Crescentic glomerulonephritis in Behçet's syndrome--results of therapy and review of the literature.

The syndrome initially described by Behçet in 1937 comprised the triad of ocular inflammation, oral and genital ulcers. The described manifestations have gradually expanded to include most tissues; renal involvement has been described but is usually mild. Although crescentic glomerulonephritis (GN) has been reported, it is extremely rare. We report two patients presenting with Behçet's syndrome complicated by an active crescentic GN. Their course, following the initiation of therapy with prednisone and cyclophosphamide is described. Both patients evolved to a sclerosed glomerulopathy documented by follow-up renal biopsy in spite of a dramatic clinical improvement in one patient and a stabilization of renal function in the other. Both developed nephrotic range proteinuria without amyloidosis.