Glomerular Diseases: Advances in Diagnosis and Management

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Nephrology & Urology".

Deadline for manuscript submissions: 15 May 2024 | Viewed by 2546

Special Issue Editors


E-Mail
Guest Editor
Nephrology, Dialysis and Transplantation Unit, Careggi University Hospital, 50139 Florence, Italy
Interests: thrombotic microangiopathy; complement; membranous nephropathy; podocytopathies; rituximab; amyloidosis; MGRS

E-Mail
Guest Editor
Department of Nephrology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
Interests: vasculitis; B-cell therapy; ANCA; IgG4-related disease; IgA vasculitis

Special Issue Information

Dear Colleagues,

The field of glomerular diseases is evolving at an unprecedented rate. A number of immunomodulating drugs have recently been introduced into routine clinical practice (e.g., Belimumab and Voclosporin in lupus nephritis, Avacopan in ANCA-associated vasculitis, Daratumumab in AL amyloidosis), while others are likely to expand our therapeutic array (e.g., complement-targeting therapies, humanized anti-CD20 monoclonal antibodies, Imlifidase). We are more aware of the advantages and shortcomings of the “standard” agents, such as cyclophosphamide and rituximab, and are implementing conservative therapy (e.g., sodium-glucose co-transporter 2 inhibitors). However, progress and challenges go far beyond novel treatments. The search for biomarkers that allow subclassifying heterogeneous entities, such as focal segmental glomerulosclerosis and IgA nephropathy, is ongoing, as is research on genetics of podocytopathies and atypical hemolytic uremic syndrome. The epidemiology of glomerular diseases is also changing, as monoclonal gammopathy of renal significance and rarer diseases are better characterized, while cases of new-onset immune-mediated disorders, post-immune checkpoint inhibitors and COVID-19 vaccines have posed fundamental questions regarding disease drivers. In this Special Issue of the Journal of Clinical Medicine, we will focus on recent advances in the study of glomerular diseases and welcome authors to submit their contribution to this rapidly transforming topic.

Dr. Marco Allinovi
Dr. Giorgio Trivioli
Guest Editors

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. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly 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 2600 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

  • glomerulonephritis
  • rituximab
  • lupus
  • ANCA
  • membranous nephropathy
  • FSGS
  • IgA nephropathy
  • amyloidosis
  • complement

Published Papers (4 papers)

Order results
Result details
Select all
Export citation of selected articles as:

Research

Jump to: Review

10 pages, 494 KiB  
Article
C3 Hypocomplementemia Predicts the Progression of CKD towards End-Stage Kidney Disease in IgA Nephropathy, Irrespective of Histological Evidence of Thrombotic Microangiopathy
by Giovanni Maria Rossi, Federico Ricco, Isabella Pisani, Marco Delsante, Umberto Maggiore, Enrico Fiaccadori and Lucio Manenti
J. Clin. Med. 2024, 13(9), 2594; https://doi.org/10.3390/jcm13092594 - 28 Apr 2024
Viewed by 219
Abstract
Background: IgA nephropathy (IgAN) is the most common primary glomerulonephritis worldwide. IgAN causes end-stage kidney disease (ESKD) in 30–40% of all cases. The activation of the complement system by pathological circulating IgAs, which is often associated with low serum C3 levels (LowC3), seems [...] Read more.
Background: IgA nephropathy (IgAN) is the most common primary glomerulonephritis worldwide. IgAN causes end-stage kidney disease (ESKD) in 30–40% of all cases. The activation of the complement system by pathological circulating IgAs, which is often associated with low serum C3 levels (LowC3), seems to play a crucial role. Previous studies have shown an association between histological evidence of TMA, which is the result of alternative complement activation, and poor outcomes. However, it is not known to what extent the decrease in serum C3 levels reflects ongoing TMA injury. Our study aimed at assessing the association between LowC3 and ESKD and whether this association reflects ongoing TMA. Methods: We enrolled all patients with biopsy-proven IgAN and followed-up patients until their last visit, ESKD, or death. Results: Of the 56 patients included in the study, 12 (21%) presented low serum C3 (LowC3) at the time of renal biopsy. TMA was significantly more frequent in the LowC3 group [7/12 (58%) vs. 9/44 (20%), p = 0.02]. After adjusting for potential confounders, LowC3 was strongly associated with an increased hazard of ESKD (hazard ratio [HR]: 5.84 [95%CI: 1.69, 20.15; p = 0.005). The association was not affected by adjusting for TMA. The estimated overall proportion of the relation between C3 and ESKD mediated by TMA was low and not statistically significant. Conclusions: Our study provides evidence that C3 hypocomplementemia is associated with an increased risk of ESKD through mechanisms that are largely independent from TMA. Full article
(This article belongs to the Special Issue Glomerular Diseases: Advances in Diagnosis and Management)
Show Figures

