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Challenges in diagnostic testing of nephritic factors

Nephritic factors (NeFs) are autoantibodies promoting the activity of the central enzymes of the complement cascade, an important first line of defense of our innate immune system. NeFs stabilize the complement convertase complexes and prevent their natural and regulator-mediated decay. They are mos...

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Autores principales: Michels, Marloes A. H. M., Volokhina, Elena B., van de Kar, Nicole C. A. J., van den Heuvel, Lambertus P.W. J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9702996/
https://www.ncbi.nlm.nih.gov/pubmed/36451820
http://dx.doi.org/10.3389/fimmu.2022.1036136
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author Michels, Marloes A. H. M.
Volokhina, Elena B.
van de Kar, Nicole C. A. J.
van den Heuvel, Lambertus P.W. J.
author_facet Michels, Marloes A. H. M.
Volokhina, Elena B.
van de Kar, Nicole C. A. J.
van den Heuvel, Lambertus P.W. J.
author_sort Michels, Marloes A. H. M.
collection PubMed
description Nephritic factors (NeFs) are autoantibodies promoting the activity of the central enzymes of the complement cascade, an important first line of defense of our innate immune system. NeFs stabilize the complement convertase complexes and prevent their natural and regulator-mediated decay. They are mostly associated with rare complement-mediated kidney disorders, in particular with C3 glomerulopathy and related diseases. Although these autoantibodies were already described more than 50 years ago, measuring NeFs for diagnostic purposes remains difficult, and this also complicates our understanding of their clinical associations. In this review, we address the multifactorial challenges of NeF diagnostics. We describe the diseases NeFs are associated with, the heterogenic mechanisms of action of different NeF types, the different methods available in laboratories used for their detection, and efforts for standardization. Finally, we discuss the importance of proper NeF diagnostics for understanding the clinical impact of these autoantibodies in disease pathophysiology and for considering future complement-directed therapy.
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spelling pubmed-97029962022-11-29 Challenges in diagnostic testing of nephritic factors Michels, Marloes A. H. M. Volokhina, Elena B. van de Kar, Nicole C. A. J. van den Heuvel, Lambertus P.W. J. Front Immunol Immunology Nephritic factors (NeFs) are autoantibodies promoting the activity of the central enzymes of the complement cascade, an important first line of defense of our innate immune system. NeFs stabilize the complement convertase complexes and prevent their natural and regulator-mediated decay. They are mostly associated with rare complement-mediated kidney disorders, in particular with C3 glomerulopathy and related diseases. Although these autoantibodies were already described more than 50 years ago, measuring NeFs for diagnostic purposes remains difficult, and this also complicates our understanding of their clinical associations. In this review, we address the multifactorial challenges of NeF diagnostics. We describe the diseases NeFs are associated with, the heterogenic mechanisms of action of different NeF types, the different methods available in laboratories used for their detection, and efforts for standardization. Finally, we discuss the importance of proper NeF diagnostics for understanding the clinical impact of these autoantibodies in disease pathophysiology and for considering future complement-directed therapy. Frontiers Media S.A. 2022-11-14 /pmc/articles/PMC9702996/ /pubmed/36451820 http://dx.doi.org/10.3389/fimmu.2022.1036136 Text en Copyright © 2022 Michels, Volokhina, van de Kar and van den Heuvel 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). 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 Immunology
Michels, Marloes A. H. M.
Volokhina, Elena B.
van de Kar, Nicole C. A. J.
van den Heuvel, Lambertus P.W. J.
Challenges in diagnostic testing of nephritic factors
title Challenges in diagnostic testing of nephritic factors
title_full Challenges in diagnostic testing of nephritic factors
title_fullStr Challenges in diagnostic testing of nephritic factors
title_full_unstemmed Challenges in diagnostic testing of nephritic factors
title_short Challenges in diagnostic testing of nephritic factors
title_sort challenges in diagnostic testing of nephritic factors
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9702996/
https://www.ncbi.nlm.nih.gov/pubmed/36451820
http://dx.doi.org/10.3389/fimmu.2022.1036136
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