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- W1974139685 abstract "Systemic lupus erythematosus (SLE) nephritis, the prototypic systemic autoimmune disease, is characterized by the influx of mononuclear inflammatory cell infiltrates within the glomeruli and renal interstitium. In order to evaluate the possibility that intrarenal T cells result from the expansion of lymphocytes utilizing limited TCR genes, we analyzed the TCR Vβ gene expression among infiltrating lymphocytes in renal tissue as compared to simultaneous peripheral blood lymphocytes in patients with SLE nephritis. Intrarenal TCR Vβ gene expression was determined by polymerase chain reaction employing 25 Vβ oligonucleotide 5' primers paired with a single 3' Cβ primer. Amplified Vβ gene products were identified by hybridization using an internal TCR Cβ probe and quantified using a phosphor-imager. Sequence analysis and CDR3 size spectratyping were employed to confirm oligoclonal expansion of intrarenal lymphocytes. Previous work in our laboratory has shown that Vβ gene usage by infiltrating lymphocytes in the kidney is restricted in children with new onset SLE nephritis. We now report on siblings (11 yo male, 23 yo female) who developed within a year of one another an aggressive diffuse proliferative glomerulonephritis resulting in end-stage-renal-disease. The peripheral TCR Vβ repertoire in these two patients were unique when compared to normal controls and other SLE patients in that they did not show the expected diverse and heterogeneous Vβ gene expression. Longitudinally acquired renal specimens from the brother showed evolution from an intrarenal Vβ 2,3, and 6 predominance to one more reflective of a later restricted peripheral repertoire with a Vβ 18 predominance. Similarly, the sister's initial biopsy showed intrarenal expansion of Vβ 2, 16 and 18 families. These findings would suggest the possibility that the local accumulation of T cells involves specific homing or expansion of a limited number of T cell clones. In addition, the phenotypic similarity in the progression of renal disease in these two siblings implicates that genetic predisposition is a key element in susceptibility." @default.
- W1974139685 created "2016-06-24" @default.
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- W1974139685 date "1998-04-01" @default.
- W1974139685 modified "2023-10-18" @default.
- W1974139685 title "Oligoclonal Expansion of Intrarenal T Cells in a Pair of Siblings With SLE Nephritis • 1983" @default.
- W1974139685 doi "https://doi.org/10.1203/00006450-199804001-02006" @default.
- W1974139685 hasPublicationYear "1998" @default.
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