Membranoproliferative glomerulonephritis (MPGN) is a rare glomerular disease characterized by mesangial hypercellularity and thickening of the glomerular basement membrane (GBM). MPGN can be idiopathic or associated with malignancy, systemic immune complex disorders and chronic infections. It has rarely been associated with solid organ tumors, such as lung, gastric, breast or prostate cancer. We report a patient with MPGN and coexisting colorectal carcinoma. A 48-year-old man presented with anemia, loss of weight, hypertension, and nephrotic syndrome. The renal biopsy findings were compatible with type 1 MPGN. The antineutrophilic cytoplasmic antibodies, antinuclear antibodies, anti-GBM, serologic markers of hepatitis B and hepatitis C and tumor markers were negative. After ruling out the secondary causes of MPGN, the patient was treated with pulse doses of methylprednisolone and a single dose of cyclophosphamide. However, due to the worsening anemia and rectal bleeding, a colonoscopy was performed, which established a diagnosis of adenocarcinoma of the descending colon. The patient was treated with left hemicolectomy and oral corticosteroids. Within a year after the cancer treatment, the patient experienced a complete resolution of the proteinuria and improvement of the kidney function. Although rare, MPGN can be associated with hematologic malignancies and solid organ tumors. The most common causes of secondary MPGN should be ruled out before starting specific treatment. In our patient, cancer treatment has led to a subsequent remission of the nephrotic syndrome, which indicated that this association was not coincidental but rather causal. In patients with a tumor and concomitant glomerulopathy which is suspected to be paraneoplastic in etiology, the treatment of the underlying malignancy should be prioritized.
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http://dx.doi.org/10.2478/prilozi-2024-0013 | DOI Listing |
Kidney Int Rep
January 2025
Lausanne University Hospital, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
Introduction: Complement 3 glomerulopathy (C3G) and primary immune complex membranoproliferative glomerulonephritis (IC-MPGN) have high risks for disease recurrence and allograft loss in transplant kidneys. Pegcetacoplan (targeted complement 3 [C3]/C3b inhibitor) may prevent excessive deposition of C3 and complement 5 [C5] breakdown products and associated renal damage.
Methods: NOBLE (NCT04572854) is a prospective, phase 2, multicenter, open-label, randomized controlled trial evaluating the efficacy and safety of pegcetacoplan in posttransplant patients with recurrent C3G or IC-MPGN.
Kidney Int Rep
January 2025
Transplantation Center, Departments of Medicine and Surgery, Lausanne University Hospital and University of Lausanne, Switzerland.
Introduction: Approximately 50% of patients with C3 glomerulopathy (C3G) and primary immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) reach kidney failure 10 years after diagnosis. Because these patients are generally young, the majority will be listed for kidney transplantation (KTx). However, reported outcomes in patients transplanted for C3G and IC-MPGN are heterogeneous and conflicting, because they are mainly based on retrospective monocentric studies.
View Article and Find Full Text PDFKidney Int Rep
January 2025
Service of Nephrology and Hypertension, Department of Medicine, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
Immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) and complement 3 glomerulopathy (C3G) are rare, complement-mediated kidney diseases, previously classified under the group of kidney disorders termed membranoproliferative glomerulonephritis (MPGN) type 1, type 2, and type 3. Despite new advances in our understanding of IC-MPGN and C3G, several unmet needs persist in the diagnosis and management of patients with these nephropathies, due in part to their rarity and their overlapping clinical presentations, histologic features, and underlying pathophysiologies. This review summarizes our current understanding of the role of complement in IC-MPGN and C3G, and underlines the key histopathologic differences between the diseases.
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.
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