Crescentic glomerulonephritis in hyper IgD syndrome

The hyperimmunoglobulinemia D syndrome (HIDS) is a well-defined entity rese mbling familial Mediterranean fever. HIDS is a systemic inflammatory diseas e associated with stimulation of T-cell-mediated immunity. These patients a re at low risk for amyloidosis and are not known to develop nephropathy. We report a boy of Mediterranean ancestry who exbibited typical HIDS and end- stage renal failure. Kidney biopsy revealed pauci-immune crescentic glomeru lonephritis (cGN). We hypothesized that: the glomerular involvement was sec ondary to the cytokine network activation observed in HIDS. Thus, cGN shoul d be considered as part of the syndrome, and kidney biopsy should be perfor med early in the course of the renal disease in patients with HIDS.