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Title: IgA myeloma presenting as Henoch-Schönlein purpura with nephritis.
Authors: Zickerman, AM
Allen, AC
Talwar, V
Olczak, SA
Brownlee, A
Holland, M
Furness, PN
Brunskill, NJ
Feehally, J
First Published: Sep-2000
Citation: AM J KIDNEY DIS, 2000, 36 (3), pp. E19-E19
Abstract: IgA nephropathy (IgAN) and Henoch-Schönlein purpura (HSP) are both characterized by IgA-mediated tissue injury, including mesangial proliferative glomerulonephritis. Abnormalities of IgA1 glycosylation are described in IgA nephropathy and HSP nephritis. IgA-antineutrophil cytoplasmic antibodies (ANCA) have been inconsistently described in the serum of patients with HSP. In IgA myeloma, the paraprotein-mediated renal lesion is typically cast nephropathy; IgAN or HSP have only rarely been reported in myeloma even when an IgA paraprotein is circulating in large concentrations. We report the case of a 50-year-old man with IgA myeloma who presented with HSP including nephritis and rapidly progressive renal failure. His IgA1 had altered O-glycosylation in the pattern seen in IgAN and also contained an IgA-ANCA. This case adds further weight to the evidence that IgA1 O-glycosylation abnormalities predispose to mesangial IgA deposition and also that IgA-ANCA may have a pathogenic role in the development of HSP.
DOI Link: 10.1053/ajkd.2000.16221
eISSN: 1523-6838
Type: Journal Article
Appears in Collections:Published Articles, Dept. of Infection, Immunity and Inflammation

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