Behçet’s Disease and AA-Type Amyloidosis
- 13 January 2000
- journal article
- case report
- Published by S. Karger AG in American Journal of Nephrology
- Vol. 20 (1), 68-70
- https://doi.org/10.1159/000013559
Abstract
Behçet’s disease (BD) is a multisystem disorder characterized by vasculitis. To our knowledge, 52 patients with BD and amyloidosis have previously been described in the literature. Nephrotic syndrome was the most common type of presentation of amyloidosis in these patients. The prognosis of patients with BD and amyloidosis has not been reported before. In this report, we present a patient with BD and AA-type amyloidosis and analyze the prognosis in these patients. Follow-up and prognosis have been reported in 23 patients. Ten of these 23 patients died and most of these deaths occurred within 3 months after the diagnosis of amyloidosis. End-stage renal disease developed shortly in 4 patients. BD should be considered in the differential diagnosis of AA amyloidosis. In conclusion, the cases with BD and amyloidosis carry poor prognosis.Keywords
This publication has 2 references indexed in Scilit:
- End-Stage Renal Failure due to Crescentic Glomerulonephritis in a Patient with Behçet’s SyndromeAmerican Journal of Nephrology, 1998
- Criteria for diagnosis of Behcet's diseaseThe Lancet, 1990