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@ARTICLE{Landt:283072,
author = {Landt, Carolin and Held, Friederike and Kolotourou,
Konstantina and Guranda, Mihaela and Vakrakou, Aigli G and
Franz, Jonas and Thomas, Carolina and Schmiedeknecht, Max
Ulrich Heiner and Bergann, Klaus and Wrzos, Claudia and
Endmayr, Verena and Tsaktanis, Thanos and Zechel, Sabrina
and Ancău, Mihai and Misgeld, Thomas and Hakroush, Samy and
Hametner, Simon and Rothhammer, Veit and Höftberger, Romana
and Hemmer, Bernhard and Stadelmann, Christine and Nessler,
Stefan},
title = {{C}omplement {C}4d {I}nforms the {D}ifferential {D}iagnosis
of {I}nflammatory {D}emyelinating {CNS} {D}iseases.},
journal = {Neurology: Neuroimmunology $\&$ Neuroinflammation ;
official journal of the American Academy of Neurology},
volume = {13},
number = {2},
issn = {2332-7812},
address = {Philadelphia, Pa.},
publisher = {Wolters Kluwer},
reportid = {DZNE-2025-01479},
pages = {e200528},
year = {2026},
abstract = {Complement-targeting therapies are pivotal in managing
neuromyelitis optica spectrum disorder (NMOSD), calling for
a deeper understanding of complement activation across
idiopathic inflammatory demyelinating diseases (IIDDs) of
the CNS. C4d, a covalently bound complement split product,
offers prolonged detectability at activation sites. This
study explores whether C4d immunohistochemistry (IHC)
extends the detection window for complement activation in
CNS biopsies of IIDDs and evaluates its usefulness as a
fluid biomarker.Forty-four IIDD biopsies with active
demyelination were analyzed for complement deposition using
IHC for C9neo and C4d. C4d levels were also quantified in
blood and CSF of patients with IIDDs. The persistence of C4d
in CNS tissue was further evaluated in an in vivo NMOSD
model.C4d IHC enhanced the sensitivity to detect complement
activation, surpassing C9neo by twofold in NMOSD and by
sixfold in ADEM, while remaining undetectable in MS
biopsies. Exclusive C4d immunopositivity at the glia
limitans distinguished NMOSD from ADEM. Furthermore, CSF C4d
levels were significantly elevated in both seronegative and
seropositive NMOSD compared with MS.C4d detection extends
the window for identifying complement activation in CNS
biopsies of IIDDs and emerges as a valuable CSF biomarker,
enhancing diagnostic precision, autoantibody target
identification, and patient stratification for
complement-targeting therapies.},
keywords = {Humans / Complement C4b: cerebrospinal fluid / Complement
C4b: metabolism / Complement C4b: analysis / Female / Male /
Middle Aged / Adult / Neuromyelitis Optica: diagnosis /
Diagnosis, Differential / Biomarkers: cerebrospinal fluid /
Biomarkers: blood / Peptide Fragments: cerebrospinal fluid /
Aged / Demyelinating Autoimmune Diseases, CNS: diagnosis /
Complement Activation / Young Adult / Animals / Multiple
Sclerosis: diagnosis / Complement C4b (NLM Chemicals) /
complement C4d (NLM Chemicals) / Biomarkers (NLM Chemicals)
/ Peptide Fragments (NLM Chemicals)},
cin = {AG Misgeld},
ddc = {610},
cid = {I:(DE-2719)1110000-4},
pnm = {351 - Brain Function (POF4-351)},
pid = {G:(DE-HGF)POF4-351},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:41435221},
pmc = {pmc:PMC12735299},
doi = {10.1212/NXI.0000000000200528},
url = {https://pub.dzne.de/record/283072},
}