Figure 1

13 pages, 1326 KiB  
Article
Contrast-Induced Acute Kidney Injury in Patients with Heart Failure on Sodium–Glucose Cotransporter-2 Inhibitors Undergoing Radiocontrast Agent Invasive Procedures: A Propensity-Matched Analysis
by Giulia Nardi, Enrico Marchi, Marco Allinovi, Gianmarco Lugli, Lucrezia Biagiotti, Francesca Maria Di Muro, Renato Valenti, Iacopo Muraca, Benedetta Tomberli, Niccolò Ciardetti, Brunetto Alterini, Francesco Meucci, Carlo Di Mario and Alessio Mattesini
J. Clin. Med. 2024, 13(7), 2041; https://doi.org/10.3390/jcm13072041 - 01 Apr 2024
Viewed by 561
Abstract
(1) Background: This single-center retrospective study aimed to evaluate whether sodium–glucose cotransporter-2 inhibitors (SGLT2-i) therapy may have a nephroprotective effect to prevent contrast-induced acute kidney injury (CI-AKI) in patients with heart failure (HF) undergoing iodinated contrast medium (ICM) invasive procedures. (2) Methods [...] Read more.
(1) Background: This single-center retrospective study aimed to evaluate whether sodium–glucose cotransporter-2 inhibitors (SGLT2-i) therapy may have a nephroprotective effect to prevent contrast-induced acute kidney injury (CI-AKI) in patients with heart failure (HF) undergoing iodinated contrast medium (ICM) invasive procedures. (2) Methods: The population was stratified into SGLT2-i users and SGLT2-i non-users according to the chronic treatment with gliflozins. The primary endpoint was CI-AKI incidence during hospitalization. Secondary endpoints were all-cause mortality and the need for continuous renal replacement therapy (CRRT). (3) Results: In total, 86 patients on SGLT2-i and 179 patients not on SGLT2-i were enrolled. The incidence of CI-AKI in the gliflozin group was lower than in the non-user group (9.3 vs. 27.3%, p < 0.001), and these results were confirmed after propensity matching analysis. Multivariable logistic regression showed that only SGLT2-i treatment was an independent preventive factor for CI-AKI (OR: 0.41, 95% CI: 0.16–0.90, p = 0.045). The need for CRRT was reported only in five patients in the non-SGLT2-i-user group compared to zero patients in the gliflozin group (p = 0.05). (4) Conclusions: SGLT2-i therapy was associated with a lower risk of CI-AKI in patients with HF undergoing ICM invasive procedures. Full article
(This article belongs to the Special Issue Glomerular Diseases: Advances in Diagnosis and Management)
Show Figures

