Nephrotic syndrome associated with Kimura disease.
Kimura disease presents as benign subcutaneous swelling predominantly around the head and neck region. It has a high incidence of renal involvement. However, the pathogenesis of this association remains elusive. Only 2 pediatric cases and 11 adult cases of Kimura disease with renal involvement have been reported in the literature. In recent years many immunopathogenetic features suggesting an underlying T-cell and related cytokine defect have been noted in Kimura disease. We describe a unique case of an Asian boy who presented with nephrotic syndrome resistant to steroid and cytotoxic therapy, and 5 years later developed cervical lymphadenopathy consistent with Kimura disease. We also review the literature, summarizing the presentation, differential diagnosis, incidence of renal disease, prognosis, immunopathogenetic features, and therapy.
Rajpoot
D K
DK
Department of Pediatrics, University of California, Irvine, USA.
Pahl
M
M
Clark
J
J
D000293:Adolescent; D000796:Angiolymphoid Hyperplasia with Eosinophilia / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D002699:Chlorambucil / Q000627:therapeutic use; D004351:Drug Resistance; D017809:Fatal Outcome; D015432:Glomerulonephritis, Membranoproliferative / Q000150:complications / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D013256:Steroids / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D018906:Antineoplastic Agents, Alkylating; D013256:Steroids; D002699:Chlorambucil; D011241:Prednisone