title: Crescentic glomerulonephritis in hyper IgD syndrome.
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.
authors.affiliation: Unité de Néphrologie Pédiatrique, Service de Pédiatrie Multidisciplinaire, Hôpital d'Enfants de la Timone, Marseille, France. gpicon@aphml.ap-hm.fr
authors.forename: M
authors.initials: M
authors.lastname: Tsimaratos
authors.forename: I
authors.initials: I
authors.lastname: Koné-Paut
authors.forename: L
authors.initials: L
authors.lastname: Daniel
authors.forename: M C
authors.initials: MC
authors.lastname: Gubler
authors.forename: B
authors.initials: B
authors.lastname: Dussol
authors.forename: G
authors.initials: G
authors.lastname: Picon
mesh_terms: D002648:Child; D016207:Cytokines / Q000097:blood; D005921:Glomerulonephritis / Q000097:blood / Q000150:complications* / Q000473:pathology; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood / Q000150:complications* / Q000473:pathology; D007072:Immunoglobulin D / Q000097:blood*; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000473:pathology / Q000601:surgery; D007678:Kidney Glomerulus / Q000473:pathology; D016030:Kidney Transplantation; D008297:Male; D013577:Syndrome
chemical_list: D016207:Cytokines; D007072:Immunoglobulin D