Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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A Case of "Full-house" Nephropathy in a Non-lupus Patient

병리조직검사에서 "Full-house" 패턴의 면역 복합체 침착이 발견된 비루푸스 신염

  • Yoo, Ha Yeong (Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine) ;
  • Son, Mikyung (Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine) ;
  • Cho, Myung Hyun (Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine) ;
  • Kwak, Byung Ok (Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine) ;
  • Park, Hye Won (Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine) ;
  • Lim, So Dug (Department of Pathology, Konkuk University Medical Center, Konkuk University School of Medicine) ;
  • Chung, Sochung (Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine) ;
  • Kim, Kyo Sun (Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine)
  • 유하영 (건국대학교 의학전문대학원 소아과학교실) ;
  • 손미경 (건국대학교 의학전문대학원 소아과학교실) ;
  • 조명현 (건국대학교 의학전문대학원 소아과학교실) ;
  • 곽병옥 (건국대학교 의학전문대학원 소아과학교실) ;
  • 박혜원 (건국대학교 의학전문대학원 소아과학교실) ;
  • 임소덕 (건국대학교 의학전문대학원 병리학교실) ;
  • 정소정 (건국대학교 의학전문대학원 소아과학교실) ;
  • 김교순 (건국대학교 의학전문대학원 소아과학교실)
  • Received : 2014.09.26
  • Accepted : 2014.10.14
  • Published : 2014.10.31
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Abstract

Histopathologic evidence of "full-house" immune complex deposits is a pathognomonic feature of lupus nephritis. This report presents the case of a 12-year-old boy with persistent microscopic hematuria and proteinuria. He was diagnosed with "full-house" nephropathy based on a renal biopsy. However, there was no other clinical or biological evidence of systemic lupus erythematosus (SLE). Although the potential for isolated "full-house" nephropathy preceding SLE is unclear, such patients should be followed for clinical signs and autoantibodies of SLE. In most cases, microscopic hematuria has a good prognosis, and follow-up usually requires only regular urinalysis. However, we should be aware of isolated "full-house" nephropathy that remains asymptomatic for a long time, as few patients with no clinical signs and negative serology ultimately develop SLE.

"full-house" 면역 복합체 침착은 루푸스 신병증의 진단적인 조직 소견이다. 이 증례 보고에서 12세 남자 환아는 지속적인 현미경적 혈뇨와 단백뇨를 주소로 내원하였다. 그는 신장 조직 검사에서 "full-house" 신병증을 진단받았으나 전신성 홍반성 낭창과 관련한 어떠한 임상적 징후나 혈청학적인 결과를 보이지 않았다. 비록 "full-house" 신병증이 전신성 홍반성 낭창에 선행하는 질병인지에 대해서는 명확히 밝혀진 바는 없으나, 루푸스와 관련된 증상이나 혈청학적인 자가 항체가 출현할 수 있으므로, 추적 관찰을 요한다. 대부분의 현미경적 혈뇨는 좋은 예후를 가지고 있고, 보통은 소변검사를 정기적으로 검사하며 관찰한다. 그러나 이 증례에서 보듯이 소변검사 이상이 발견 되었을 때 오랫동안 무증상으로 잠복하는 "full-house" 신병증 또한 고려되어야 하며, 이는 몇몇의 임상적인 증상 및 혈청학적 소견이 음성이었던 환자가 전신성 홍반성 낭창으로 진행할 수 있기 때문이다.

Keywords

References

  1. Provost TT, Razzaque A, Maddison PJ, Reichlin M. Antibodies to cytoplasmic antigens in lupus erythematosus. serologic marker for systemic disease. Arthritis Rheum 1977;20:1457-63. https://doi.org/10.1002/art.1780200803
  2. Cameron JS. Lupus nephritis. J Am Soc Nephrol 1999;10:413-24.
  3. Von Feldt JM. Systemic lupus erythematosus. recognizing its various presentations. Postgrad Med 1995;97:79, 83, 86 passim.
  4. Contreras G, Roth D, Pardo V, Striker LG, Schultz DR. Lupus nephritis: A clinical review for practicing nephrologists. Clin Nephrol 2002;57:95-107. https://doi.org/10.5414/CNP57095
  5. Berden JH. Lupus nephritis. Kidney Int 1997;52:538-58. https://doi.org/10.1038/ki.1997.365
  6. Caltik A, Demircin G, Bulbul M, Erdogan O, Akyuz SG, Arda N. An unusual case of ANA negative systemic lupus erythematosus presented with vasculitis, long-standing serositis and full-house nephropathy. Rheumatol Int 2013;33:219-22. https://doi.org/10.1007/s00296-010-1540-0
  7. Baskin E, Agras PI, Menekse N, Ozdemir H, Cengiz N. Full-house nephropathy in a patient with negative serology for lupus. Rheumatol Int 2007;27:281-4.
  8. Wen YK, Chen ML. Clinicopathological study of originally non-lupus "full-house" nephropathy. Ren Fail 2010;32:1025-30. https://doi.org/10.3109/0886022X.2010.510614
  9. Cairns SA, Acheson EJ, Corbett CL, Dosa S, Mallick NP, Lawler W, et al. The delayed appearance of an antinuclear factor and the diagnosis of systemic lupus erythematosus in glomerulonephritis. Postgrad Med J 1979;55:723-7. https://doi.org/10.1136/pgmj.55.648.723
  10. Jones E, Magil A. Nonsystemic mesangiopathic glomerulonephritis with "full house" immunofluorescence. pathological and clinical observation in five patients. Am J Clin Pathol 1982;78:29-34.
  11. Adu D, Williams DG, Taube D, Vilches AR, Turner DR, Cameron JS, et al. Late onset systemic lupus erythematosus and lupus-like disease in patients with apparent idiopathic glomerulonephritis. Q J Med 1983;52:471-87.
  12. Enriquez JL, Rajaraman S, Kalia A, Brouhard BH, Travis LB. Isolated antinuclear antibody-negative lupus nephropathy in young children. Child Nephrol Urol 1988;9:340-6.
  13. Gianviti A, Barsotti P, Barbera V, Faraggiana T, Rizzoni G. Delayed onset of systemic lupus erythematosus in patients with "full-house" nephropathy. Pediatr Nephrol 1999;13:683-7. https://doi.org/10.1007/s004670050681
  14. Cameron JS. Lupus nephritis in childhood and adolescence. Pediatr Nephrol 1994;8:230-49. https://doi.org/10.1007/BF00865490