The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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Systemic Lupus Erythematosus Preceded by Distal Renal Tubular Acidosis

원위신세관산증이 선행한 전신홍반루푸스

  • Kang, Jin-Young (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Kang, Jong-Wan (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Kim, Ji-Hun (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Jin, Mi-Kyung (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Im, Churl-Hyun (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Nam, Eon-Jeong (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Kang, Young-Mo (Department of Internal Medicine, Kyungpook National University School of Medicine)
  • 강진영 (경북대학교 의학전문대학원 내과학교실) ;
  • 강종완 (경북대학교 의학전문대학원 내과학교실) ;
  • 김지훈 (경북대학교 의학전문대학원 내과학교실) ;
  • 진미경 (경북대학교 의학전문대학원 내과학교실) ;
  • 임철현 (경북대학교 의학전문대학원 내과학교실) ;
  • 남언정 (경북대학교 의학전문대학원 내과학교실) ;
  • 강영모 (경북대학교 의학전문대학원 내과학교실)
  • Published : 2011.10.01
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Abstract

Distal renal tubular acidosis (RTA) is characterized by a decreased net H+ secretion in the collecting tubules, which results in a failure of urine acidification and results in metabolic acidosis, hypokalemia, and nephrocalcinosis. The acquired form of distal RTA is associated with tubulointerstitial involvement of immune-mediated disorders such as Sjogren's syndrome and systemic lupus erythematosus (SLE). Only a few case reports have indicated that distal RTA precedes SLE by months to years. We present a 39-year-old woman who had manifestations of distal RTA for 21 years before the development of overt symptoms of SLE.

원위신세관산증은 세관내 산 분비의 저하로 소변의 산성화 장애와 전신적인 산혈증 및 저칼륨혈증을 특징으로 하는 질환이다. 루푸스를 비롯한 자가면역질환에 동반되는 원위신세관산증은 대부분 자가면역질환 발현 후에 나타나며 원위신세관산증이 먼저 발생하고 자가면역질환이 수개월에서 수년 후 발생하는 경우는 세계적으로 매우 드물게 보고되어 있다. 저자들은 원위신세관산증으로 진단되고 21년 후에 전형적인 루푸스 임상 증상들이 나타난 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Keywords

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