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

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          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.

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          Most cited references 6

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          The immune complex pathogenesis of glomerulonephritis and pulmonary vasculitis in Behçet's disease.

          A kidney and lung biopsy were performed on a patient with active Behçet's disease with renal and pulmonary involvement. Histologic, immunohistochemical and electron microscopic studies of the kidney biopsy specimen revealed a focal segmental necrotizing glomerulonephritis characterized by the presence of numerous subendothelial and occasional intramembranous deposits containing immunoglobulin G (IgG), the third component of complement (C3), the fourth component of complement (C4) and fibrin(ogen). Histologic and immunohistochemical studies of the lung biopsy specimen showed an acute venulitis and septal capillaritis associated with the presence of identical deposits within the walls of affected vessels. Circulating immune complexes were detected in the patient's serum by Raji cell assay. The findings indicate that the glomerulonephritis and pulmonary vasculitis occasionally occurring in Behçet's disease are due to the deposition of circulating antigen-antibody complexes. In addition, they strongly suggest that the majority of the major and minor manifestations of the disease, such as uveitis, cutaneous vasculitis, synovitis and meningoencephalitis, are a result of vascular immune complex deposition.
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            Proliferative glomerulonephritis with crescent formation in behcet's syndrome

             P. Olsson (1980)
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              • Abstract: not found
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              Rapidly progressive glomerulonephritis in Behcet's syndrome

               D. Landwehr (1980)

                Author and article information

                Am J Nephrol
                American Journal of Nephrology
                S. Karger AG
                August 1998
                05 June 1998
                : 18
                : 4
                : 321-324
                Department of Internal Medicine, Saga Medical School, Saga, Japan
                13358 Am J Nephrol 1998;18:321–324
                © 1998 S. Karger AG, Basel

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                Figures: 2, Tables: 2, References: 20, Pages: 4
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