Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature

Subal Kumar Pradhan; Snehamayee Nayak

doi:doi:10.11648/j.ajp.20210701.14

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Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature

Subal Kumar Pradhan, Snehamayee Nayak

Published in American Journal of Pediatrics (Volume 7, Issue 1)

Received: 28 December 2020 Accepted: 14 January 2021 Published: 23 February 2021

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Abstract

‘Double-positive’ with anti-glomerular basement membrane (anti-GBM) antibody and anti-neutrophil cytoplasmic antibody (ANCA) is an extremely rare cause of small vessel vasculitis in children. Studies have reported a distinct hybrid phenotype in double-positive patients which requires an aggressive treatment approach. The data on double positive children are scarce with few case reports available with varied outcomes. A case of 9-year-old girl from India who had double-positive anti-GBM disease is reported here. The patient presented with complaints of edema, oliguria, gross hematuria with rising creatine and was diagnosed as rapidly progressive glomerulonephritis (RPGN). Histological examinations of linear immunoglobulin G deposits along with glomerular capillaries were suggestive of anti-GBM disease. The anti-GBM and p-ANCA antibody titers were also high, and the aforementioned findings led to a diagnosis of ‘double-positive’ anti-GBM with RPGN. She was treated with standard plasma exchange therapy along with pulse methyl prednisolone (3 doses of 30 mg/kg/day on Days 1 to 3), oral prednisolone (1.5 mg/kg on Day 4), single intravenous injection of cyclophosphamide (500 mg/m² on Day 5) and mycophenolate mofetil (MMF; 1000 mg/m² on Day 6) followed by maintenance treatment with oral prednisolone (1 mg/kg/day) and MMF (800 mg/m²/day) from Day 7 onwards. Overall, the anti-GBM and p-ANCA levels declined throughout the treatment period but the patient progressed towards end stage renal disease.

Published in	American Journal of Pediatrics (Volume 7, Issue 1)
DOI	10.11648/j.ajp.20210701.14
Page(s)	14-18
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Anti-GBM, p-ANCA, Double-Positive, Small Vessel Vasculitis, Children

References

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[2]	Canney M and Little MA (2017) ANCA in anti-GBM disease: moving beyond a one-dimensional clinical phenotype. Kidney International 92: 544-6.
[3]	McAdoo SP, Tanna A, Hrušková Z, Holm L, Weiner M, Arulkumaran N, Kang A, Satrapová V, Levy J, Ohlsson S, Tesar V, Segelmark M and Pusey CD (2017) Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients. Kidney international 92: 693-702.
[4]	J DEZ, Taylor D, Thein H and Yehia M (2011) Incidence and features of dual anti-GBM-positive and ANCA-positive patients. Nephrology (Carlton) 16: 725-9.
[5]	Jain R, Dgheim H and Bomback AS (2018) Rituximab for Anti-Glomerular Basement Membrane Disease. Kidney international reports 4: 614-8.
[6]	Nasr SH, Collins AB, Alexander MP, Schraith DF, Herrera Hernandez L, Fidler ME, Sethi S, Leung N, Fervenza FC and Cornell LD (2016) The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis. Kidney Int 89: 897-908.
[7]	Levy JB, Hammad T, Coulthart A, Dougan T and Pusey CD (2004) Clinical features and outcome of patients with both ANCA and anti-GBM antibodies. Kidney Int 66: 1535-40.
[8]	Segelmark M, Hellmark T and Wieslander J (2003) The prognostic significance in Goodpasture's disease of specificity, titre and affinity of anti-glomerular-basement-membrane antibodies. Nephron Clin Pract 94:c59-68.
[9]	Bogdanović R, Minić P, Marković-Lipkovski J, Stajić N, Savić N and Rodić M (2013) Pulmonary renal syndrome in a child with coexistence of anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane disease: case report and literature review. BMC nephrology 14: 66.
[10]	Paueksakon P, Hunley TE, Lee SM and Fogo AB (1999) A 12-year-old girl with pulmonary hemorrhage, skin lesions, and hematuria. Am J Kidney Dis 33: 404-9.
[11]	Hijosa MM, Román LE, Camblor CF, García ML, Salcedo DP and Torres MN (2005) Anti-GBM and anti-MPO antibodies coexist in a case of pulmonary renal syndrome. Pediatr Nephrol 20: 807-10.
[12]	Naidoo S and Waller S (2009) Anti-GBM antibodies co-exist with MPO-ANCA in a 4-year-old girl with acute renal failure. Pediatr Nephrol 24: 215-6.
[13]	Williamson SR, Phillips CL, Andreoli SP and Nailescu C (2011) A 25-year experience with pediatric anti-glomerular basement membrane disease. Pediatr Nephrol 26: 85-91.
[14]	Xie L-j, Cui Z, Jia X-y, Chen Z, Liu X-r and Zhao M-h (2015) Coexistence of Anti-Glomerular Basement Membrane Antibodies and Anti-Neutrophil Cytoplasmic Antibodies in a Child With Human Leukocyte Antigen Susceptibility and Detailed Antibody Description: A Case Report. Medicine 94:e1179-e.
[15]	Timmermans SAMEG, van Dam MJCM, Vink E, Horuz FAPT, van Paassen P and Rosias PPR (2019) Rituximab for the Treatment of Pediatric Double-Positive Small-Vessel Vasculitis. Kidney international reports 5: 235-8.

