Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a small- and medium-sized vasculitis classically seen in adult patients, with peak onset near the fifth to seventh decade of life. There is little data on ANCA-associated vasculitis in pediatric patients, and most studies have limited follow-up.



This is a retrospective chart review of 22 patients with ANCA-positive glomerulonephritis in a single institution from 1991 to 2013.



Of the 22 patients, eight (36 %) required renal replacement therapy (RRT) at diagnosis; four of these patients recovered sufficient renal function to initially discontinue dialysis. Five patients (23 %) were treated with plasmapheresis at presentation. The median time from presentation until first clinical or serologic relapse was 1.7 ± 1.2 years. After a median follow-up of 5.8 years, just over half of our patients had chronic kidney disease (CKD) stages 1-3 (55 %). Seven (32 %) patients progressed to end-stage renal disease (ESRD) and eventually required kidney transplant.



ANCA-associated glomerulonephritis is a rare disorder in children. Presentation and outcomes vary significantly among patients. More research is required to follow these patients who are diagnosed in childhood to further characterize the long-term outcome of the disease.
Department of Pediatric Nephrology, Indiana University School of Medicine, 699 Riley Hospital Drive, Rm 230, Indianapolis, IN, 46202, USA. akouri@iupui.edu.
Anne M
AM
Kouri
Department of Pediatric Nephrology, Indiana University School of Medicine, 699 Riley Hospital Drive, Rm 230, Indianapolis, IN, 46202, USA.
Sharon P
SP
Andreoli
D000293:Adolescent; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000276:immunology / Q000628:therapy*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D002648:Child; D003956:Dialysis; D018450:Disease Progression; D005260:Female; D005921:Glomerulonephritis / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000601:surgery; D016030:Kidney Transplantation; D008297:Male; D010956:Plasmapheresis; D012008:Recurrence; D051436:Renal Insufficiency, Chronic / Q000209:etiology; D017582:Renal Replacement Therapy; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome
D019268:Antibodies, Antineutrophil Cytoplasmic
ANCA-positive glomerulonephritis; Children; Granulomatosis with polyangiitis; Microscopic polyangiitis; Pediatric glomerulonephritis; Rapidly progressive glomerulonephritis
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