Rituximab Treatment of Fibrillary Glomerulonephritis

ArticleinAmerican Journal of Kidney Diseases 52(6):1158-62 · October 2008with64 Reads
DOI: 10.1053/j.ajkd.2008.07.011 · Source: PubMed
Fibrillary glomerulonephritis belongs to a group of disorders characterized by pathogenic deposition of fibrils in glomeruli. This glomerulopathy tends to progress to end-stage kidney disease, and there currently are no treatments of proven benefit, including corticosteroids and cytotoxic agents. Because the glomerular deposits contain an immunoglobulin component, it was postulated that anti-B-cell therapy with rituximab, an anti-CD20 monoclonal antibody, may be effective in the treatment of patients with fibrillary glomerulonephritis. We describe 3 patients with fibrillary glomerulonephritis who were treated with rituximab for nephrotic-range proteinuria. Each patient also received standard antiproteinuria therapy, including blockade of the renin-angiotensin system and strict blood pressure control. All patients showed a decrease in proteinuria to less than 1.5 g/d of protein by 27 months, and kidney function was preserved throughout the duration of therapy and follow-up. No adverse effects were seen with rituximab. These outcomes suggest that treatment with rituximab may be a promising approach to the management of fibrillary glomerulonephritis, an entity previously considered refractory to therapy.
    • "Regarding treatment, although no effective therapy has been established for FGN, some clinicians have treated the disease according to pathologic findings. Cyclosporine has been used successfully to treat patients with a membranous pattern, and rituximab has been used successfully to treat patients with an MPGN pattern [9, 16] . In previous investigations , the prognosis of patients with FGN has been poor, and 40 %-50 % of such patients have developed ESRD within 6 years of presentation [2, 17]. "
    [Show abstract] [Hide abstract] ABSTRACT: Background Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that seldom coexists with other diseases. Membranoproliferative glomerulonephritis is a pathologic finding of renal lesions associated with IgM-secreting monoclonal proliferations. We present a case study of a patient with unusual simultaneous FGN and IgM-related renal disorder in nonmalignant monoclonal IgM gammopathy. Case presentation A 63-year-old male presented with nephrotic syndrome and elevated serum creatinine levels. Laboratory examination revealed elevated levels of serum IgM and low C3 levels. Serum and urine immunofixation electrophoresis showed a monoclonal IgM with a kappa light chain. A bone marrow biopsy revealed less than 5 % bone marrow infiltration by lymphoplasmacytic lymphoma, and a renal biopsy revealed mesangiocapillary glomerulonephritis on light microscopy. Immunofluorescent and immunohistochemical staining indicated granular deposits of immunoglobulin G in the mesangium and granular deposits of immunoglobulin M and κ light chains along the capillary wall. Electron microscopy revealed randomly arranged nonbranching fibrils of approximately 15 nm in diameter in the glomerular mesangium and subendothelial electron-dense deposits. According to these results, we confirmed FGN and membranoproliferative glomerulonephritis, which were attributed to monoclonal IgM deposits. Conclusion To the best of our knowledge, this is the first report of simultaneous FGN and membranoproliferative glomerulonephritis in nonmalignant IgM monoclonal gammopathy.
    Full-text · Article · Dec 2016
    • "m 2 . Most reported treatment 'successes' of fibrillary GN with any immunosuppression regimen have been in patients with preserved renal function [8, 9, 12] . Three of our non-progressors (Patients 4, 10 and 12) had serum creatinine of <1.2 mg/ dL and eGFR of >60 mL/min/1.73 "
    [Show abstract] [Hide abstract] ABSTRACT: Background Approximately 50% of patients with fibrillary glomerulonephritis (GN) progress to end-stage renal disease (ESRD) within 2 years of diagnosis, and no standard therapy exists. The data on rituximab therapy for fibrillary GN are limited and have inconsistent outcomes. Here, we report the largest case series to date using rituximab for fibrillary GN.
    Full-text · Article · May 2014
    • "There have been no controlled trials that agree on recommending an ideal therapy, and most reports choose the treatment according to light microscopy findings. Different agents have been proposed as treatment of this rare entity: corticosteroid or cytotoxic agents have been used with limited effectiveness [19]; some groups have published their successful experience using mycophenolate mofetil, rituximab concomitantly with corticosteroids, immunoglobulin, or calcineurin inhibitors [4, 20, 21]. There is controversy in relation to the use of plasmapheresis as a therapy. "
    [Show abstract] [Hide abstract] ABSTRACT: Although clinical presentation of fibrillary glomerulonephritis is similar to most forms of glomerulonephritis, it is usually difficult to make the diagnosis. Clinical manifestations include proteinuria, microscopic haematuria, nephrotic syndrome, and impairment of renal function. A diagnosis of fibrillary glomerulonephritis is only confirmed by renal biopsy and it must comprise electronmicroscopy-verified ultrastructural findings. We report four cases between 45-50 years old with documented type 2 diabetes mellitus (T2DM) and arterial hypertension. All patients were found to have fibrils on kidney biopsy. The differential diagnosis of fibrils in the setting of diabetes mellitus is also discussed.
    Full-text · Article · May 2013
Show more