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. 2024 Oct 10;19(10):e0311678.
doi: 10.1371/journal.pone.0311678. eCollection 2024.

Clinical impact of ceruloplasmin levels at ANCA-associated vasculitis diagnosis

Louis Camboulive  1 , Frédérique Grandhomme  2 , Nicolas Martin Silva  1 , Kathy Khoy  3 , Delphine Mariotte  3 , Thierry Lobbedez  4 , Anaël Dumont  1 , Alexandre Nguyen  1 , Hubert de Boysson  1   5 , Achille Aouba  1 , Samuel Deshayes  1
Affiliations

Clinical impact of ceruloplasmin levels at ANCA-associated vasculitis diagnosis

Louis Camboulive et al. PLoS One. .

Abstract

Objectives: Ceruloplasmin is an inhibitor of myeloperoxidase (MPO) activity that plays an important role in the pathophysiology of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). This study aimed to evaluate the prognostic impact of serum level of ceruloplasmin at diagnosis in patients with anti-MPO antibody-positive AAV.

Methods: This retrospective monocentric study in Caen University Hospital involved all consecutive adult anti-MPO antibody-positive patients with microscopic polyangiitis or granulomatosis with polyangiitis, diagnosed between January 2010 and January 2022 with available serum sample at inclusion. Patients outcomes were analyzed from two subgroups constituted according to the median serum level of ceruloplasmin. The same analyses were then performed in anti-proteinase 3 (PR3) antibody-positive patients.

Results: Within the 92 patients analyzed, 50 patients had anti-MPO antibodies with a median ceruloplasmin level of 0.44 [quartiles 1-3, 0.40-0.49] g/L and a median Birmingham Vasculitis Activity Score of 19 [14-22]. After a median follow-up period of 40 [22-86] months, 13 (26%) patients had died: 10 (40%) in the low ceruloplasmin group and 3 (12%) in the high ceruloplasmin group (p = 0.03), with a significantly worse survival rate in the low ceruloplasmin group (p = 0.021). No significant differences in relapse rate or renal failure was observed between the two groups. The same analyses performed in the group of AAV patients with anti-PR3 antibody did not show any differences.

Conclusion: In anti-MPO AAV patients, serum level of ceruloplasmin at diagnosis seems to be associated with a significant impact on survival.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Fig 1
Fig 1
A. Survival curves of patients with anti-MPO ANCA vasculitis. B. Renal survival curves of patients with anti-PR3 ANCA vasculitis. C. Survival curves of patients with a microscopic polyangiitis phenotype. D. Renal survival curves of patients with a microscopic polyangiitis phenotype. E. Survival curves of patients with a polyangiitis granulomatosis phenotype. F. Renal survival curve of patients with a polyangiitis granulomatosis phenotype.
See this image and copyright information in PMC

References

    1. Saeki T, Kuroda T, Morita T, Suzuki K, Arakawa M, Kawasaki K. Significance of myeloperoxidase in rapidly progressive glomerulonephritis. Am J Kidney Dis 1995;26:13–21. doi: 10.1016/0272-6386(95)90147-7 - DOI - PubMed
    1. Clark RA, Stone PJ, El Hag A, Calore JD, Franzblau C. Myeloperoxidase-catalyzed inactivation of alpha 1-protease inhibitor by human neutrophils. J Biol Chem 1981;256:3348–53. - PubMed
    1. Shiratori-Aso S, Nakazawa D. The involvement of NETs in ANCA-associated vasculitis. Front Immunol 2023;14:1261151. doi: 10.3389/fimmu.2023.1261151 - DOI - PMC - PubMed
    1. Sokolov AV, Ageeva KV, Pulina MO, Cherkalina OS, Samygina VR, Vlasova II, et al.. Ceruloplasmin and myeloperoxidase in complex affect the enzymatic properties of each other. Free Radic Res 2008;42:989–98. doi: 10.1080/10715760802566574 - DOI - PubMed
    1. Deshayes S, Martin Silva N, Grandhomme F, Khoy K, Mariotte D, Boutemy J, et al.. Clinical Effect of Alpha-1 Antitrypsin Deficiency in Antineutrophil Cytoplasmic Antibody-associated Vasculitis: Results from a French Retrospective Monocentric Cohort. J Rheumatol 2019;46:1502–8. doi: 10.3899/jrheum.180591 - DOI - PubMed

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