Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis

Anne M. Kouri, Sharon Andreoli

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

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

Original languageEnglish (US)
Pages (from-to)1-7
Number of pages7
JournalPediatric Nephrology
DOIs
StateAccepted/In press - Sep 27 2016

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Antineutrophil Cytoplasmic Antibodies
Glomerulonephritis
Pediatrics
Vasculitis
Kidney
Renal Replacement Therapy
Plasmapheresis
Chronic Renal Insufficiency
Chronic Kidney Failure
Dialysis
Transplants
Recurrence

Keywords

  • ANCA-positive glomerulonephritis
  • Children
  • Granulomatosis with polyangiitis
  • Microscopic polyangiitis
  • Pediatric glomerulonephritis
  • Rapidly progressive glomerulonephritis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology

Cite this

Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis. / Kouri, Anne M.; Andreoli, Sharon.

In: Pediatric Nephrology, 27.09.2016, p. 1-7.

Research output: Contribution to journalArticle

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