Yiqin Zuo, Livia Barreira Cavalcante, James Monroe Smelser, Neil Sanghani, Jamie P Dwyer, Julia Breyer Lewis, Agnes B Fogo
{"title":"Anti-Phospholipase A2 Receptor in Nonlupus Patients with Membranous Nephropathy and Crescents.","authors":"Yiqin Zuo, Livia Barreira Cavalcante, James Monroe Smelser, Neil Sanghani, Jamie P Dwyer, Julia Breyer Lewis, Agnes B Fogo","doi":"10.1159/000520641","DOIUrl":"https://doi.org/10.1159/000520641","url":null,"abstract":"<p><strong>Introduction: </strong>Anti-phospholipase A2 receptor (PLA2R) is detected in approximately 70% of biopsies of \"primary\" membranous nephropathy (MN). Crescents in MN in nonlupus patients suggest additional injury, such as antineutrophil cytoplasmic antibody (ANCA) or anti-glomerular basement membrane (anti-GBM)-associated glomerulonephritis and are postulated to reflect injury by a mechanism that unmasks cryptic epitopes leading to the second autoantibody.</p><p><strong>Methods: </strong>We studied PLA2R staining in nonlupus patients with MN and crescents. Native renal biopsies in 16 nonlupus patients with MN and crescents were stained for PLA2R.</p><p><strong>Results: </strong>The patients included 5 women and 11 men, with mean age 61 years and elevated serum creatinine (mean 4.68 mg/dL). Hematuria and proteinuria (mean 4.97 g/day) were documented in 13 patients. Two patients had positive serum anti-GBM antibody. Nine of 11 patients tested for ANCA were positive, with p-ANCA (<i>n</i> = 4), c-ANCA (<i>n</i> = 2), or both (<i>n</i> = 1), with 2 not specified. On average, 27% of glomeruli had crescents. One patient had an initial biopsy with MN, 4 years later had MN with crescent, and 7 years later had rebiopsy with persistent MN with crescents. One patient had ANCA-associated vasculitis, and 5 years later had MN and crescent. The remaining 14 patients had concurrent diagnoses of MN and crescents. PLA2R was positive in 5 cases, 3 with ANCA positivity, 2 with unknown ANCA status, and none with anti-GBM disease. The patient with initial MN preceding crescent was PLA2R positive; the patient with initial ANCA-associated vasculitis preceding MN was PLA2R negative.</p><p><strong>Conclusions: </strong>Most patients (64%) presented with concomitant MN and crescents, with rare occurrence of an initial disease process followed later by the second injury. PLA2R was positive in 31% of patients, suggesting most are secondary MN. Further study to determine the cryptic epitopes may shed light on the triggering mechanisms for these rare but unlikely coincidental glomerular injuries.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 2","pages":"75-82"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/74/2d/gdz-0002-0075.PMC9670025.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10674929","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":"KM55 in the Evaluation of IgA-Containing Glomerular Diseases.","authors":"Rahul Raj, Alok Sharma, Adarsh Barwad, Soumita Bagchi, Sanjay Kumar Agarwal, Arvind Bagga, Amit Kumar Dinda, Geetika Singh","doi":"10.1159/000520640","DOIUrl":"https://doi.org/10.1159/000520640","url":null,"abstract":"<p><strong>Introduction: </strong>Mucosal-derived galactose-deficient IgA is central to the pathogenesis of primary IgA nephropathy (IgAN). Recent reports suggest similar pathogenesis in Henoch-Schonlein purpura (HSP) and secondary IgAN. Its role in other IgA-containing glomerular diseases is still under investigation. It can be detected in glomeruli with the recently described antibody KM55. We aimed to evaluate the role of KM55 by immunostaining a wide spectrum of IgA-containing glomerular diseases.</p><p><strong>Methods: </strong>After standardization and colocalization in a case of IgAN, a spectrum of 60 cases including IgAN, HSP, chronic liver disease (CLD)-related IgAN, other secondary IgAN, IgA-dominant/codominant membranoproliferative glomerulonephritis (MPGN), and lupus nephritis were subjected to immunofluorescence with KM55. KM55 was used to resolve diagnostic dilemma in cases of IgA deposition with confounding histology.</p><p><strong>Results: </strong>The group of primary IgAN (17 cases), HSP (4 cases), and secondary IgAN (19 cases) including CLD showed 2-3+ granular staining with KM55, suggesting mucosal-derived IgA. In contrast, cases of IgA-dominant/codominant MPGN (8 cases) and lupus nephritis (12 cases) were negative for KM55, suggesting systemic derivation of IgA. In cases of IgA deposition with confounding histology such as membranoproliferative or diffuse endocapillary proliferative pattern, KM55 helped to resolve the diagnosis.</p><p><strong>Discussion/conclusion: </strong>This cross-sectional study concludes that KM55 is useful in the evaluation of IgA-containing glomerular diseases from a pathogenetic perspective and is a practical tool in resolving differential diagnosis in cases with overlapping histopathological features.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 2","pages":"59-74"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a8/d4/gdz-0002-0059.PMC9670030.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10674926","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}
