J Korean Soc Pediatr Nephrol.  2008 Oct;12(2):164-169.

Antineutrophil Cytoplasmic Antibody in Korean Children with Henoch-Schonlein Purpura

• Choi JN
• Shin JI
• Lee JS
• Kim HS

Abstract

PURPOSE: There have been some reports on the prevalence of positive antineutrophil cytoplasmic antibody(ANCA) in Henoch-Schonlein 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.
CONCLUSION
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.

Keyword

Henoch-Schonlein purpura; Antineutrophil cytoplasmic antibodies; Proteinase 3; Myeloperoxidase

MeSH Terms

Abdominal Pain
Aged
Antibodies, Antineutrophil Cytoplasmic
Arthralgia
Autoantibodies
Blood Sedimentation
Child
Cytoplasm
Fluorescent Antibody Technique, Indirect
Follow-Up Studies
Humans
Immunoglobulin A
Leukocytosis
Medical Records
Myeloblastin
Peroxidase
Prevalence
Purpura
Purpura, Schoenlein-Henoch
Retrospective Studies
Thrombocytosis
Antibodies, Antineutrophil Cytoplasmic
Autoantibodies
Immunoglobulin A
Myeloblastin
Peroxidase