Two Female Siblings with Childhood-Onset Systemic Lupus Erythematosus

  • SANO Fumie
    Department of Pediatrics, Yokohama City University School of Medicine
  • OZAWA Remi
    Department of Pediatrics, Yokohama City University School of Medicine
  • MACHIDA Hiroyuki
    Department of Pediatrics, Yokohama City University School of Medicine
  • MIYAMAE Takako
    Department of Pediatrics, Yokohama City University School of Medicine
  • ITO Shuichi
    Department of Pediatrics, Yokohama City University School of Medicine
  • IMAGAWA Tomoyuki
    Department of Pediatrics, Yokohama City University School of Medicine
  • MORI Masaaki
    Department of Pediatrics, Yokohama City University School of Medicine
  • OKUYAMA Kenichi
    Department of Pediatrics, Kanagawa Prefectural Ashigara Kami Hospital
  • YOKOTA Shumpei
    Department of Pediatrics, Yokohama City University School of Medicine

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Other Title
  • 小児期発症全身性エリテマトーデスの姉妹例

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Abstract

  We herein report two female siblings with childhood-onset Systemic Lupus Erythematosus (SLE) who developed membranous lupus nephritis. The children were diagnosed as having SLE in reverse birth order at ages 11 and 14 years. Younger sister's initial symptom was edema and laboratory findings indicated proteinurea, hypocomplementemia and positive ANA/anti-dsDNA antibody. She was diagnosed as being SLE with membranous lupus nephritis based on International Society of Nephrology/Renal Pathology Society (ISN/RPS) 2003 classification. Elder sister manifested general fatigue and edema twelve months after her sister. Laboratory findings showed proteinurea, hypocomplementemia, and positive ANA/anti-dsDNA antibody. A renal biopsy revealed mixed form of mesangial proliferative glomerulonephritis and membranous nephritis. Moreover, both of them were complicated with secondary Sjögren's syndrome. HLA typing was performed and the siblings were noted to have the same haplotype; A*0207, A*2402, B*4601, B*5201, B*5201, Cw*0102, Cw*1202, DRB1*0101, DRB1*0803.<br>

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