Harish Seethapathy, Sayna Norouzi, Kate J Robson, Lida Gharibvand, Ali Poyan Mehr
{"title":"Glomerular Disease Education Experience across Nephrology Fellowship Programs: An International Survey.","authors":"Harish Seethapathy, Sayna Norouzi, Kate J Robson, Lida Gharibvand, Ali Poyan Mehr","doi":"10.1159/000521598","DOIUrl":"https://doi.org/10.1159/000521598","url":null,"abstract":"<p><strong>Introduction: </strong>Glomerulonephritis (GN) education is an important, albeit a challenging, component of nephrology fellowship training. We hypothesized that trainee experience varies widely across programs, leading to differences in self-reported competency levels in the diagnosis and management of glomerular diseases.</p><p><strong>Methods: </strong>The Glomerular Disease Study & Trial Consortium (GlomCon) conducted an anonymous online survey to determine the educational experience of nephrology trainees. We used multiple-choice questions to obtain data about (a) curriculum-based education, (b) dedicated specialty clinic, and (c) exposure to pathology. We leveraged a visual analog scale of 1-100 (with a higher number indicating a higher comfort level) to assess self-reported levels of clinical competency. The survey was disseminated via email to the subscribing members of GlomCon and through Twitter.</p><p><strong>Results: </strong>In total, there were 109 respondents to our survey, and 56% were from training programs in the USA. Exposure to a specialized GN clinic was reported by 45%, while 77% reported the presence of an onsite nephropathologist at their training program. Self-reported competency scores were 59 ± 25 and 52 ± 25 for diagnosis and treatment of glomerular diseases, respectively. Days spent in a GN clinic per year, years of fellowship, and dedicated nephropathology didactics were associated with higher diagnosis and treatment competency scores.</p><p><strong>Conclusion: </strong>Trainees report a wide variation in glomerular disease education across fellowship programs. A lack of nephropathology exposure and a dedicated GN curriculum was associated with lower scores in self-reported clinical competency in caring for patients with glomerular disease.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 2","pages":"89-94"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b1/53/gdz-0002-0089.PMC9670032.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10681231","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}
S. Brix, D. Kanigicherla, D. Wallace, Tejas B. Desai, R. Lennon
{"title":"The 13th International Podocyte Conference","authors":"S. Brix, D. Kanigicherla, D. Wallace, Tejas B. Desai, R. Lennon","doi":"10.1159/000525410","DOIUrl":"https://doi.org/10.1159/000525410","url":null,"abstract":"na","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"16 1","pages":"1 - 78"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88324293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Glomerular diseasesPub Date : 2022-01-24eCollection Date: 2022-07-01DOI: 10.1159/000522158
Cynthia Ciwei Lim, Irene Y J Mok, Jun Jie Leeu, Zhong Hong Liew, Hui Zhuan Tan, Yok Mooi Chin, Wei Ling Teng, Fiona Yeo, Chieh Suai Tan, Jason C J Choo
{"title":"A Descriptive Evaluation of Health Literacy and Determinants of COVID-19 Vaccine Acceptance among Patients with IgA Nephropathy with High Vaccine Uptake.","authors":"Cynthia Ciwei Lim, Irene Y J Mok, Jun Jie Leeu, Zhong Hong Liew, Hui Zhuan Tan, Yok Mooi Chin, Wei Ling Teng, Fiona Yeo, Chieh Suai Tan, Jason C J Choo","doi":"10.1159/000522158","DOIUrl":"10.1159/000522158","url":null,"abstract":"<p><strong>Aims: </strong>Shared decision-making regarding COVID-19 vaccination in IgA nephropathy involves the ability to handle health information regarding potential benefits and risk of flare, but few studies have evaluated health literacy in the context of vaccination. We aimed to evaluate the health literacy and COVID-19 vaccination uptake and acceptance in IgA nephropathy.