Figure 1

10 pages, 468 KiB  
Article
Using Renal Elastography to Predict the Therapeutic Response of Nephrological Patients
by Nicoletta Mancianti, Guido Garosi, Ernesto Iadanza, Sergio Antonio Tripodi, Andrea Guarnieri, Massimo Belluardo, Edoardo La Porta, Marta Calatroni, Maria Antonietta Mazzei and Palmino Sacco
J. Clin. Med. 2023, 12(23), 7385; https://doi.org/10.3390/jcm12237385 - 29 Nov 2023
Viewed by 737
Abstract
Background: The standard method for assessing chronic renal damage is renal biopsy, which has limitations due to its invasiveness. Ultrasound elastography is a non-invasive technique that quantifies tissue elasticity and can be used to determine Young’s modulus (YM). Although this breakthrough technology has [...] Read more.
Background: The standard method for assessing chronic renal damage is renal biopsy, which has limitations due to its invasiveness. Ultrasound elastography is a non-invasive technique that quantifies tissue elasticity and can be used to determine Young’s modulus (YM). Although this breakthrough technology has been successfully employed to evaluate liver stiffness and the extent of fibrosis, its application in kidney-related conditions still needs improvement. Methods: Our study aimed to verify the correlation between renal elastography and the chronic histological score determined via renal biopsy, evaluate the correlation between elastography and response to treatment in the short-term follow-up (6 months), and compare elastography data between renal disease patients (AKD-P) and healthy controls (HP). Results: The analyzed population consisted of 82 patients (41 HP and 41 AKD-P). The AKD-P were divided into responders (R) or non-responders (NR) based on the criteria established by the guidelines. No association was found between renal stiffness and chronic histological score. Elastography data revealed median YM values of 6.15 kPa for AKD-P and 12.2 kPa for HP, with a statistically significant difference. The median YM values of the R and NR groups were 7.4 KPa and 5.6 KPa, respectively (p = 0.037). Conclusions: Patient responsiveness was associated with YM, with lower values observed in the NR group. We also found that the healthy controls exhibited significantly higher YM values than the renal disease population. Full article
(This article belongs to the Special Issue Glomerular Diseases: Advances in Diagnosis and Management)
Show Figures

Figure 1

Review

Jump to: Research

15 pages, 695 KiB  
Review
Thrombotic Microangiopathy as a Life-Threatening Complication of Long-Term Interferon Beta Therapy for Multiple Sclerosis: Clinical Phenotype and Response to Treatment—A Literature Review
by Marco Allinovi, Tommaso Mazzierli, Selene Laudicina, Luisa Pastò, Emilio Portaccio, Maria Pia Amato and Giorgio Trivioli
J. Clin. Med. 2024, 13(6), 1598; https://doi.org/10.3390/jcm13061598 - 11 Mar 2024
Viewed by 726
Abstract
Thrombotic microangiopathy (TMA) has been observed in some patients receiving interferon beta (IFNβ) therapy for relapsing-remitting multiple sclerosis, but little is known about its clinical features and outcomes. We searched the literature to identify cases with IFNβ-related TMA and assessed their pattern of [...] Read more.
Thrombotic microangiopathy (TMA) has been observed in some patients receiving interferon beta (IFNβ) therapy for relapsing-remitting multiple sclerosis, but little is known about its clinical features and outcomes. We searched the literature to identify cases with IFNβ-related TMA and assessed their pattern of organ involvement, the presence of prodromal manifestations, the treatments used, and the outcomes. Thirty-five articles met the inclusion criteria, and data of 67 patients were collected. The median duration of IFNβ therapy before the diagnosis of TMA was 8 years, and 56/67 (84%) presented with acute kidney injury (AKI), of which 33 required acute dialysis. All but three patients had manifestations during the four weeks before TMA onset, including flu-like symptoms, headache, and worsening blood pressure control. In only two patients, ADAMTS13 activity was reduced, while 27% had low C3 levels. However, none showed causative genetic mutations associated with development of atypical hemolytic uremic syndrome. All patients discontinued IFNβ, 34 (55%) also received plasma exchange, and 12 (18%) received eculizumab. Complete renal recovery was achieved by 20 patients (30%), while 13 (20%) developed end-stage renal disease. Among those with AKI requiring dialysis, eculizumab therapy was associated with a significantly reduced risk of ESRD compared with plasma exchange. Therefore, TMA with features of aHUS mainly occurs after prolonged treatment with IFNβ and is preceded by prodromes, which may lead to an early diagnosis before life-threatening complications occur. Eculizumab appears beneficial in cases with severe kidney involvement, which supports a role of the complement system in the pathogenesis of these forms. Full article
(This article belongs to the Special Issue Glomerular Diseases: Advances in Diagnosis and Management)
Show Figures

Figure 1

Back to TopTop