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APA Style

Subal Kumar Pradhan, Snehamayee Nayak. (2021). Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature. American Journal of Pediatrics, 7(1), 14-18. https://doi.org/10.11648/j.ajp.20210701.14

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ACS Style

Subal Kumar Pradhan; Snehamayee Nayak. Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature. Am. J. Pediatr. 2021, 7(1), 14-18. doi: 10.11648/j.ajp.20210701.14

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AMA Style

Subal Kumar Pradhan, Snehamayee Nayak. Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature. Am J Pediatr. 2021;7(1):14-18. doi: 10.11648/j.ajp.20210701.14

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@article{10.11648/j.ajp.20210701.14,
  author = {Subal Kumar Pradhan and Snehamayee Nayak},
  title = {Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature},
  journal = {American Journal of Pediatrics},
  volume = {7},
  number = {1},
  pages = {14-18},
  doi = {10.11648/j.ajp.20210701.14},
  url = {https://doi.org/10.11648/j.ajp.20210701.14},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajp.20210701.14},
  abstract = {‘Double-positive’ with anti-glomerular basement membrane (anti-GBM) antibody and anti-neutrophil cytoplasmic antibody (ANCA) is an extremely rare cause of small vessel vasculitis in children. Studies have reported a distinct hybrid phenotype in double-positive patients which requires an aggressive treatment approach. The data on double positive children are scarce with few case reports available with varied outcomes. A case of 9-year-old girl from India who had double-positive anti-GBM disease is reported here. The patient presented with complaints of edema, oliguria, gross hematuria with rising creatine and was diagnosed as rapidly progressive glomerulonephritis (RPGN). Histological examinations of linear immunoglobulin G deposits along with glomerular capillaries were suggestive of anti-GBM disease. The anti-GBM and p-ANCA antibody titers were also high, and the aforementioned findings led to a diagnosis of ‘double-positive’ anti-GBM with RPGN. She was treated with standard plasma exchange therapy along with pulse methyl prednisolone (3 doses of 30 mg/kg/day on Days 1 to 3), oral prednisolone (1.5 mg/kg on Day 4), single intravenous injection of cyclophosphamide (500 mg/m2 on Day 5) and mycophenolate mofetil (MMF; 1000 mg/m2 on Day 6) followed by maintenance treatment with oral prednisolone (1 mg/kg/day) and MMF (800 mg/m2/day) from Day 7 onwards. Overall, the anti-GBM and p-ANCA levels declined throughout the treatment period but the patient progressed towards end stage renal disease.},
 year = {2021}
}

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TY  - JOUR
T1  - Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature
AU  - Subal Kumar Pradhan
AU  - Snehamayee Nayak
Y1  - 2021/02/23
PY  - 2021
N1  - https://doi.org/10.11648/j.ajp.20210701.14
DO  - 10.11648/j.ajp.20210701.14
T2  - American Journal of Pediatrics
JF  - American Journal of Pediatrics
JO  - American Journal of Pediatrics
SP  - 14
EP  - 18
PB  - Science Publishing Group
SN  - 2472-0909
UR  - https://doi.org/10.11648/j.ajp.20210701.14
AB  - ‘Double-positive’ with anti-glomerular basement membrane (anti-GBM) antibody and anti-neutrophil cytoplasmic antibody (ANCA) is an extremely rare cause of small vessel vasculitis in children. Studies have reported a distinct hybrid phenotype in double-positive patients which requires an aggressive treatment approach. The data on double positive children are scarce with few case reports available with varied outcomes. A case of 9-year-old girl from India who had double-positive anti-GBM disease is reported here. The patient presented with complaints of edema, oliguria, gross hematuria with rising creatine and was diagnosed as rapidly progressive glomerulonephritis (RPGN). Histological examinations of linear immunoglobulin G deposits along with glomerular capillaries were suggestive of anti-GBM disease. The anti-GBM and p-ANCA antibody titers were also high, and the aforementioned findings led to a diagnosis of ‘double-positive’ anti-GBM with RPGN. She was treated with standard plasma exchange therapy along with pulse methyl prednisolone (3 doses of 30 mg/kg/day on Days 1 to 3), oral prednisolone (1.5 mg/kg on Day 4), single intravenous injection of cyclophosphamide (500 mg/m2 on Day 5) and mycophenolate mofetil (MMF; 1000 mg/m2 on Day 6) followed by maintenance treatment with oral prednisolone (1 mg/kg/day) and MMF (800 mg/m2/day) from Day 7 onwards. Overall, the anti-GBM and p-ANCA levels declined throughout the treatment period but the patient progressed towards end stage renal disease.
VL  - 7
IS  - 1
ER  -

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Author Information

Subal Kumar Pradhan

Department of Paediatrics, Sardar Vallav Bhai Patel Post Graduate Institute of Pediatrics and Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India
Snehamayee Nayak