</p><p><strong>Methods: </strong>Single-center cross-sectional study of 126 consecutive patients with IgA nephropathy. Health literacy was assessed using the HLS-EU-47 questionnaire. Determinants of vaccine acceptance such as contextual influences, individual and group influences, and vaccine-specific issues were adapted from the World Health Organization framework.</p><p><strong>Results: </strong>Forty-eight patients (38.1%) with IgAN nephropathy completed the survey between June and August 2021. The participants' median age was 40.5 (31.6, 52.8) years with median disease duration of 2.8 (1.3, 4.3) years. The median general health literacy index was 31.74 (29.88, 35.82) with significantly greater difficulty in the competency of appraising health information and in the domain of disease prevention (<i>p</i> < 0.001). Forty-five patients (93.8%) received at least one dose of COVID-19 vaccine between January and August 2021. Among the 3 unvaccinated patients, 2 intended to receive the vaccination while and 1 did not intend to get vaccinated. There was a high level of trust and belief that their government and healthcare providers had their best interests at heart and that the healthcare providers were honest about the vaccine's risk and benefits, although 31.2% did not understand how the vaccine works and 22.9% believed that there were other ways to prevent infection. Most thought there was adequate safety information, were confident in the system for tracking adverse events and had no issues with access to the vaccine.</p><p><strong>Conclusion: </strong>Participants with IgA nephropathy had high health literacy scores and low vaccine hesitancy. The determinants for vaccine acceptance can potentially guide efforts to optimize vaccination coverage.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 3","pages":"132-138"},"PeriodicalIF":0.0,"publicationDate":"2022-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/48/47/gdz-0002-0132.PMC9059031.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10724710","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":"IgA Nephropathy: A Chinese Perspective.","authors":"Zhao Zhang, Yuemiao Zhang, Hong Zhang","doi":"10.1159/000520039","DOIUrl":"https://doi.org/10.1159/000520039","url":null,"abstract":"<p><strong>Background: </strong>IgA nephropathy (IgAN) is the most common primary glomerular disease worldwide and remains a leading cause of chronic kidney disease and end-stage renal disease. The disease prevalence, clinical and pathological phenotypes, the underlying pathogenic molecular mechanisms, and the response to treatments are highly heterogeneous in different ethnic populations, which raise the concern that IgAN may differ across different parts of the world.</p><p><strong>Summary: </strong>From a Chinese perspective, we stated the disease burden of IgAN, summarized genome-wide association studies and research into pathological molecules, and compared them with findings based on other populations. The emerging biomarkers, indigenous clinical trials, and major challenges for Chinese researchers and nephrologists in studying IgAN are also discussed.</p><p><strong>Key messages: </strong>In this review, we described a higher risk of major susceptible loci in mucosal immunity, IgA production, and complement activation pathways in Chinese patients with IgAN. With our understanding of the pathogenesis of IgAN, novel biomarkers are emerging. Although there are challenges for conducting high-quality clinical trials in China, it is still feasible to conduct innovative and well-designed studies of IgAN. In the future, international collaborations on research infrastructure would be helpful to advance clinical and basic research in China.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 1","pages":"30-41"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/09/9a/gdz-0002-0030.PMC9677733.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10676271","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}
Catherine R Kavanagh, Francesca Zanoni, Rita Leal, Namrata G Jain, Megan Nicole Stack, Elena-Rodica Vasilescu, Geo Serban, Carley Shaut, Jeanne Kamal, Satoru Kudose, António Martinho, Rui Alves, Dominick Santoriello, Pietro A Canetta, David Cohen, Jai Radhakrishnan, Gerald B Appel, Michael B Stokes, Glen S Markowitz, Vivette D D'Agati, Krzysztof Kiryluk, Nicole K Andeen, Ibrahim Batal
