소아 Henoch-Schönlein 자반증에서의 항호중구 항체
Antineutrophil Cytoplasmic Antibody in Korean Children with Henoch-Schönlein Purpura
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목 적:Henoch-Schönlein 자반증에서 항호중구 항체에 대한 외국의 여러 논문이 있었으나 이들은 대조적인 연구결과를 보였고, 국내에서는 Henoch-Schönlein 자반증 환아를 대상으로 한 항호중구 항체의 연구가 없었다. 따라서 한국 소아 Henoch-Schönlein 자반증에서 항호중구 항체의 임상적인 의의를 알아보고자 본 연구를 시행하였다. 방 법:2007년 5월부터 2008년 8월까지 신촌 세브란스병원 소아과에 내원하여 Henoch- Schönlein 자반증으로 진단받은 30명의 환아들을 대상으로 의무기록을 후향적으로 조사하여 분석하였고, 일반적인 특징으로 나이, 성별, 자반증 등의 증상등을 조사하였다. 검사 소견으로는 백혈구수, 혈소판수, 혈침 속도, Anti-streptolysin O titer, 대변 잠혈 검사, 혈청 면역 글로불린(IgG, IgA, IgM), 혈청 보체(C3, C4), 항핵 항체, 항호중구 항체(c-ANCA, p-ANCA)를 조사하였다. 결 과:총 30명의 환아중 남자가 13명, 여자가 17명이었고 평균 연령은 6.0±1.9세(범위: 5-12세)였다. 자반은 모든 환아(100%)에서 관찰되었고, 복통은 20 명(67%), 관절통은 17명(57%), 신침범은 11명(37%)에서 관찰되었다. Anti-streptolysin O titer는 검사한 환아 15명 중 1명(7%)에서 증가된 소견을 보었고, 혈청 IgA 수치는 모든 환아에서 증가되지 않았다. 간접면역형광법으로 시행한 항호중구 항체 검사에는 오직 한 명(3%)에서만 p-ANCA가 약양성 소견을 보였으나 이는 추적 검사시 음성으로 전환되었고 나머지 환아에서는 c-ANCA, p-ANCA가 모두 음성 소견을 보였다. 결 론:항호중구 항체는 소아 Henoch-Schönlein 자반증에서 진단적 혹은 면역학적으로 특이한 검사소견은 아닌 것으로 보이며, 이는 항호중구 항체가 소아 Henoch-Schönlein 자반증의 병인과 연관성이 없음을 암시한다.
Abstract
Purpose : There have been some reports on the prevalence of positive antineutrophil cytoplasmic antibody(ANCA) in Henoch-Schönlein purpura(HSP), but the results were conflicting. We performed this study to evaluate the clinical significance of ANCA(c-ANCA and p- ANCA) in Korean children with HSP. Methods : The medical records of 30 patients(13 boys and 17 girls) aged 6.0±1.9(5-12) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2008, the sera from children with acute HSP were tested for antineutrophil cytoplasmic antibodies(ANCA). The target antigens of these autoantibodies are proteinase 3(c-ANCA) or myeloperoxidase(p-ANCA). Results : Palpable purpura was seen in all 30 patients(100%), abdominal pain in 20(67%), arthralgia in 17(57%), and renal involvement in 11(37%). Laboratory findings showed leukocytosis in 4 patients(13%), thrombocytosis 18 in(60%), and elevated erythrocyte sedimentation rate in 18(60%). Anti-streptolysin O titers were elevated in 7% of the patients and no patient showed elevation of serum IgA level. The sera from 29 patients were negative for c-ANCA and p-ANCA by indirect immunofluorescence, but only one patient had weakly positive results, which became negative at follow-up. Conclusions : We conclude that c-ANCA or p-ANCA is not an important serologic marker in children with HSP, because it was neither diagnostically nor immunologically specific in children with HSP. These results suggest that ANCA are not involved in the pathogenesis of HSP in children.
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