Document Detail


The clinical features of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis in Chinese children.
MedLine Citation:
PMID:  16491416     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis is reported mainly in adults. Studies in children are limited. The current study retrospectively analyzed the clinical characteristics and pathology of ANCA-associated systemic vasculitis in children in our hospital during the past 7 years. Twenty-four pediatric patients were diagnosed as having ANCA-associated systemic vasculitis, including 19 patients with microscopic polyangiitis (MPA), one with Wegener's granulomatosis (WG), three with propylthiouracil (PTU)-induced ANCA-positive vasculitis and one with anti-glomerular basement membrane (GBM) disease. Of patients with primary ANCA-associated systemic vasculitis (MPA and WG), with an average age of 10.8+/-2.8 (6-14) years, 18 patients (90%) were female and two (10%) were male. Nineteen patients (95%) were p-ANCA/MPO-ANCA positive and one (5%) was c-ANCA/PR3-ANCA positive. The interval between onset and diagnosis was 8.5+/-24.3 (0.2-108) months. The majority of the patients (85%) had multi-organ involvement. All patients had clinical evidence of renal involvement and presented with hematuria and proteinuria. Of 20 patients, 16 (80%) also had acute renal failure, and five patients were dialysis dependent. Nine patients underwent renal biopsy and were diagnosed with necrotizing and crescentic glomerulonephritis. However, six biopsies showed immune complex deposition. All patients received immunosuppressive therapy including prednisone and cyclophosphamide, and ten patients also received intravenous administration of methylprednisone pulse therapy according to their clinical situation and renal pathology. Sixteen patients achieved clinical remission, and four patients presented as treatment failure. Patients were followed up for 12.3+/-5.1 months (median 12 months; range 1 to 91 months). Ten patients maintained their clinical remission, and ten progressed to renal failure requiring dialysis. Our study showed that the clinical features and pathology of primary ANCA-associated systemic vasculitis in children were similar to those of adults, but there were a predominance of female patients and late diagnoses. We suggest that early recognition and prompt aggressive treatment might improve outcome.
Authors:
Feng Yu; Jian-Ping Huang; Wan-Zhong Zou; Ming-Hui Zhao
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Publication Detail:
Type:  Journal Article     Date:  2006-02-21
Journal Detail:
Title:  Pediatric nephrology (Berlin, Germany)     Volume:  21     ISSN:  0931-041X     ISO Abbreviation:  Pediatr. Nephrol.     Publication Date:  2006 Apr 
Date Detail:
Created Date:  2006-03-29     Completed Date:  2006-10-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8708728     Medline TA:  Pediatr Nephrol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  497-502     Citation Subset:  IM    
Affiliation:
Department of Nephrology, Peking University First Hospital, Beijing, People's Republic of China.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Antibodies, Antineutrophil Cytoplasmic / blood*
Child
China
Female
Humans
Male
Retrospective Studies
Vasculitis / diagnosis*,  immunology*
Chemical
Reg. No./Substance:
0/Antibodies, Antineutrophil Cytoplasmic

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