Glomerular Disease and Cystic Kidney Disease: From Pathogenesis to Novel Therapeutic Approaches—2nd Edition

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Molecular and Translational Medicine".

Deadline for manuscript submissions: 31 December 2025 | Viewed by 1262

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Guest Editor
Department of Nephrology and Kidney Transplantation, School of Medicine, National and Kapodistrian University of Athens, General Hospital of Athens “Laiko”, 11527 Athens, Greece
Interests: polycystic kidney disease; glomerular disease; kidney transplantation; immunology
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Guest Editor
Department of Nephrology and Kidney Transplantation, School of Medicine, National and Kapodistrian University of Athens, General Hospital of Athens “Laiko”, 11527 Athens, Greece
Interests: Glomerular disease, kidney transplantation; acute kidney injury
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

This Special Issue aims to gather publications related to both primary and secondary glomerular disease, as well as cystic kidney disease, especially polycystic kidney disease, which is the most common hereditary kidney disease. Glomerular disease describes a variety of relatively rare immune-mediated diseases characterized by damage to the glomerular compartment of the kidney. If not properly treated, glomerular disease can lead to chronic kidney disease and irreversible kidney failure. On the other hand, cystic kidney disease represents multisystemic disorders that can develop due to genetic or non-genetic causes in children and adults and may have serious renal and extrarenal complications. Both of these entities have shown notable progress in their complex pathophysiology, and various potential therapeutic targets are available for which novel treatments are being developed or repurposed. The growing awareness of the immunopathogenesis of glomerular diseases and the improvements in immune phenotyping for the classification of patients have facilitated the choice of targeted immunotherapies. Regarding polycystic kidney disease, tolvaptan, a selective vasopressin V2 antagonist, has been proven to attenuate disease progression, and several other disease-modifying therapies are currently being investigated in clinical trials. Furthermore, extended genetic testing, with next-generation sequencing, is another evolving diagnostic tool to ultimately define a molecular diagnosis and appears extremely useful in both of these entities.

This Special Issue welcomes original articles and reviews focused on the pathogenesis and treatment of glomerular diseases and cystic kidney diseases.

Dr. Vassilis Filiopoulos
Dr. Chrysanthi Skalioti
Guest Editors

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Keywords

  • glomerular disease
  • glomerulonephritis
  • glomerulopathy
  • podocytopathies
  • ciliopathies
  • cystic kidney disease
  • polycystic kidney disease
  • tolvaptan
  • vasopressin

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24 pages, 1045 KB  
Review
Anti-B Cell Strategy in Nephrotic Syndrome: Beyond Rituximab
by Yanyan Jin, Yi Xie, Haidong Fu, Fei Liu and Jianhua Mao
Biomedicines 2025, 13(9), 2063; https://doi.org/10.3390/biomedicines13092063 - 24 Aug 2025
Viewed by 1110
Abstract
Nephrotic syndrome (NS) is a complex kidney disorder characterized by profound proteinuria, hypoalbuminemia, hyperlipidemia, and edema, significantly impacting patients’ quality of life. While corticosteroids and calcineurin inhibitors (CNIs) have traditionally been the primary treatments, B cell-targeted therapies, especially the anti-CD20 monoclonal antibody rituximab, [...] Read more.
Nephrotic syndrome (NS) is a complex kidney disorder characterized by profound proteinuria, hypoalbuminemia, hyperlipidemia, and edema, significantly impacting patients’ quality of life. While corticosteroids and calcineurin inhibitors (CNIs) have traditionally been the primary treatments, B cell-targeted therapies, especially the anti-CD20 monoclonal antibody rituximab, have transformed the management of steroid-dependent and multidrug-resistant NS (MRNS). Rituximab has demonstrated efficacy in reducing relapse rates and steroid dependence by depleting CD20+ B cells, which play a pivotal role in autoantibody production and immune dysregulation. However, limitations such as incomplete B cell depletion, immunogenicity leading to anti-rituximab antibodies, and variable efficacy in refractory cases have led to the development of next-generation therapies. This review critically examines recent advances in B cell-targeted therapies for NS, with a particular focus on overcoming the limitations of conventional rituximab treatment. This review systematically analyzes next-generation anti-CD20 monoclonal antibodies, CD38-targeted therapies, and emerging CAR-T cell approaches, evaluating their distinct mechanisms of action and clinical trial outcomes. The analysis extends to innovative combination strategies and biomarker-guided treatment algorithms for refractory cases. By synthesizing preclinical data with clinical evidence, this work provides a framework for optimizing therapeutic decision-making in NS, while identifying key knowledge gaps that warrant future investigation. Collaborative research and translational studies are essential for advancing precision medicine in NS, ensuring that new therapies provide lasting clinical benefits for patients. The evolving field of anti-B cell therapies marks a new era in managing refractory NS, offering hope for better long-term prognoses. Full article
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