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
Hauptverfasser: Leonard, Deanne, Cortese, Cherise, Wadei, Hani M, Porter Ii, Ivan E, Aslam, Nabeel
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container_issue 5
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container_title Clinical nephrology
container_volume 89
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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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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