Double-Positive Anti-GBM and ANCA Vasculitis: 2 Case Reports and Review of the Literature
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Abstract
Double-Positive Patients (DPPs), characterized by the simultaneous presence of Anti-Neutrophil Cytoplasmic Antibody (ANCA) and anti-Glomerular Basement Membrane (anti-GBM) antibodies, represent a rare subset in systemic vasculitis.
We present two cases of DPPs with renal involvement and review the existing literature to elucidate the clinical characteristics, histopathological findings, management strategies, and prognostic outcomes associated with this condition. Both cases exhibited renal involvement with rapidly progressive glomerulonephritis, requiring renal replacement therapy. Renal biopsies confirmed crescentic glomerulonephritis with features of both anti-GBM disease and ANCA-associated vasculitis. Management included high-dose glucocorticoids, cyclophosphamide, and consideration of plasma exchanges.
Double-positive ANCA and anti-GBM vasculitis pose challenges in management and prognosis. Further research is essential to improve therapeutic strategies for this rare and heterogeneous condition.
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Copyright (c) 2025 Selim B, et al.
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Philip R, Dumont A, Le Mauff B, Martinet M, Martin Silva N, de Boysson H, et al. ANCA and anti-MBG double-positive vasculitis: An update on the clinical and therapeutic specificities and comparison with the two eponymous vasculitis. La Rev Med Interne. 2020;41(1):21-6. Available from: https://doi.org/10.1016/j.revmed.2019.10.334
Rutgers A, Slot M, van Paassen P, van Breda Vriesman P, Heeringa P, Tervaert JW. Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis. Am J Kidney Dis. 2005;46(2):253-62. Available from: https://doi.org/10.1053/j.ajkd.2005.05.003
Srivastava A, Rao GK, Segal PE, Shah M, Geetha D. Characteristics and outcome of crescentic glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody. Clin Rheumatol. 2013;32(9):1317-22. Available from: https://doi.org/10.1007/s10067-013-2268-5
Zhao J, Yang R, Cui Z, Chen M, Zhao MH, Wang HY. Characteristics and outcome of Chinese patients with both antineutrophil cytoplasmic antibody and antiglomerular basement membrane antibodies. Nephron Clin Pract. 2007;107(2):c56-62. Available from: https://doi.org/10.1159/000107803
Lindic J, Vizjak A, Ferluga D, Kovac D, Ales A, Kveder R, et al. Clinical outcome of patients with coexistent antineutrophil cytoplasmic antibodies and antibodies against glomerular basement membrane. Ther Apher Dial. 2009;13(4):278-81. Available from: https://doi.org/10.1111/j.1744-9987.2009.00724.x
Weber MF, Andrassy K, Pullig O, Koderisch J, Netzer K. Antineutrophil-cytoplasmic antibodies and antiglomerular basement membrane antibodies in Goodpasture's syndrome and Wegener's granulomatosis. J Am Soc Nephrol. 1992;2(7):1227-34. Available from: https://doi.org/10.1681/asn.v271227
Hu X, Shen C, Meng T, Ooi JD, Eggenhuizen PJ, Zhou Y-o, et al. Clinical features and prognosis of MPO-ANCA and antiGBM double-seropositive patients. Front Immunol. 2022;13:991469. Available from: https://doi.org/10.3389/fimmu.2022.991469
McAdoo SP, Tanna A, Hrušková Z, Holm L, Weiner M, Arulkumaran N, et al. Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients. Kidney Int. 2017;92(3):693-702. Available from: https://doi.org/10.1016/j.kint.2017.03.014
Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR, et al. ANCA-associated vasculitis. Nat Rev Dis Primers. 2020;6(1):71. Available from: https://doi.org/10.1038/s41572-020-0204-y
Gulati K, McAdoo SP. Anti-glomerular basement membrane disease. Rheum Dis Clin North Am. 2018;44(4):651-73. Available from: https://doi.org/10.1016/j.rdc.2018.06.011
Geetha D, Jefferson JA. ANCA-associated vasculitis: Core curriculum 2020. Am J Kidney Dis. 2020;75(1):124-37. Available from: https://doi.org/10.1053/j.ajkd.2019.04.031
Nishibata Y, Nonokawa M, Tamura Y, Higashi R, Suzuki K, Hayashi H, et al. Possible implication of intermolecular epitope spreading in the production of antiglomerular basement membrane antibody in anti-neutrophil cytoplasmic antibody-associated vasculitis. Clin Exp Rheumatol. 2022;40(4):691-704. Available from: https://doi.org/10.55563/clinexprheumatol/6oq9du
Hellmark T, Niles JL, Collins AB, McCluskey RT, Brunmark C. Comparison of anti-GBM antibodies in sera with or without ANCA. J Am Soc Nephrol. 1997;8(3):376-85. Available from: https://doi.org/10.1681/asn.v83376
Cui Z, Zhao J, Jia XY, Zhu SN, Zhao MH. Clinical features and outcomes of anti-glomerular basement membrane disease in older patients. Am J Kidney Dis. 2011;57(4):575-82. Available from: https://doi.org/10.1053/j.ajkd.2010.09.022
Levy JB, Hammad T, Coulthart A, Dougan T, Pusey CD. Clinical features and outcome of patients with both ANCA and anti-GBM antibodies. Kidney Int. 2004;66(4):1535-40. Available from: https://doi.org/10.1111/j.1523-1755.2004.00917.x
Walsh M, Merkel PA, Peh CA, Szpirt WM, Puéchal X, Fujimoto S, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med. 2020;382(7):622-631. Available from: https://pubmed.ncbi.nlm.nih.gov/32053298/
Taaam Z, Benabdelhak M, Haddiya I, Bentata Y. The use of rituximab in double-positive ANCA and anti-GBM vasculitis: A case report. Néphrol Thérapeutique. 2022;18(5):410-411. Available from: https://doi.org/10.1016/j.nephro.2022.07.363
Segelmark M, Hellmark T, Wieslander J. The prognostic significance in Goodpasture's disease of specificity, titre, and affinity of anti-glomerular-basement-membrane antibodies. Nephron Clin Pract. 2003;94(3):c59-68. Available from: https://doi.org/10.1159/000072022
Jayne DR, Marshall PD, Jones SJ, Lockwood CM. Autoantibodies to GBM and neutrophil cytoplasm in rapidly progressive glomerulonephritis. Kidney Int. 1990;37(3):965-70. Available from: https://doi.org/10.1038/ki.1990.72