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Rapidly Progressive Glomerulonephritis Associated With Anti-glomerular Basement Membrane Disease: A Rare Diagnosis.
Cureus
December 2024
Medicine, Faculdade de Medicina da Universidade do Porto (FMUP), Porto, PRT.
Anti-glomerular basement membrane disease is a rare small vessel vasculitis caused by the deposition of immunoglobulin G (IgG) autoantibodies in the basement membrane of glomerular capillaries and lung alveoli, leading to rapidly progressive renal failure and/or alveolar hemorrhage. We report the case of an 83-year-old female patient presenting with uremic symptoms, rapidly progressive kidney failure, and a high titer of anti-glomerular basement membrane antibodies. Given the urgent need for kidney replacement therapy, the substantial fibrosis and glomerular scarring observed in the kidney biopsy suggesting a chronic process, and the absence of pulmonary involvement, neither immunosuppressive treatment nor plasmapheresis was initiated, since a low likelihood of a favorable response to these interventions was expected.
View Article and Find Full Text PDFMyeloperoxidase anti-neutrophil cytoplasmic antibody-associated vasculitis with silicosis, alveolar hemorrhage, and rapidly progressive glomerulonephritis: a case report.
AME Case Rep
October 2024
Center for Asbestos-Related Diseases, Toyama Rosai Hospital, Toyama, Japan.
Background: The underlying pathophysiology of some occupational diseases such as silicosis involves autoantibodies. An autoantibody, antineutrophil cytoplasmic antibody (ANCA), has been recently reported and is known to be elevated in diseases such as vasculitis; therefore, the disease is currently known as ANCA-associated vasculitis. The risk of ANCA-associated vasculitis is known to be 25 times higher in patients with silicosis than in those without any occupational disease.
View Article and Find Full Text PDFImproved therapeutic efficacy of a bifunctional anti-C5 mAb-FH SCR1-5 fusion protein over anti-C5 mAb in an accelerated mouse model of C3 glomerulopathy.
Immunohorizons
January 2025
Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States.
C3 glomerulopathy (C3G), a rare kidney disease caused by dysregulation of alternative pathway complement activation, is characterized by glomerular C3 deposition, proteinuria, crescentic glomerulonephritis, and renal failure. The anti-C5 monoclonal antibody (mAb) drug eculizumab has shown therapeutic effects in some but not all patients with C3G, and no approved therapy is currently available. Here, we developed and used a triple transgenic mouse model of fast progressing lethal C3G (FHm/mP-/-hFDKI/KI) to compare the therapeutic efficacy of a bifunctional anti-C5 mAb fused to a functional factor H (FH) fragment (short consensus repeat 1-5 [SCR1-5]) and the anti-C5 mAb itself.
View Article and Find Full Text PDFFibronectin Glomerulopathy Without Typical Renal Biopsy Features in a 4-Year-Old Girl with Incidentally Discovered Proteinuria and a G417V Gene Mutation.
Int 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 PDFRapidly progressive glomerulonephritis as an unusual type of renal involvement in sarcoidosis: a case report.
J Med Case Rep
January 2025
Transplant-Nephrology Department, Transplantation Center, University Hospital Martin, Kollarova 2, 03601, Martin, Slovakia.
Introduction: Sarcoidosis is a multisystem inflammatory disease of unknown etiology characterized by the formation of noncaseating epithelioid granulomas. Clinically significant renal involvement is rare in sarcoidosis. It most commonly manifests as chronic tubulointerstitial nephritis and nephrocalcinosis with nephrolithiasis.
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