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Case report: A pediatric case of MPO-ANCA-associated granulomatosis with polyangiitis superimposed on post-streptococcal acute glomerulonephritis

An eight-year-old girl was admitted with vomiting, gross hematuria, and progressive renal dysfunction. A renal biopsy revealed endocapillary proliferative glomerulopathy and crescent formation. Immunofluorescence staining revealed diffuse granular deposits of IgG and C3. Post-streptococcal acute glo...

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Autores principales: Kuang, Qianhuining, He, Xu, Jia, Lili, Zhang, Zhiqiang, Gui, Chunhong, Gao, Chunlin, Xia, Zhengkun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10364118/
https://www.ncbi.nlm.nih.gov/pubmed/37492611
http://dx.doi.org/10.3389/fped.2023.1148132
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author Kuang, Qianhuining
He, Xu
Jia, Lili
Zhang, Zhiqiang
Gui, Chunhong
Gao, Chunlin
Xia, Zhengkun
author_facet Kuang, Qianhuining
He, Xu
Jia, Lili
Zhang, Zhiqiang
Gui, Chunhong
Gao, Chunlin
Xia, Zhengkun
author_sort Kuang, Qianhuining
collection PubMed
description An eight-year-old girl was admitted with vomiting, gross hematuria, and progressive renal dysfunction. A renal biopsy revealed endocapillary proliferative glomerulopathy and crescent formation. Immunofluorescence staining revealed diffuse granular deposits of IgG and C3. Post-streptococcal acute glomerulonephritis (PSAGN) was suspected, based on the elevated anti-streptolysin O levels, decreased serum C3 concentrations, and histologic findings. The myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) test was positive, and the young patient gradually developed palisaded neutrophilic and granulomatous dermatitis (PNGD), orbital and paranasal sinus granulomatous neoplasms, along with intermittent nose, head, and orbital pain. Finally, she was diagnosed with the rare MPO-ANCA-associated granulomatosis with polyangiitis (GPA) superimposed on PSAGN. The patient was treated with aggressive renal replacement therapy, methylprednisolone pulse therapy, and intravenous pulse cyclophosphamide; her renal function normalized, and her pain symptoms improved.
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spelling pubmed-103641182023-07-25 Case report: A pediatric case of MPO-ANCA-associated granulomatosis with polyangiitis superimposed on post-streptococcal acute glomerulonephritis Kuang, Qianhuining He, Xu Jia, Lili Zhang, Zhiqiang Gui, Chunhong Gao, Chunlin Xia, Zhengkun Front Pediatr Pediatrics An eight-year-old girl was admitted with vomiting, gross hematuria, and progressive renal dysfunction. A renal biopsy revealed endocapillary proliferative glomerulopathy and crescent formation. Immunofluorescence staining revealed diffuse granular deposits of IgG and C3. Post-streptococcal acute glomerulonephritis (PSAGN) was suspected, based on the elevated anti-streptolysin O levels, decreased serum C3 concentrations, and histologic findings. The myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) test was positive, and the young patient gradually developed palisaded neutrophilic and granulomatous dermatitis (PNGD), orbital and paranasal sinus granulomatous neoplasms, along with intermittent nose, head, and orbital pain. Finally, she was diagnosed with the rare MPO-ANCA-associated granulomatosis with polyangiitis (GPA) superimposed on PSAGN. The patient was treated with aggressive renal replacement therapy, methylprednisolone pulse therapy, and intravenous pulse cyclophosphamide; her renal function normalized, and her pain symptoms improved. Frontiers Media S.A. 2023-07-10 /pmc/articles/PMC10364118/ /pubmed/37492611 http://dx.doi.org/10.3389/fped.2023.1148132 Text en © 2023 Kuang, He, Jia, Zhang, Gui, Gao and Xia. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) (https://creativecommons.org/licenses/by/4.0/) . The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Pediatrics
Kuang, Qianhuining
He, Xu
Jia, Lili
Zhang, Zhiqiang
Gui, Chunhong
Gao, Chunlin
Xia, Zhengkun
Case report: A pediatric case of MPO-ANCA-associated granulomatosis with polyangiitis superimposed on post-streptococcal acute glomerulonephritis
title Case report: A pediatric case of MPO-ANCA-associated granulomatosis with polyangiitis superimposed on post-streptococcal acute glomerulonephritis
title_full Case report: A pediatric case of MPO-ANCA-associated granulomatosis with polyangiitis superimposed on post-streptococcal acute glomerulonephritis
title_fullStr Case report: A pediatric case of MPO-ANCA-associated granulomatosis with polyangiitis superimposed on post-streptococcal acute glomerulonephritis
title_full_unstemmed Case report: A pediatric case of MPO-ANCA-associated granulomatosis with polyangiitis superimposed on post-streptococcal acute glomerulonephritis
title_short Case report: A pediatric case of MPO-ANCA-associated granulomatosis with polyangiitis superimposed on post-streptococcal acute glomerulonephritis
title_sort case report: a pediatric case of mpo-anca-associated granulomatosis with polyangiitis superimposed on post-streptococcal acute glomerulonephritis
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10364118/
https://www.ncbi.nlm.nih.gov/pubmed/37492611
http://dx.doi.org/10.3389/fped.2023.1148132
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