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      Behçet’s Disease and AA-Type Amyloidosis

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          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.

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          Criteria for diagnosis of Behcet's disease

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            End-Stage Renal Failure due to Crescentic Glomerulonephritis in a Patient with Behçet’s Syndrome

            We report a rare case of a patient with Behçet’s syndrome who developed end-stage renal failure due to crescentic glomerulonephritis (GN). A 20-year-old male patient had suffered from uveitis, aphthous mouth ulcers and genital ulceration for the past 7 years. His renal function rapidly deteriorated and renal biopsy specimens obtained when his serum creatinine level was 3 mg/dl showed diffuse proliferative GN with fibrous crescent formation in 75% of glomeruli excluding totally sclerotic glomeruli. Immune complexes were identified by demonstration of complement and immunoglobulins in the glomeruli. He developed end-stage renal failure during a 1-year course and received maintenance hemodialysis. We reviewed the literature on severe forms of GN in patients with Behçet’s syndrome.

              Author and article information

              Am J Nephrol
              American Journal of Nephrology
              S. Karger AG
              February 2000
              13 January 2000
              : 20
              : 1
              : 68-70
              Departments of aNephrology and bPathology, School of Medicine, Ondokuz Mayıs University, Samsun, Turkey
              13559 Am J Nephrol 2000;20:68–70
              © 2000 S. Karger AG, Basel

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              Page count
              Figures: 1, References: 24, Pages: 3
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              Case Report

              Cardiovascular Medicine, Nephrology

              Amyloidosis, Behçet’s disease, Prognosis


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