Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
권호 표지

소아에서 전신성 낭창의 임상적 고찰

A clinical study of systemic lupus erythematosus in children

  • 김지태 (연세대학교 의과대학 소아과학교실) ;
  • 남영미 (연세대학교 의과대학 소아과학교실) ;
  • 이재승 (연세대학교 의과대학 소아과학교실) ;
  • 김동수 (연세대학교 의과대학 소아과학교실)
  • Kim, Ji Tae (Department of Pediatrics, Severance Children's Hospital, Yonsei University Medical Center) ;
  • Nam, Young Mee (Department of Pediatrics, Severance Children's Hospital, Yonsei University Medical Center) ;
  • Lee, Jae Seung (Department of Pediatrics, Severance Children's Hospital, Yonsei University Medical Center) ;
  • Kim, Dong Soo (Department of Pediatrics, Severance Children's Hospital, Yonsei University Medical Center)
  • 투고 : 2006.10.13
  • 심사 : 2006.12.19
  • 발행 : 2007.01.15
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초록

목 적 : 전신성 홍반성 낭창(systemic lupus erythematosus, SLE)은 장기간에 걸쳐 다양한 임상경과를 나타내며 여러장기를 침범하는 자가 면역성 질환으로 유전적, 환경적, 면역학적 요인 등에 의한 면역 조절계의 이상으로 생각되고 있다. 소아에서는 갑작스럽게 발병하며, 경과가 빠르게 악화되며 여러 장기를 동시에 침범한다. 따라서 소아에서 조기 진단하여 조기 치료하는 것이 SLE의 예후 개선에 더욱 중요한 점으로 생각된다. 이에 저자들은 소아에서 발생한 SLE 환아의 임상 양상, 검사 소견, 예후에 대해 전반적으로 고찰하여 질환의 진단 및 예후의 향상을 기대하고자 한다. 방 법 : 1996년 1월부터 2005년 12월까지 10년간 신촌 세브란스 병원 소아과에서 전신성 홍반성 낭창으로 진단받은 45례의 환아를 대상으로 의무기록을 후향적으로 고찰하였다. 결 과 : 진단 당시 평균발병 연령은 $10.8{\pm}3.8$(0-15)세였으며 10-15세 사이에서 호발(68.9%)했으며 환아의 남녀비는 1:4였다. 진단시 초기증상으로 안면 부종(51.1%), 나비모양 홍반(44.4%), 발열(28.9%), 관절통(15.6%), 빈혈(13.3%) 및 광과민성(11.1%)순 이였다. SLE의 ARA 진단 기준중 항목별 양성률은 형광항핵항체(97.8%), 항ds DNA항체(82.2%), 루프스 신염(71.1%), 나비형 홍반(71.1%) 및 혈액학적 이상(66.7%)의 순이었다. 진단 당시 루프스 신염이 동반 되었던 경우는 45례 중 23례(51.1%)였으며 치료 중 8례가 루프스 신염으로 새로 진단되어 그 빈도가 71.1%로 조사되었고 신생검상 WHO class IV 병변인 미만성 증식성 사구체 신염(43.8%)이 가장 많았다. 2례는 완치되었으며, 2례 모두 신생아 루프스였다. 우리나라 아동에서 외국에 비하여 발열, 광과민성, 입궤양, 관절통, 늑막염은 적게 관찰되는 양상을 보였다. 결 론 : 소아에서 전신성 홍반성 낭창의 임상 양상과 예후는 다양하다. 장기적인 추적 관찰이 필요하며 예후의 향상을 위해 조기 진단과 적극적인 치료가 중요하며, 향후 이 질환에 대한 심도 있는 연구가 지속되어야 할 것이다.

Purpose : Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease with complex clinical manifestations. It probably involves genetic, environmental and immunologic factors. In this study, we investigated the clinical manifestations, laboratory findings and prognosis of pediatric SLE to aid clinical care of pediatric SLE. Methods : The data of 45 patients who were diagnosed as pediatric SLE in Severance Children's Hospital from Jan. 1996 to Dec. 2005 were analysed retrospectively. Results : The mean age at diagnosis was 10.8 (0-15) years old. And the ratio of male to female patients was 1:4. The initial manifestations were facial edema (51.1 percent), malar rash (44.4 percent), and fever (28.9 percent). The ANA (97.8 percent), anti-ds DNA antibody (82.2 percent), lupus nephritis (71.1 percent), malar rash (71.1 percent), and cytopenia (66.7 percent) were the most common findings among the classification criteria by ACR (American College of Rhematology, 1997). Conclusion : Clinical manifestations and prognosis are various in pediatric SLE. Intensive studies of SLE in children should be continued for more effective treatment.

키워드

참고문헌

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