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Medicina
Special Issues
Treatment of Refractory Glomerular Diseases: Challenges and Solutions
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Special Issue "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 3523

Special Issue Editor

Dr. Kouichi Hirayama

E-Mail Website
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

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Medicina is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 1800 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

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 (3 papers)

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Review

18 pages, 868 KiB  
Review
The Immunobiological Agents for Treatment of Antiglomerular Basement Membrane Disease
by
Marina Yamashita
,
Mamiko Takayasu
,
Hiroshi Maruyama
and
Kouichi Hirayama
Medicina 2023, 59(11), 2014; https://doi.org/10.3390/medicina59112014 - 16 Nov 2023
Viewed by 554
Abstract
Combination therapy with glucocorticoids, cyclophosphamide, and plasmapheresis is recommended as the standard treatment for anti-glomerular basement membrane (anti-GBM) disease, but the prognosis of this disease remains poor. Several immunobiological agents have been administered or are expected to be useful for anti-GBM disease in [...] Read more.
Combination therapy with glucocorticoids, cyclophosphamide, and plasmapheresis is recommended as the standard treatment for anti-glomerular basement membrane (anti-GBM) disease, but the prognosis of this disease remains poor. Several immunobiological agents have been administered or are expected to be useful for anti-GBM disease in light of refractory disease or the standard treatments’ tolerability. Many data regarding the use of biologic agents for anti-GBM disease have accumulated, verifying the effectiveness and potential of biologic agents as a new treatment option for anti-GBM disease. Tumor necrosis factor (TNF) inhibitors were shown to be useful in animal studies, but these agents have no clinical use and were even shown to induce anti-GBM disease in several cases. Although the efficacy of the TNF-receptor antagonist has been observed in animal models, there are no published case reports of its clinical use. There are also no published reports of animal or clinical studies of anti-B-cell-activating factor, which is a member of the TNF family of agents. Anti-interleukin (IL)-6 antibodies have been demonstrated to have no effect on or to exacerbate nephritis in animal models. Anti-C5 inhibitor was observed to be useful in a few anti-GBM disease cases. Among the several immunobiological agents, only rituximab has been demonstrated to be useful in refractory or poor-tolerance patients or small uncontrolled studies. Rituximab is usually used in combination with steroids and plasma exchange and is used primarily as an alternative to cyclophosphamide, but there is insufficient evidence regarding the efficacy of rituximab for anti-GBM disease, and thus, randomized controlled studies are required. 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 1 | Viewed by 912
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 1 | Viewed by 1579
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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