGFR was 53 [IQR, 40 - 66] ml/min/1.73m2. Overall, 205 (34%) children reached the primary outcome of CKD. A single regression tree-based model using the most informative predictors with data driven biomarker thresholds suggested a final set of 4 prognosis groups. In the final model, urine albumin/creatinine was the variable with the highest importance, and along with urine EGF/creatinine identified the highest risk group of 24 children, 100% of whom developed CKD progression at a median time of 1.3 years [95% CI: 1.0, 1.7]. When the regression tree-derived risk group classifications were added to prediction models including the clinical risk factors, the C-statistic increased from 0.76 [95%CI: 0.71 - 0.80] to 0.85 [95%CI: 0.81 - 0.88].CONCLUSIONSUsing regression tree-based methods, we identified a biomarker panel of urine albumin/creatinine, urine EGF/creatinine, plasma KIM-1, and eGFR which significantly improved discrimination for CKD progression.","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":"99 1","pages":""},"PeriodicalIF":13.6,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142988795","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}
Jingbo Niu,Omar Rosales,Abiodun Oluyomi,Susie Q Lew,Glenn M Chertow,Wolfgang C Winkelmayer,Kevin F Erickson
BACKGROUNDIn March 2020, responding to the COVID-19 pandemic, federal emergency waivers in the United States enabled kidney care providers (nephrologists and advanced practice providers) to substitute face-to-face in-center hemodialysis visits with telemedicine encounters. We examined whether the frequency of kidney care provider visits and hospitalizations were associated with telemedicine use in hemodialysis care.METHODSWe used Medicare claims to identify US patients receiving in-center hemodialysis during the first 16 months of the COVID-19 pandemic. We examined the association between telemedicine use during in-center hemodialysis, the frequency with which kidney care providers visited patients at dialysis four-or-more times per month, and hospitalizations. We also examined whether the association between telemedicine use and visit frequency varied at facilities located in more remote areas. Multivariable regression models adjusted for patient, physician, geographic and dialysis facility characteristics along with the frequency with which kidney care providers saw patients at each facility before the pandemic. We focused on kidney care providers who demonstrated knowledge of how to bill for telemedicine visits by using the telemedicine modifier on prior claims.RESULTSWe identified 1,881 providers who saw patients between 3/2020-6/2021 and were definitively using telemedicine. In the adjusted model, a 35% absolute higher use of telemedicine at a facility (representing one standard deviation difference) was associated with a 1.4% higher rate of four-or-more visits (Incidence Rate Ratio (IRR) 1.014; 95% Confidence Interval 1.007-1.022). The association between telemedicine use and visit frequency was stronger where travel distances to facilities were farther (interaction p=0.01). There was no significant association between telemedicine use and hospitalizations.CONCLUSIONSThe use of telemedicine to care for patients receiving in-center hemodialysis was associated with a slightly higher frequency of four-or-more visits per month but not with hospitalizations; the association with visit frequency was more pronounced in areas where providers had to travel longer distances to see patients in-person.
{"title":"Utilization of Telemedicine for Patients Receiving In-center Hemodialysis in the United States.","authors":"Jingbo Niu,Omar Rosales,Abiodun Oluyomi,Susie Q Lew,Glenn M Chertow,Wolfgang C Winkelmayer,Kevin F Erickson","doi":"10.1681/asn.0000000619","DOIUrl":"https://doi.org/10.1681/asn.0000000619","url":null,"abstract":"BACKGROUNDIn March 2020, responding to the COVID-19 pandemic, federal emergency waivers in the United States enabled kidney care providers (nephrologists and advanced practice providers) to substitute face-to-face in-center hemodialysis visits with telemedicine encounters. We examined whether the frequency of kidney care provider visits and hospitalizations were associated with telemedicine use in hemodialysis care.METHODSWe used Medicare claims to identify US patients receiving in-center hemodialysis during the first 16 months of the COVID-19 pandemic. We examined the association between telemedicine use during in-center hemodialysis, the frequency with which kidney care providers visited patients at dialysis four-or-more times per month, and hospitalizations. We also examined whether the association between telemedicine use and visit frequency varied at facilities located in more remote areas. Multivariable regression models adjusted for patient, physician, geographic and dialysis facility characteristics along with the frequency with which kidney care providers saw patients at each facility before the pandemic. We focused on kidney care providers who demonstrated knowledge of how to bill for telemedicine visits by using the telemedicine modifier on prior claims.RESULTSWe identified 1,881 providers who saw patients between 3/2020-6/2021 and were definitively using telemedicine. In the adjusted model, a 35% absolute higher use of telemedicine at a facility (representing one standard deviation difference) was associated with a 1.4% higher rate of four-or-more visits (Incidence Rate Ratio (IRR) 1.014; 95% Confidence Interval 1.007-1.022). The association between telemedicine use and visit frequency was stronger where travel distances to facilities were farther (interaction p=0.01). There was no significant association between telemedicine use and hospitalizations.CONCLUSIONSThe use of telemedicine to care for patients receiving in-center hemodialysis was associated with a slightly higher frequency of four-or-more visits per month but not with hospitalizations; the association with visit frequency was more pronounced in areas where providers had to travel longer distances to see patients in-person.","PeriodicalId":17217,"journal":{"name":"Journal of The American Society of Nephrology","volume":"30 1","pages":""},"PeriodicalIF":13.6,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142988791","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}
Jordan B Strom, Brandon M Herbert, Marnie Bertolet, Maria M Brooks, Shahbaz A Malik, Gilles Lemesle, Mina Madan, Philippe Gabriel Steg, Paul C Hebert, Jay H Traverse, Harvey D White, Caroline Alsweiler, Rajesh Gupta, Luiz Eduardo F Ritt, Mark A Menegus, John H Alexander, Renato D Lopes, Bernard R Chaitman, Jeffrey L Carson
{"title":"Restrictive or Liberal Blood Transfusion in Patients with Myocardial Infarction and CKD.","authors":"Jordan B Strom, Brandon M Herbert, Marnie Bertolet, Maria M Brooks, Shahbaz A Malik, Gilles Lemesle, Mina Madan, Philippe Gabriel Steg, Paul C Hebert, Jay H Traverse, Harvey D White, Caroline Alsweiler, Rajesh Gupta, Luiz Eduardo F Ritt, Mark A Menegus, John H Alexander, Renato D Lopes, Bernard R Chaitman, Jeffrey L Carson","doi":"10.1681/ASN.0000000595","DOIUrl":"10.1681/ASN.0000000595","url":null,"abstract":"","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":"142950721","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}
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}
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