We report a case of mesangial proliferative glomerulonephritis with interstitial nephritis associated with multicentric Castleman's disease (MCD) successfully treated with an anti-interleukin-6 receptor antibody (tocilizumab). This mesangial proliferative glomerulonephritis with interstitial nephritis was resistant to methylprednisolone treatment; however, it was markedly improved with tocilizumab, which was administered intravenously at a dose of 8 mg/kg every 2 weeks. These results suggest that tocilizumab is effective for the treatment of mesangial proliferative glomerulonephritis with interstitial nephritis associated with MCD.
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http://dx.doi.org/10.2169/internalmedicine.51.6555 | DOI Listing |
Nephrology (Carlton)
February 2025
Phoenix Children's Hospital-Thomas Campus, Phoenix, Arizona, USA.
Complement 3 glomerulonephritis (C3GN) is a rare glomerular disease involving dysregulation of the complement system. We describe our experience using pegcetacoplan, an inhibitor of C3 and its activation fragment, C3b, for treatment-resistant C3GN in a 9-year-old boy referred for evaluation of refractory membranoproliferative glomerulonephritis. Despite treatment with intense immunosuppression (high-dose steroids, mycophenolate mofetil and calcineurin inhibitor), he continued to have high disease activity with low C3 levels (35 mg/dL), hypertension, symptomatic oedema, anaemia, and nephrotic-range proteinuria (e.
View Article and Find Full Text PDFInt J Mol Sci
January 2025
Department of Pathology, Albert Szent-Györgyi Medical Center, Faculty of Medicine, University of Szeged, 6720 Szeged, Hungary.
Fibronectin glomerulopathy (FG) is caused by fibronectin 1 () gene mutations. A renal biopsy was performed on a 4-year-old girl with incidentally discovered proteinuria (150 mg/dL); her family history of renal disease was negative. Markedly enlarged glomeruli (mean glomerular diameter: 196 μm; age-matched controls: 140 μm), α-SMA-positive and Ki-67-positive mesangial cell proliferation (glomerular proliferation index 1.
View Article and Find Full Text PDFZhonghua Er Ke Za Zhi
February 2025
Department of Nephrology, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai Kidney Development and Pediatric Kidney Disease Research Center State, Key Laboratory of Kidney Diseases, Shanghai 201102, China.
To investigate the short-term efficacy and safety of rituximab (RTX) in children with calcineurin inhibitor (CNI) resistant steroid resistant nephrotic syndrome (SRNS). A retrospective case analysis was conducted. Thirteen children with CNI resistant SRNS who were regularly treated with RTX (375 mg/m per dose (maximum dose 500 mg), 1 dose per week, a total of 4 doses) in Department of Nephrology, Children's Hospital of Fudan University from January 2016 to December 2023 were enrolled.
View Article and Find Full Text PDFSci Rep
January 2025
Department of Nephrology, The First Affiliated Hospital, Sun Yat-sen University, 58 Zhongshan Road II, Guangzhou, 510080, China.
Nephrotic syndrome (NS) occurs in 5-15% of patients with IgA nephropathy (IgAN), resulting in poorer long-term outcomes compared to those without NS. Clinical features and renal prognosis for patients with both NS and IgAN across different kidney pathologies have not been fully elucidated. This study included patients with primary IgAN through renal biopsy at the First Affiliated Hospital of Sun Yat-sen University from January 2001 to November 2021 presenting with NS.
View Article and Find Full Text PDFThe maintenance of a healthy epithelial-endothelial juxtaposition requires cross-talk within glomerular cellular niches. We sought to understand the spatially-anchored regulation and transition of endothelial and mesangial cells from health to injury in DKD. From 74 human kidney samples, an integrated multi-omics approach was leveraged to identify cellular niches, cell-cell communication, cell injury trajectories, and regulatory transcription factor (TF) networks in glomerular capillary endothelial (EC-GC) and mesangial cells.
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