Kirk N. Campbell, Keisha L. Gibson, Kenar D. Jhaveri
{"title":"Nephritic Syndromes: A Lot to Progress","authors":"Kirk N. Campbell, Keisha L. Gibson, Kenar D. Jhaveri","doi":"10.1053/j.akdh.2024.05.003","DOIUrl":"https://doi.org/10.1053/j.akdh.2024.05.003","url":null,"abstract":"","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 3","pages":"Pages 167-169"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141606385","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}
{"title":"A Comprehensive and Practical Approach to the Management of Lupus Nephritis in the Current Era","authors":"Nasim Wiegley , Swati Arora , Sayna Norouzi , Brad Rovin","doi":"10.1053/j.akdh.2023.11.003","DOIUrl":"https://doi.org/10.1053/j.akdh.2023.11.003","url":null,"abstract":"<div><p>Lupus nephritis (LN) is a severe complication of systemic lupus erythematosus (SLE) and is one of the leading causes of morbidity and mortality in patients with SLE. It is estimated that up to 60% of individuals with SLE will develop LN, which can manifest at any stage of a patient's life; however, it commonly emerges early in the course of SLE and tends to exhibit a more aggressive phenotype in men compared to women. Black and Hispanic patients are more likely to progress to kidney failure than white patients. LN is characterized by kidney inflammation and chronic parenchymal damage, leading to impaired kidney function and potential progression to kidney failure. This article provides a comprehensive overview of the epidemiology, pathogenesis, clinical presentation, diagnosis, and management of LN, highlighting the importance of early recognition and treatment of LN to prevent progressive, irreversible kidney damage and improve patient outcomes. Additionally, the article discusses current and emerging therapies for LN, including traditional immunosuppressive agents, biological agents, and novel therapies targeting specific pathways involved in LN pathogenesis, to provide a practical guide for clinicians in properly diagnosing LN and determining a patient-centered treatment plan.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 3","pages":"Pages 234-245"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141606389","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}
Haresh Selvaskandan , Kenar D. Jhaveri , Dana V. Rizk
{"title":"Primary IgA Nephropathy: New Insights and Emerging Therapies","authors":"Haresh Selvaskandan , Kenar D. Jhaveri , Dana V. Rizk","doi":"10.1053/j.akdh.2024.04.002","DOIUrl":"https://doi.org/10.1053/j.akdh.2024.04.002","url":null,"abstract":"<div><p>Primary IgA nephropathy (IgAN) is a common glomerular disorder defined by predominant mesangial IgA deposition. Once thought to follow a progressive course in 10-20% of those diagnosed, emerging evidence now suggests most will progress to kidney failure over their lifetimes. Although the lack of safe and effective treatments to impede disease progression continues to present a challenge, the landscape of IgAN has dramatically evolved over the last 2 years. Driven by fundamental changes to accepted end points for IgAN clinical trials as well as fascinating new insights into the pathophysiology of IgAN, a swathe of novel and repurposed therapies are currently being evaluated. Already, two novel drugs, targeted-release formulation budesonide and sparsentan, have received conditional approvals for the treatment of IgAN, with sodium glucose co-transporter 2 inhibitors establishing themselves as further options. Soon to join this ensemble are likely to be treatments that modulate the complement system and B-cell activity; several are currently undergoing clinical trials in IgAN with promising interim results. In this review, we provide an overview of evolving epidemiological insights, disease mechanisms, emerging therapies, and contemporary challenges surrounding the management of IgAN.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 3","pages":"Pages 180-193"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141606386","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}
{"title":"Thrombotic Microangiopathies and the Kidney","authors":"Anuja Java , Richard Burwick , Anthony Chang","doi":"10.1053/j.akdh.2023.09.003","DOIUrl":"https://doi.org/10.1053/j.akdh.2023.09.003","url":null,"abstract":"<div><p>Thrombotic microangiopathy (TMA) is a pathological lesion that occurs due to endothelial injury. It can be seen in a heterogenous group of disorders, typically characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemia. TMA can also be renal limited with no systemic manifestations. There are multiple etiologies of a TMA with complement activation being a core underlying mechanism, although the nature and extent of complement involvement can vary. A further complicated factor is the cross talk between complement, neutrophils, and coagulation pathways in the pathophysiology of TMAs. Therefore, a thorough and systematic clinical history and laboratory evaluation are critical to establish the cause and pathophysiology of a TMA. Furthermore, TMAs are associated with significant morbidity and mortality, and timely diagnosis is key for appropriate management and to prevent end-stage kidney disease and other associated complications. In this review, we focus on the pathology, mechanisms, diagnostic work up and treatment of TMAs associated with various etiologies. We also define the complement evaluations that should be conducted in these patients and further highlight the currently approved complement therapies as well as others in the pipeline.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 3","pages":"Pages 255-264"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141606387","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}
