Treatment of Refractory Glomerular Diseases: Challenges and Solutions

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Urology & Nephrology".

Deadline for manuscript submissions: closed (20 November 2023) | Viewed by 9238

Special Issue Editor


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Guest Editor
Department of Nephrology, Tokyo Medical University, Inashiki, Ibaraki, Japan
Interests: glomerulonephritis; vasculitis; immunology

Special Issue Information

Dear Colleagues,

Glomerular diseases other than diabetic nephropathy account for approximately 25% of chronic kidney disease (CKD) patients worldwide. Given the long duration of glomerular disease and the complications and prognosis associated with underlying disease and treatment, it is critical to optimize management to control and prevent progressive kidney disease. Recently, the KDIGO 2021 clinical practice guidelines for the management of glomerular disease were published and are expected to improve the prognosis and complications of glomerular disease. However, the treatment of glomerular disease still primarily consists of corticosteroids with or without several immunosuppressants, and there are only a few established treatments for the molecules involved in onset and progression, such as biological agents.

This issue focuses on primary glomerular diseases (idiopathic nephrotic syndrome, membranous nephropathy, IgA nephropathy, etc.), secondary glomerular diseases other than diabetic nephropathy (IgA vasculitis, lupus nephritis, ANCA-associated vasculitis, etc.) and hereditary glomerular disorders (Alport syndrome, Fabry disease, etc.). This Special Issue also aims to accumulate knowledge about new treatments for these refractory glomerular diseases.

In this Special Issue, original research articles and reviews are both welcome. Research topics may include (but are not limited to) the following:

  1. Outcomes of and problems with gold-standard treatments;
  2. Potential novel therapeutic agents in animal models;
  3. Introducing new treatment options in case series or pilot studies;
  4. The discovery of new therapeutic biomarkers.

I look forward to receiving your contributions.

Dr. Kouichi Hirayama
Guest Editor

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Keywords

  • idiopathic nephrotic syndrome
  • focal and segmental glomerulosclerosis
  • membranous nephropathy
  • membranoproliferative glomerulonephritis/C3 nephropathy
  • IgA nephropathy/vasculitis
  • lupus nephritis
  • ANCA-associated vasculitis
  • anti-GBM disease
  • hereditary glomerular diseases

Published Papers (4 papers)

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Research

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14 pages, 604 KiB  
Article
Refractory IgA Nephropathy: A Challenge for Future Nephrologists
by Vincenzo Di Leo, Francesca Annese, Federica Papadia, Maria Serena Russo, Marica Giliberti, Fabio Sallustio and Loreto Gesualdo
Medicina 2024, 60(2), 274; https://doi.org/10.3390/medicina60020274 - 5 Feb 2024
Viewed by 2195
Abstract
IgA nephropathy (IgAN) represents the most prevalent form of primary glomerulonephritis, and, on a global scale, it ranks among the leading culprits behind end-stage kidney disease (ESKD). Presently, the primary strategy for managing IgAN revolves around optimizing blood pressure and mitigating proteinuria. This [...] Read more.
IgA nephropathy (IgAN) represents the most prevalent form of primary glomerulonephritis, and, on a global scale, it ranks among the leading culprits behind end-stage kidney disease (ESKD). Presently, the primary strategy for managing IgAN revolves around optimizing blood pressure and mitigating proteinuria. This is achieved through the utilization of renin–angiotensin system (RAS) inhibitors, namely, angiotensin-converting enzyme inhibitors (ACEi) and angiotensin receptor blockers (ARBs). As outlined by the KDIGO guidelines, individuals who continue to show a persistent high risk of progressive ESKD, even with comprehensive supportive care, are candidates for glucocorticoid therapy. Despite these therapies, some patients have a disease refractory to treatment, defined as individuals that present a 24 h urinary protein persistently >1 g after at least two rounds of regular steroids (methylprednisolone or prednisone) and/or immunosuppressant therapy (e.g., mycophenolate mofetil), or who do not tolerate regular steroids and/or immunosuppressant therapy. The aim of this Systematic Review is to revise the current literature, using the biomedical database PubMed, to investigate possible therapeutic strategies, including SGLT2 inhibitors, endothelin receptor blockers, targeted-release budesonide, B cell proliferation and differentiation inhibitors, fecal microbiota transplantation, as well as blockade of complement components. Full article
(This article belongs to the Special Issue Treatment of Refractory Glomerular Diseases: Challenges and Solutions)
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Review

