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Journal of the Korean Society of Pediatric Nephrology 2008;12(2): 164-169. doi: https://doi.org/10.3339/jkspn.2008.12.2.164
소아 Henoch-$Sch{ddot{o}}nlein$ 자반증에서의 항호중구 항체
최지나, 신재일, 이재승, 김현숙
1연세대학교 의과대학 소아과학교실, 세브란스 어린이 병원 소아과
2연세대학교 의과대학 소아과학교실, 세브란스 어린이 병원 소아과
3연세대학교 의과대학 소아과학교실, 세브란스 어린이 병원 소아과
4연세대학교 의과대학 신장질환 연구소, 진단검사의학교실
Antineutrophil Cytoplasmic Antibody in Korean Children with Henoch-$Sch{ddot{o}}nlein$ Purpura
Ji-Na Choi, Jae-Il Shin, Jae-Seung Lee, Hyon-Suk Kim
1The Institute of Kidney Disease, Severance Childrens Hospital, Department of Pediatrics, Yonsei University College of Medicine
2The Institute of Kidney Disease, Severance Childrens Hospital, Department of Pediatrics, Yonsei University College of Medicine
3The Institute of Kidney Disease, Severance Childrens Hospital, Department of Pediatrics, Yonsei University College of Medicine
4The Institute of Kidney Disease, Severance Childrens Hospital, Department of Laboratory Medicine, Yonsei University College of Medicine
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ABSTRACT
Purpose : There have been some reports on the prevalence of positive antineutrophil cytoplasmic antibody(ANCA) in Henoch-$Sch{ddot{o}}nlein$ 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$pm$1.9(5-12) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2008, the sera from children with acute HSP were tested for antineutrophil cytoplasmic antibodies(ANCA). The target antigens of these autoantibodies are proteinase 3(c-ANCA) or myeloperoxidase(p-ANCA).
Results : Palpable purpura was seen in all 30 patients(100%), abdominal pain in 20(67%), arthralgia in 17(57%), and renal involvement in 11(37%). Laboratory findings showed leukocytosis in 4 patients(13%), thrombocytosis 18 in(60%), and elevated erythrocyte sedimentation rate in 18(60%). Anti-streptolysin O titers were elevated in 7% of the patients and no patient showed elevation of serum IgA level. The sera from 29 patients were negative for c-ANCA and p-ANCA by indirect immunofluorescence, but only one patient had weakly positive results, which became negative at follow-up.
Conclusions : We conclude that c-ANCA or p-ANCA is not an important serologic marker in children with HSP, because it was neither diagnostically nor immunologically specific in children with HSP. These results suggest that ANCA are not involved in the pathogenesis of HSP in children.
Key words: Henoch-$Sch{\ddot{o}}nlein$ purpura | Antineutrophil cytoplasmic antibodies | Proteinase 3 | Myeloperoxidase
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