{"title":"Infection-Associated Glomerulonephritis","authors":"Ryan W. Bonner , Vanessa Moreno , Koyal Jain","doi":"10.1053/j.akdh.2024.01.001","DOIUrl":"https://doi.org/10.1053/j.akdh.2024.01.001","url":null,"abstract":"<div><p>The nephritic syndrome has been associated with a wide variety of infections, spanning many organisms and myriad clinical presentations. Infection-associated glomerulonephritis is challenging to diagnose given the many confounding factors linking kidney injury to infection; however, urine microscopy can assist in identifying abnormal cellular elements suggestive of glomerulonephritis. Kidney biopsy remains the gold standard for diagnosing the underlying pathologic lesion. Treatment of infection-associated glomerulonephritis centers around aggressive and complete treatment of the underlying infectious driver. It is often hard to know exactly when immunosuppression may be required in addition to treating the infection.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 3","pages":"Pages 246-254"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141606388","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}
{"title":"Alport Syndrome","authors":"Efren Chavez , Stefania Goncalves , Michelle N. Rheault , Alessia Fornoni","doi":"10.1053/j.akdh.2024.02.004","DOIUrl":"https://doi.org/10.1053/j.akdh.2024.02.004","url":null,"abstract":"<div><p>Alport syndrome (AS) is characterized by progressive kidney failure, hematuria, sensorineural hearing loss, and ocular abnormalities. Pathogenic variants in the <em>COL4A3-5</em> genes result in a defective deposition of the collagen IV α3α4α5 protomers in the basement membranes of the glomerulus in the kidney, the cochlea in the ear and the cornea, lens capsule and retina in the eye. The presence of a large variety of <em>COL4A3-5</em> gene(s) pathogenetic variants irrespective of the mode of inheritance (X-linked, autosomal recessive, autosomal dominant, or digenic) with and without syndromic features is better defined as the “Alport spectrum disorder”, and represents the most common cause of genetic kidney disease and the second most common cause of genetic kidney failure. The clinical course and prognosis of individuals with AS is highly variable. It is influenced by gender, mode of inheritance, affected gene(s), type of genetic mutation, and genetic modifiers. This review article will discuss the epidemiology, classification, pathogenesis, diagnosis, clinical course with genotype-phenotype correlations, and current and upcoming treatment of patients with AS. It will also review current recommendations with respect to when to evaluate for hearing loss or ophthalmologic abnormalities.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 3","pages":"Pages 170-179"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141606390","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}
Joyita Bharati , Kenar D. Jhaveri , Alan D. Salama , Louise Oni
{"title":"Anti–Glomerular Basement Membrane Disease: Recent Updates","authors":"Joyita Bharati , Kenar D. Jhaveri , Alan D. Salama , Louise Oni","doi":"10.1053/j.akdh.2024.04.007","DOIUrl":"https://doi.org/10.1053/j.akdh.2024.04.007","url":null,"abstract":"<div><p>Anti–glomerular basement membrane disease is a small-vessel vasculitis involving the kidneys (∼90%) and the lungs (∼60%). Antibodies against the glomerular basement membrane are directly pathogenic in anti–glomerular basement membrane disease; however, recent research has highlighted the critical role of T cells. Novel autoantigens within the glomerular basement membrane are also now recognized. Atypical forms of the disease are reported along with preceding triggers, such as immune checkpoint inhibitors, immunomodulatory drugs, and vaccines. Kidney outcomes in anti–glomerular basement membrane disease remain poor despite significant improvement in patient survival in the last 2 to 3 decades. Treatment typically relies on combined plasmapheresis with intensive immunosuppression. Dialysis dependency at presentation is a dominant predictor of kidney outcome. Histologically, a low (<10%) percentage of normal glomeruli, 100% crescents, together with dialysis dependency at presentation, is associated with poor kidney outcomes. In such cases, an individualized approach weighing the risks and benefits of treatment is recommended. There is a need for better ways to stop the toxic inflammatory activity associated with this disease. In this narrative review, we discuss recent updates on the pathogenesis and management of anti–glomerular basement membrane disease relevant to patients of all ages.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 3","pages":"Pages 206-215"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2949813924000818/pdfft?md5=6f64d91a0bc87d5651d2bfe7fa303c6f&pid=1-s2.0-S2949813924000818-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141605628","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}
