Abstract
The hyperimmunoglobulinemia D syndrome (HIDS) is a well-defined entity resembling familial Mediterranean fever. HIDS is a systemic inflammatory disease associated with stimulation of T-cell-mediated immunity. These patients are at low risk for amyloidosis and are not known to develop nephropathy. We report a boy of Mediterranean ancestry who exhibited typical HIDS and end-stage renal failure. Kidney biopsy revealed pauci-immune crescentic glomerulonephritis (cGN). We hypothesized that the glomerular involvement was secondary to the cytokine network activation observed in HIDS. Thus, cGN should be considered as part of the syndrome, and kidney biopsy should be performed early in the course of the renal disease in patients with HIDS.
Similar content being viewed by others
Author information
Authors and Affiliations
Additional information
Received: 2 February 1998 / Revised: 30 June 1998 / Accepted: 2 July 1998
Rights and permissions
About this article
Cite this article
Tsimaratos, M., Koné-Paut, I., Daniel, L. et al. Crescentic glomerulonephritis in hyper IgD syndrome. Pediatr Nephrol 13, 132–134 (1999). https://doi.org/10.1007/s004670050579
Issue Date:
DOI: https://doi.org/10.1007/s004670050579