Pediatric Nephrology

, Volume 19, Issue 12, pp 1404–1407 | Cite as

Kimura disease with advanced renal damage with anti-tubular basement membrane antibody

  • Mehul P. DixitEmail author
  • Katherine M. Scott
  • Erika Bracamonte
  • Naznin M. Dixit
  • Michael J. Schumacher
  • John Hutter
  • Raymond Nagle
Brief Report


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/μl (×106/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 µmol/l). We present a 4-year-old AA boy with KD, NS, relapsing thrombocytopenia, and renal damage with anti-TBM antibody.


African-American Nephrotic syndrome Thrombocytopenia Asthma Tubulo-interstitial nephritis 


  1. 1.
    Rajpoot DK, Pahl M, Clark J (2000) Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 14:486–488CrossRefPubMedGoogle Scholar
  2. 2.
    Chartapisak W, Opastirakul S (2002) Steroid-resistant nephrotic syndrome associated with Kimura’s disease. Am J Nephrol 22:381–384CrossRefPubMedGoogle Scholar
  3. 3.
    Romao JE, Saldanha LB, Ianez LE, Sabbaga E (1998) Recurrence of focal segmental glomerulosclerosis associated with Kimura’s disease after kidney transplantation. Am J Kidney Dis 31:E3Google Scholar
  4. 4.
    Whelan TV, Maher JF, Kragel P, Dysart N, Dannenhoffer R, Prager L (1988) Nephrotic syndrome associated with Kimura’s disease. Am J Kidney Dis 11:353–356PubMedGoogle Scholar
  5. 5.
    Atar S, Oberman AS, Ben-Izhak O, Flatau E (1994) Recurrent nephrotic syndrome associated with Kimura’s disease in a young non-Oriental male. Nephron 68:259–261PubMedGoogle Scholar
  6. 6.
    Daaleman TP, Woodroof J (2000) Kimura’s disease presenting as subcutaneous facial plaque in an African American. Cutis 66:201–204PubMedGoogle Scholar
  7. 7.
    Hung CC, Liao PL, Chang CT, Wu MS, Huang CC (2000) Steroid-sensitive anemia in a boy on dialysis—an association with Kimura disease. Pediatr Nephrol 15:183–185CrossRefPubMedGoogle Scholar
  8. 8.
    Lee CT, Huang CC, Lam KK, Chen JB (2001) Kimura’s disease in a chronic hemodialysis patient. Am J Nephrol 21:47–50CrossRefPubMedGoogle Scholar
  9. 9.
    Qunibi WY, Al-Sibai MB, Akhtar M (1988) Mesangio-proliferative glomerulonephritis associated with Kimura’s disease. Clin Nephrol 30:111–114PubMedGoogle Scholar
  10. 10.
    Kuo TT, Shih LY, Chan HL (1988) Kimura’s disease. Involvement of regional lymph nodes and distinction from angiolymphoid hyperplasia with eosinophilia. Am J Surg Pathol 12:843–854PubMedGoogle Scholar
  11. 11.
    Agus D, Mann R, Clayman M, Kelly C, Michaud L, Cohn D, Neilson EG (1986) The effects of daily cyclophosphamide administration on the development and extent of primary experimental interstitial nephritis in rats. Kidney Int 29:635–640PubMedGoogle Scholar
  12. 12.
    Makker SP, Widstrom R, Huang J (1996) Membranous nephropathy, interstitial nephritis, and Fanconi syndrome—glomerular antigen. Pediatr Nephrol 10:7–13PubMedGoogle Scholar
  13. 13.
    Miyazato H, Yoshioka K, Hino S, Aya N, Matsuo S, Suzuki N, Suzuki Y, Sinohara H, Maki S (1994) The target antigen of anti-tubular basement membrane antibody-mediated interstitial nephritis. Autoimmunity 18:259–265PubMedGoogle Scholar
  14. 14.
    Khoo BP, Chan R (2002) Kimura disease: 2 case reports and a literature review. Cutis 70:57–61PubMedGoogle Scholar
  15. 15.
    Umemoto S, Itagaki K, Kimura M, Itoh S, Tanaka M, Haraguchi M, Matsuzaki M (2003) Effect of suplatast tosilate, an antiallergic selective Th2 cytokine inhibitor, on acute eosinophilic myocarditis: a case report. Heart Vessels 18:100–102CrossRefPubMedGoogle Scholar

Copyright information

© IPNA 2004

Authors and Affiliations

  • Mehul P. Dixit
    • 1
    • 3
    Email author
  • Katherine M. Scott
    • 2
  • Erika Bracamonte
    • 1
  • Naznin M. Dixit
    • 1
  • Michael J. Schumacher
    • 1
  • John Hutter
    • 1
  • Raymond Nagle
    • 2
  1. 1.Department of Pediatrics, Steele Memorial Research CenterUniversity of ArizonaTucsonUSA
  2. 2.Department of Pathology, Steele Memorial Research CenterUniversity of ArizonaTucsonUSA
  3. 3.Department of Pediatrics, AHSCUniversity of ArizonaTucsonUSA

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