{"title":"Clinical Predictors and Prognosis of Recurrent IgA Nephropathy in the Kidney Allograft.","authors":"Catherine R Kavanagh, Francesca Zanoni, Rita Leal, Namrata G Jain, Megan Nicole Stack, Elena-Rodica Vasilescu, Geo Serban, Carley Shaut, Jeanne Kamal, Satoru Kudose, António Martinho, Rui Alves, Dominick Santoriello, Pietro A Canetta, David Cohen, Jai Radhakrishnan, Gerald B Appel, Michael B Stokes, Glen S Markowitz, Vivette D D'Agati, Krzysztof Kiryluk, Nicole K Andeen, Ibrahim Batal","doi":"10.1159/000519834","DOIUrl":"https://doi.org/10.1159/000519834","url":null,"abstract":"<p><strong>Introduction: </strong>Although IgA nephropathy (IgAN) is the most common recurrent glomerulonephritis encountered in the kidney allograft, the clinical and immunogenetic characteristics remain poorly understood. We sought to study determinants and prognosis of recurrent IgAN with special focus on HLA antigens.</p><p><strong>Materials and methods: </strong>Between 2005 and 2019, we identified 282 transplanted patients with failure secondary to IgAN from two North American and one European Medical Centers, including 80 with recurrent IgAN and 202 without recurrence. Prevalence of HLA antigens was compared to external healthy controls of European ancestry (n=15,740). Graft survival was assessed by Kaplan-Meier method and log rank test. Cox proportional hazards were used for multivariable analyses.</p><p><strong>Results: </strong>Compared to external controls of European ancestry, kidney transplant recipients of European ancestry with kidney failure secondary to IgAN had higher frequency of HLA-DQ5 (42% vs. 30%, OR=1.68, P=0.002) and lower frequency of HLA-DR15 (15% vs. 28%, OR=0.46, P<0.001) and HLA-DQ6 (32% vs. 45%, OR=0.59, P=0.003); however, the frequency of these HLA antigens were similar in recurrent versus non-recurring IgAN. Younger recipient age at transplantation was an independent predictor of recurrence. HLA-matching was an independent predictor for recurrent IgAN only in recipients of living-related but not deceased or living unrelated transplants. Recurrent IgAN was an independent predictor of allograft failure, along with acute rejection. In patients with recurrent IgAN, serum creatinine at biopsy, degree of proteinuria, and concurrent acute rejection were associated with inferior allograft survival.</p><p><strong>Discussion/ conclusion: </strong>Recurrent IgAN negatively affects allograft survival. Younger recipient age at transplantation is an independent predictor of recurrent IgAN, while the presence of HLA antigens associated with IgAN in the native kidney and HLA-matching in recipients of deceased or living unrelated transplants are not.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 1","pages":"42-53"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/18/14/gdz-0002-0042.PMC9017582.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10807212","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}
Nicole K Andeen, Vanderlene L Kung, Josh Robertson, Susan B Gurley, Rupali S Avasare, Sneha Sitaraman
{"title":"Fibrillary Glomerulonephritis, DNAJB9, and the Unfolded Protein Response.","authors":"Nicole K Andeen, Vanderlene L Kung, Josh Robertson, Susan B Gurley, Rupali S Avasare, Sneha Sitaraman","doi":"10.1159/000525542","DOIUrl":"https://doi.org/10.1159/000525542","url":null,"abstract":"Background: Fibrillary glomerulonephritis (FGN) is found in approximately 1% of native kidney biopsies and was traditionally defined by glomerular deposition of fibrils larger than amyloid (12–24 nm diameter) composed of polyclonal IgG. Recent identification of DNAJB9 as a sensitive and specific marker of FGN has revolutionized FGN diagnosis and opened new avenues to studying FGN pathogenesis. In this review, we synthesize recent literature to provide an updated appraisal of the clinical and pathologic features of FGN, discuss diagnostic challenges and pitfalls, and propose molecular models of disease in light of DNAJB9. Summary: DNAJB9 tissue assays, paraffin immunofluorescence studies, and IgG subclass testing demonstrate that FGN is distinct from other glomerular diseases with organized deposits and highlight FGN morphologic variants. Additionally, these newer techniques show that FGN is only rarely monoclonal, and patients with monoclonal FGN usually do not have a monoclonal gammopathy. DNAJB9 mutation does not appear to affect the genetic architecture of FGN; however, the accumulation of DNAJB9 in FGN deposits suggests that disease is driven, at least in part, by proteins involved in the unfolded protein response. Treatments for FGN remain empiric, with some encouraging data suggesting that rituximab-based therapy is effective and that transplantation is a good option for patients progressing to ESKD. Key Messages: DNAJB9 aids in distinguishing FGN from other glomerular diseases with organized deposits. Further investigations into the role of DNAJB9 in FGN pathogenesis are necessary to better understand disease initiation and progression and to ultimately develop targeted therapies.","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 4","pages":"164-175"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a5/f4/gdz-0002-0164.PMC9936766.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10772190","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}
