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. 2024 Nov;54(11):e2450998.
doi: 10.1002/eji.202450998. Epub 2024 Aug 20.

Circulating levels of endogenous complement inhibitors correlate inversely with complement consumption in systemic lupus erythematosus

Stef van der Meulen  1 , Rory C Monahan  2 , Kyra A Gelderman  3 , Cees van Kooten  4 , Y K Onno Teng  4 , Tom W J Huizinga  2 , Gerda M Steup-Beekman  2   5 , Leendert A Trouw  1
Affiliations

Circulating levels of endogenous complement inhibitors correlate inversely with complement consumption in systemic lupus erythematosus

Stef van der Meulen et al. Eur J Immunol. 2024 Nov.

Abstract

Systemic lupus erythematosus (SLE) is marked by excessive complement activation, contributing to tissue damage. Complement activation can be detected in many organs including the skin, kidney, and brain. The involvement of the central nervous system is particularly relevant to understanding neuropsychiatric SLE (NPSLE), one of the poorest understood manifestations of SLE for which no biomarkers are available. We studied the levels of complement inhibitors in SLE in relation to disease activity and as possible biomarkers to identify NPSLE. Serum levels of complement inhibitors C1-inhibitor (C1-INH), C4b-binding protein (C4BP), Factor I, and Factor H were measured in 345 SLE patients (including 102 with NPSLE) and 108 healthy controls. Compared with controls, SLE patients had higher C1-INH and C4BP but lower Factor I and H levels. All inhibitors positively correlated with total C3 and C4 levels. While correlating with the SLE Disease Activity Index (SLEDAI), no distinction in inhibitor levels was found between SLE and NPSLE patients. Over time, C1-INH and Factor H levels normalized, but no significant changes were observed for C4BP and Factor I. In SLE the levels of circulating complement inhibitors are inversely correlated to complement consumption but do not serve as biomarkers for NPSLE.

Keywords: Autoimmunity; Complement system.

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References

    1. Pons‐Estel, G. J., Ugarte‐Gil, M. F. and Alarcon, G. S., Epidemiology of systemic lupus erythematosus. Expert Rev. Clin. Immunol. 2017. 13: 799–814.
    1. Steup‐Beekman, G. M., Zirkzee, E. J., Cohen, D., Gahrmann, B. M., Emmer, B. J., Steens, S. C., Bollen, E. L. et al., Neuropsychiatric manifestations in patients with systemic lupus erythematosus: epidemiology and radiology pointing to an immune‐mediated cause. Ann. Rheum Dis. 2013. 72: ii76–79.
    1. Schwartz, N., Stock, A. D. and Putterman, C., Neuropsychiatric lupus: new mechanistic insights and future treatment directions. Nat. Rev. Rheumatol. 2019. 15: 137–152.
    1. Cui, Y., Sheng, Y. and Zhang, X., Genetic susceptibility to SLE: recent progress from GWAS. J. Autoimmun. 2013. 41: 25–33.
    1. Justiz Vaillant, A. A., Goyal, A. and Varacallo, M., Systemic lupus erythematosus StatPearls. Treasure Island (FL) 2022.

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