Back to Journals » International Journal of Nephrology and Renovascular Disease » Volume 7

Granulomatosis with polyangiitis: rapidly progressive necrotizing glomerulonephritis in a pediatric patient

Authors Luna M, Bocanegra V, Vallés P

Received 6 November 2013

Accepted for publication 2 December 2013

Published 23 April 2014 Volume 2014:7 Pages 153—156

DOI https://doi.org/10.2147/IJNRD.S57109

Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 3


Mariana Luna,1 Victoria Bocanegra,3 Patricia G Vallés1,2

1
Nephrology Division, Pediatric Department, Dr Humberto Notti Pediatric Hospital, Mendoza, Argentina; 2Pathophysiology Area, Pathology Department, School of Medicine, National Cuyo University, Mendoza, Argentina; 3National Council of Scientific and Technical Research of Argentina (CONICET), Buenos Aires, Argentina

Abstract: Granulomatosis with polyangiitis (GPA) is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffered from pharyngitis 1 week before admittance to hospital. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome. Renal failure with rapidly progressive glomerulonephritis occurred within 24 hours. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies (c-ANCA with antiproteinase 3 specificity) and renal biopsy revealed pauci-immune crescentic focal necrotizing glomerulonephritis. Treatment including methylprednisolone and cyclophosphamide intravenous pulses allowed renal recovery after 3 weeks. The clinical, hematological, and biochemical parameters improved substantially, achieving remission. Granulomatosis with polyangiitis, although rare in children, should be considered in the above clinical scenario. This case underlines that knowledge of renal histology diagnosis and early aggressive immunosuppressive therapy are essential for the management of these patients.

Keywords: acute renal failure, vasculitis, crescentic pauci-immune glomerulonephritis-Type-C, antineutrophil cytoplasmic antibodies (c-ANCA)-macroscopic hematuria

Creative Commons License This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.

Download Article [PDF]  View Full Text [HTML][Machine readable]