Tarak Srivastava, Katherine M Dell, Kevin V Lemley, Debbie S Gipson, Frederick J Kaskel, Kevin Edward Meyers, Christian Faul, Ayelet Goldhaber, LauraJane Pehrson, Howard Trachtman
{"title":"Gluten-Free Diet in Childhood Difficult-to-Treat Nephrotic Syndrome: A Pilot Feasibility Study.","authors":"Tarak Srivastava, Katherine M Dell, Kevin V Lemley, Debbie S Gipson, Frederick J Kaskel, Kevin Edward Meyers, Christian Faul, Ayelet Goldhaber, LauraJane Pehrson, Howard Trachtman","doi":"10.1159/000525587","DOIUrl":"https://doi.org/10.1159/000525587","url":null,"abstract":"<p><strong>Introduction: </strong>Minimal change disease in childhood can follow a frequently relapsing or steroid-dependent course in up to 40% of cases. Second-line immunosuppressive medications that are used to manage these patients are associated with significant adverse effects. There is a need for safer alternative treatments for difficult-to-treat nephrotic syndrome. Therefore, we conducted an open-label feasibility study to assess the safety and efficacy of a gluten-free diet as treatment for pediatric patients with difficult-to-treat nephrotic syndrome. As a second aim, we sought to determine if the plasma zonulin concentration can identify those who are more likely to respond to this intervention.</p><p><strong>Methods: </strong>Seventeen patients were placed on a gluten-free diet for 6 months. A positive response was defined as a 50% reduction in the relapse rate compared to the preceding 6 months or the ability to discontinue 1 immunosuppressive drug.</p><p><strong>Results: </strong>Five (29%) participants had a positive response to the dietary intervention. The gluten-free diet was well tolerated with no clinical or laboratory adverse events. Plasma zonulin concentration was elevated in patients who failed to benefit from the gluten-free diet.</p><p><strong>Discussion/conclusion: </strong>A gluten-free diet may be a useful adjunctive intervention for patients with difficult-to-treat nephrotic syndrome that can be implemented prior to resorting to second-line immunosuppressive therapy. Development of the plasma zonulin level as a biomarker to predict efficacy would facilitate rational use of a gluten-free diet in the management of nephrotic syndrome.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 4","pages":"176-183"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a5/dd/gdz-0002-0176.PMC9936750.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9073959","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":"IgA Nephropathy: \"The Times They Are a-Changin\".","authors":"Richard J Glassock","doi":"10.1159/000515199","DOIUrl":"https://doi.org/10.1159/000515199","url":null,"abstract":"<p><strong>Background: </strong>Primary IgA Nephropathy (IgA N) is a very common and often progressive glomerular disease. At present, the diagnosis of IgA N is totally dependent on kidney biopsy, but the prospect for a future diagnosis by means of a \"liquid\" biopsy is promising. A great deal is now understood regarding its diverse clinical and pathological features as well as its epidemiology, genetics, prognosis, and pathogenesis. Treatment approaches are now on increasingly solid evidence-based grounds, but many uncertainties continue to be devil the field. Better means of categorization of patients into a hierarchy of progression risk at the time of diagnosis will undoubtedly refine and personalize treatment decisions.</p><p><strong>Summary: </strong>The panorama of treatment strategies is undergoing a rapid transformation, largely due to an increase in large randomized clinical trials testing available agents and novel therapeutic classes. It is anticipated that the combination of better prognostic tools and new strategies for treatment of IgA N will alter the landscape of therapeutic algorithms for patients with IgA N.</p><p><strong>Key messages: </strong>This review seeks to describe some of the evolutionary changes in the approach to treatment of IgA N, to place them in the context of current management, and to identify knowledge gaps that need to be addressed.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 1","pages":"4-14"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000515199","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9228657","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}