Benjamin Wooden , Carla M. Nester , Andrew S. Bomback
{"title":"Update on C3 Glomerulopathy","authors":"Benjamin Wooden , Carla M. Nester , Andrew S. Bomback","doi":"10.1053/j.akdh.2024.05.002","DOIUrl":"https://doi.org/10.1053/j.akdh.2024.05.002","url":null,"abstract":"<div><p>C3 glomerulopathy (C3G) is a rare disorder marked by deposition of C3 in the glomerulus, resulting in damage to the glomerular filtration unit and presenting with features of the nephritic and nephrotic syndromes. Fundamentally, C3G is caused by dysregulation of the alternative pathway of the complement cascade, either due to genetic variants or acquired humoral factors. Despite significant advances in recent years in the understanding of the underlying mechanisms and culprit lesions that result in the development of C3G, treatment options remain severely limited, and the prognosis is often poor. Fortunately, a number of anticomplement therapies are emerging from the drug development pipeline, with several in late-stage testing in patients with C3G, and there is hope that we will soon have more targeted options for managing patients with this devastating disease. In this review, we provide an overview of C3G, as well as summarizing the evidence for current treatments and detailing the clinical trials that are currently underway.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 3","pages":"Pages 223-233"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141606381","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}
{"title":"ANCA-Associated Vasculitis","authors":"Purva Sharma , Reza Zonozi , Duvuru Geetha","doi":"10.1053/j.akdh.2024.04.005","DOIUrl":"https://doi.org/10.1053/j.akdh.2024.04.005","url":null,"abstract":"<div><p>ANCA-associated vasculitis (AAV) is a necrotizing, small-to-medium vessel vasculitis associated with significant morbidity and mortality. AAV is a systemic autoimmune disease affecting kidneys, eyes, sinuses, peripheral nerves, skin, and upper and lower respiratory tracts. AAV tends to present in characteristic phenotypes categorized clinically as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA). Kidney involvement is a common feature of AAV, and has important implications on disease prognosis and management. Existing therapies have been refined and improvements in our understanding of the pathophysiology of AAV has led to approval of novel therapies. In this review, we provide an overview of epidemiology, disease mechanisms, clinical presentation and review therapeutic strategies for induction and maintenance of remission.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 3","pages":"Pages 194-205"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141605629","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}
Samuel Mon-Wei Yu , Margaret Deoliveira , Miriam Chung , Richard Lafayette
{"title":"Membranoproliferative Glomerulonephritis Pattern of Injury","authors":"Samuel Mon-Wei Yu , Margaret Deoliveira , Miriam Chung , Richard Lafayette","doi":"10.1053/j.akdh.2024.03.005","DOIUrl":"https://doi.org/10.1053/j.akdh.2024.03.005","url":null,"abstract":"<div><p>Membranoproliferative glomerulonephritis (MPGN) is no longer a disease but a pattern of injury in various diseases. Characterized by electron-dense deposits, mesangial proliferation, and duplication of the glomerular basement membrane, MPGN was previously classified by findings seen by electron microscopy. However, recognizing complement dysfunction in relation to cases with the MPGN pattern of injury substantially changed our view of its pathogenesis. A new classification, including immune complex-mediated and complement-mediated MPGN, has become preferable and has been adopted by international guidelines. Despite these advancements, accurate diagnosis of MPGN remains a clinical challenge, given the pathological and clinical similarities between immune complex-mediated and complement-mediated MPGN. Additional testing, such as molecular and genetic testing, is often necessary. Here, we will summarize our current understanding of the MPGN pattern of injury from a pathology perspective as an introductory article in the following chapters.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":"31 3","pages":"Pages 216-222"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141606380","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}
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