title: Kimura disease with advanced renal damage with anti-tubular basement membrane antibody.
abstract: Kimura disease (KD) is an autoimmune eosinophilic granulomatous disorder with generalized lymphadenopathy. A handful of pediatric patients with renal disease have been described, none of whom have been African-American (AA). We present an AA boy with KD and nephrotic syndrome (NS). Two months after stopping steroids, fever, asthma, eczema, and proteinuria recurred. His NS did not relapse but his platelet count decreased to 51,000/microl (x10(6)/l). On restarting prednisone, his platelet count normalized. A kidney biopsy revealed 23 of 37 glomeruli obsolescent and advanced damage with over 50% of cortical tissue replaced by interstitial fibrosis and chronic inflammation. Glomerular immunofluorescence was largely negative; very intense linear anti-tubular basement membrane (TBM) deposits of IgA, IgG, C3, and C4 were noted. At present, 36 months from onset, serum creatinine is 1.2 mg/dl (106 micromol/l). We present a 4-year-old AA boy with KD, NS, relapsing thrombocytopenia, and renal damage with anti-TBM antibody.
authors.affiliation: Department of Pediatrics, Steele Memorial Research Center, University of Arizona, Tucson, Arizona 85724, USA. mdixit@peds.arizona.edu
authors.forename: Mehul P
authors.initials: MP
authors.lastname: Dixit
authors.forename: Katherine M
authors.initials: KM
authors.lastname: Scott
authors.forename: Erika
authors.initials: E
authors.lastname: Bracamonte
authors.forename: Naznin M
authors.initials: NM
authors.lastname: Dixit
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Schumacher
authors.forename: John
authors.initials: J
authors.lastname: Hutter
authors.forename: Raymond
authors.initials: R
authors.lastname: Nagle
mesh_terms: D000796:Angiolymphoid Hyperplasia with Eosinophilia / Q000150:complications* / Q000473:pathology; D000906:Antibodies / Q000032:analysis; D001323:Autoantibodies; D002675:Child, Preschool; D018450:Disease Progression; D006801:Humans; D007223:Infant; D008297:Male; D009404:Nephrotic Syndrome / Q000150:complications* / Q000276:immunology
chemical_list: D000906:Antibodies; D001323:Autoantibodies; C051951:antiglomerular basement membrane antibody