Department of Paediatrics, Sardar Vallav Bhai Patel Post Graduate Institute of Pediatrics and Srirama Chandra Bhanja Medical College, Cuttack, Odisha, India

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APA Style

Subal Kumar Pradhan, Snehamayee Nayak. (2021). Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature. American Journal of Pediatrics, 7(1), 14-18. https://doi.org/10.11648/j.ajp.20210701.14

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ACS Style

Subal Kumar Pradhan; Snehamayee Nayak. Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature. Am. J. Pediatr. 2021, 7(1), 14-18. doi: 10.11648/j.ajp.20210701.14

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AMA Style

Subal Kumar Pradhan, Snehamayee Nayak. Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature. Am J Pediatr. 2021;7(1):14-18. doi: 10.11648/j.ajp.20210701.14

Copy | Download

@article{10.11648/j.ajp.20210701.14,
  author = {Subal Kumar Pradhan and Snehamayee Nayak},
  title = {Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature},
  journal = {American Journal of Pediatrics},
  volume = {7},
  number = {1},
  pages = {14-18},
  doi = {10.11648/j.ajp.20210701.14},
  url = {https://doi.org/10.11648/j.ajp.20210701.14},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajp.20210701.14},
  abstract = {‘Double-positive’ with anti-glomerular basement membrane (anti-GBM) antibody and anti-neutrophil cytoplasmic antibody (ANCA) is an extremely rare cause of small vessel vasculitis in children. Studies have reported a distinct hybrid phenotype in double-positive patients which requires an aggressive treatment approach. The data on double positive children are scarce with few case reports available with varied outcomes. A case of 9-year-old girl from India who had double-positive anti-GBM disease is reported here. The patient presented with complaints of edema, oliguria, gross hematuria with rising creatine and was diagnosed as rapidly progressive glomerulonephritis (RPGN). Histological examinations of linear immunoglobulin G deposits along with glomerular capillaries were suggestive of anti-GBM disease. The anti-GBM and p-ANCA antibody titers were also high, and the aforementioned findings led to a diagnosis of ‘double-positive’ anti-GBM with RPGN. She was treated with standard plasma exchange therapy along with pulse methyl prednisolone (3 doses of 30 mg/kg/day on Days 1 to 3), oral prednisolone (1.5 mg/kg on Day 4), single intravenous injection of cyclophosphamide (500 mg/m2 on Day 5) and mycophenolate mofetil (MMF; 1000 mg/m2 on Day 6) followed by maintenance treatment with oral prednisolone (1 mg/kg/day) and MMF (800 mg/m2/day) from Day 7 onwards. Overall, the anti-GBM and p-ANCA levels declined throughout the treatment period but the patient progressed towards end stage renal disease.},
 year = {2021}
}

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TY  - JOUR
T1  - Double-positive Anti-glomerular Basement Membrane Disease in a Child with Crescentic Glomerulonephritis: A Case Report and Review of Literature
AU  - Subal Kumar Pradhan
AU  - Snehamayee Nayak
Y1  - 2021/02/23
PY  - 2021
N1  - https://doi.org/10.11648/j.ajp.20210701.14
DO  - 10.11648/j.ajp.20210701.14
T2  - American Journal of Pediatrics
JF  - American Journal of Pediatrics
JO  - American Journal of Pediatrics
SP  - 14
EP  - 18
PB  - Science Publishing Group
SN  - 2472-0909
UR  - https://doi.org/10.11648/j.ajp.20210701.14
AB  - ‘Double-positive’ with anti-glomerular basement membrane (anti-GBM) antibody and anti-neutrophil cytoplasmic antibody (ANCA) is an extremely rare cause of small vessel vasculitis in children. Studies have reported a distinct hybrid phenotype in double-positive patients which requires an aggressive treatment approach. The data on double positive children are scarce with few case reports available with varied outcomes. A case of 9-year-old girl from India who had double-positive anti-GBM disease is reported here. The patient presented with complaints of edema, oliguria, gross hematuria with rising creatine and was diagnosed as rapidly progressive glomerulonephritis (RPGN). Histological examinations of linear immunoglobulin G deposits along with glomerular capillaries were suggestive of anti-GBM disease. The anti-GBM and p-ANCA antibody titers were also high, and the aforementioned findings led to a diagnosis of ‘double-positive’ anti-GBM with RPGN. She was treated with standard plasma exchange therapy along with pulse methyl prednisolone (3 doses of 30 mg/kg/day on Days 1 to 3), oral prednisolone (1.5 mg/kg on Day 4), single intravenous injection of cyclophosphamide (500 mg/m2 on Day 5) and mycophenolate mofetil (MMF; 1000 mg/m2 on Day 6) followed by maintenance treatment with oral prednisolone (1 mg/kg/day) and MMF (800 mg/m2/day) from Day 7 onwards. Overall, the anti-GBM and p-ANCA levels declined throughout the treatment period but the patient progressed towards end stage renal disease.
VL  - 7
IS  - 1
ER  -

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