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22 pages, 819 KiB  
Review
IgA Nephropathy: Beyond the Half-Century
by Yoshio Shimizu, Yasuhiko Tomino and Yusuke Suzuki
Medicina 2024, 60(1), 54; https://doi.org/10.3390/medicina60010054 - 27 Dec 2023
Viewed by 1876
Abstract
In 1968, Jean Berger first introduced the medical world to IgA nephropathy (IgAN). Fifty-five years later, its pathogenesis is still unclear, but treatments such as renin–angiotensin–aldosterone system inhibitors (RAAS-Is), tonsillectomies, and glucocorticoids are currently used worldwide. There have been great strides in the [...] Read more.
In 1968, Jean Berger first introduced the medical world to IgA nephropathy (IgAN). Fifty-five years later, its pathogenesis is still unclear, but treatments such as renin–angiotensin–aldosterone system inhibitors (RAAS-Is), tonsillectomies, and glucocorticoids are currently used worldwide. There have been great strides in the past 20 years since the discoveries of the specific dysregulation of mucosal immunity, galactose-deficient IgA1 (Gd-IgA1), and Gd-IgA1 immune complexes in patients with IgAN. According to these findings, a multi-hit hypothesis was developed, and this multi-hit hypothesis has provided several putative therapeutic targets. A number of novel agents, including molecularly targeted drugs for targets such as APRIL, plasma cells, complement systems, and endothelin, are undergoing clinical trials. Some candidate drugs have been found to be effective, with minimal side effects. Over half a century after the discovery of IgAN, these therapies will soon be available for clinical use. Full article
(This article belongs to the Special Issue Treatment of Refractory Glomerular Diseases: Challenges and Solutions)
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12 pages, 323 KiB  
Review
Treatment of Fabry Nephropathy: A Literature Review
by Homare Shimohata, Marina Yamashita, Kota Yamada, Kouichi Hirayama and Masaki Kobayashi
Medicina 2023, 59(8), 1478; https://doi.org/10.3390/medicina59081478 - 17 Aug 2023
Cited by 4 | Viewed by 1854
Abstract
Fabry disease is an X-linked inherited lysosomal storage disorder with a deficiency of α-galactosidase A activity, which results in the intracellular accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in various organs. Fabry nephropathy is one of the major complications of Fabry disease, and [...] Read more.
Fabry disease is an X-linked inherited lysosomal storage disorder with a deficiency of α-galactosidase A activity, which results in the intracellular accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in various organs. Fabry nephropathy is one of the major complications of Fabry disease, and kidney damage is often related to cardiovascular disease and mortality. The treatment of Fabry nephropathy thus helps prolong life expectancy. Two treatment options for Fabry nephropathy and cardiopathy are now commercially available: enzyme replacement therapy (agalsidase α agalsidase β, and a biosimilar of agalsidase β) and pharmacological chaperone therapy (migalastat). In this review, we summarize the efficacy of these treatment options for Fabry nephropathy with respect to renal function, proteinuria, and renal pathological findings. We also describe the importance of adjunctive therapy for Fabry nephropathy. Full article
(This article belongs to the Special Issue Treatment of Refractory Glomerular Diseases: Challenges and Solutions)
17 pages, 371 KiB  
Review
Immunosuppressive Agent Options for Primary Nephrotic Syndrome: A Review of Network Meta-Analyses and Cost-Effectiveness Analysis
by Kei Nagai
Medicina 2023, 59(3), 601; https://doi.org/10.3390/medicina59030601 - 17 Mar 2023
Cited by 2 | Viewed by 2619
Abstract
Therapeutic options with immunosuppressive agents for glomerular diseases have widened with refinements to the Kidney Disease Improving Global Outcomes (KDIGO) guidelines from 2012 to 2021. However, international guidelines do not necessarily match the reality in each country. Expensive therapies such as rituximab and [...] Read more.
Therapeutic options with immunosuppressive agents for glomerular diseases have widened with refinements to the Kidney Disease Improving Global Outcomes (KDIGO) guidelines from 2012 to 2021. However, international guidelines do not necessarily match the reality in each country. Expensive therapies such as rituximab and calcineurin inhibitors are sometimes inaccessible to patients with refractory nephrotic syndrome due to cost or regulations. Under the Japanese medical insurance system, rituximab is accessible but still limited to steroid-dependent patients who developed idiopathic nephrotic syndrome in childhood. Based on international KDIGO guidelines and other national guidelines, possible applications of immunosuppressive agents for nephrotic syndrome are comprehensively examined in this review. While rituximab has become the mainstay of immunosuppressive therapy for nephrotic syndrome, clinical trials have indicated that options such as cyclophosphamide, calcineurin inhibitors, and mycophenolate mofetil would be preferable. Given the rising number of patients with nephrotic syndrome worldwide, KDIGO guidelines mention the need for further consideration of cost-effectiveness. If the new option of rituximab is to be the first choice in combination with steroids for nephrotic syndrome, its cost-effectiveness should also be verified. Among the few studies examining the cost-effectiveness of treatments for nephrotic syndrome, administration of rituximab to young adults has been shown to be cost-beneficial, at least in Japan. However, further large-scale studies involving multiple facilities are needed to verify such findings. Network meta-analyses have concluded that the efficacy of rituximab remains controversial and confirmation through high-quality studies of large cohorts is needed. To this end, the mechanisms of action underlying immunosuppressive agents, both old and new, need to be understood and experience must be accumulated to evaluate possible effects and side effects. Full article
(This article belongs to the Special Issue Treatment of Refractory Glomerular Diseases: Challenges and Solutions)
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