Behçet's syndrome and focal segmental glomerulosclerosis with nephrotic syndrome - successful treatment with etanercept
Behçet's syndrome (BS) often presents with aphthous and genital ulcers, uveitis, and erythema nodosum. Renal involvement has been reported with most cases presenting with renal amyloidosis, IgA nephropathy, or crescentic glomerulonephritis. We describe a case of a 49-year-old woman with relapsi...
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Veröffentlicht in: | Clinical nephrology 2018-05, Vol.89 (5), p.371-375 |
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creator | Leonard, Deanne Cortese, Cherise Wadei, Hani M Porter Ii, Ivan E Aslam, Nabeel |
description | Behçet's syndrome (BS) often presents with aphthous and genital ulcers, uveitis, and erythema nodosum. Renal involvement has been reported with most cases presenting with renal amyloidosis, IgA nephropathy, or crescentic glomerulonephritis. We describe a case of a 49-year-old woman with relapsing BS symptoms coinciding with new-onset development of nephrotic syndrome. Renal biopsy showed focal segmental glomerulosclerosis (FSGS) that was treated with prednisone and etanercept for BS therapy. Both proteinuria and BS symptoms responded to treatment with complete remission. To our knowledge, our report is the first to show evidence of a possible connection between BS and FSGS and discusses the pathophysiologic mechanism that explains this link.
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Renal involvement has been reported with most cases presenting with renal amyloidosis, IgA nephropathy, or crescentic glomerulonephritis. We describe a case of a 49-year-old woman with relapsing BS symptoms coinciding with new-onset development of nephrotic syndrome. Renal biopsy showed focal segmental glomerulosclerosis (FSGS) that was treated with prednisone and etanercept for BS therapy. Both proteinuria and BS symptoms responded to treatment with complete remission. To our knowledge, our report is the first to show evidence of a possible connection between BS and FSGS and discusses the pathophysiologic mechanism that explains this link.
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subjects | Biopsy Blood pressure Creatinine Edema Erythema Hypertension Kidney diseases Laboratories Microscopy Nephrology Pathogenesis Patients Proteins Remission (Medicine) Tumor necrosis factor-TNF Ulcers Vein & artery diseases Women |
title | Behçet's syndrome and focal segmental glomerulosclerosis with nephrotic syndrome - successful treatment with etanercept |
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