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@ARTICLE{Nitsch:278784,
author = {Nitsch, Louisa and Skowasch, Dirk and Courtin, Edouard and
Duffau, Pierre and Otto, Carolin and Ruprecht, Klemens and
Dambietz, Christine and Heming, Michael and Meyer Zu
Hörste, Gerd and Byg, Keld-Erik and Kindler, Christine and
Gutzwiller, Julia and Pretzsch, Roxanne and Pröbstel,
Anne-Katrin and Weller, Johannes M and Zimmermann, Julian},
title = {{F}actors {A}ssociated {W}ith {R}elapse in {P}atients
{W}ith {N}eurosarcoidosis.},
journal = {Neurology},
volume = {104},
number = {11},
issn = {0028-3878},
address = {Philadelphia, Pa.},
publisher = {Wolters Kluwer},
reportid = {DZNE-2025-00620},
pages = {e213705},
year = {2025},
abstract = {Five to 10 percent of patients with sarcoidosis show
involvement of the nervous system. Given the serious nature
of neurosarcoidosis and the high risk of irreversible
neurologic damage, rapid diagnosis, accurate
prognostication, and individualized treatment are crucial.
The aim of this study was to identify factors associated
with relapses in neurosarcoidosis.In this multicenter
retrospective cohort study, patients diagnosed with
possible, probable, or definite neurosarcoidosis between
January 1, 2010, and June 30, 2024, at 6 tertiary
neuroimmunology centers across Europe were included.
Patients were identified from the respective hospital-based
databases by the participating centers. Clinical
presentation, imaging results, CSF analysis, and
immunosuppressive therapies were evaluated. Predictors of
relapse, defined as clinical relapse or progression, or new
MRI lesions on follow-up ≥2 months after initial
manifestation, were analyzed with log-rank tests and Cox
regression models, and therapeutic strategies in clinical
practice were assessed. The association between identified
risk factors (RFs) and therapeutic strategies was explored.A
total of 174 patients with neurosarcoidosis were included
with a median follow-up of 24 months (interquartile range
12-48.8). The mean age was 48.1 years, and $57.5\%$ were
female. CNS parenchymal lesions including encephalitis,
myelitis, and optic neuritis (hazard ratio [HR] 2.3, $95\%$
CI 1.3-4.2); CSF-specific oligoclonal bands (OCBs) (HR 2.1,
$95\%$ CI 1.3-3.6); CSF glucose <40 mg/dL (HR 2, $95\%$ CI
1.1-3.4); and CSF protein ≥1,000 mg/dL (HR 2.1, $95\%$ CI
1.3-3.5) were identified as RFs of relapse. Patients with
3-4 RFs had a median relapse-free survival of 12 months
$(95\%$ CI 4.3-19.7), compared with 36 months $(95\%$ CI
24.3-47.7) in patients with 1-2 RFs and 120 months $(95\%$
CI 2.1-237.9) in patients without RFs (p < 0.01). The
likelihood of treatment escalation increased with the number
of RFs, from $14.3\%$ in patients without RFs to $28.2\%$
and $50\%$ in patients with 1-2 or 3-4 RFs, respectively (p
< 0.01).The identification of specific RFs, including
parenchymal lesions, OCBs, CSF glucose <40 mg/dL, and CSF
protein ≥1,000 mg/dL, enables better prognostication and
might inform individualized treatment approaches. Patients
with multiple RFs are at greater risk of relapse, possibly
suggesting the need for more aggressive therapies.},
keywords = {Humans / Sarcoidosis: cerebrospinal fluid / Sarcoidosis:
diagnostic imaging / Sarcoidosis: epidemiology /
Sarcoidosis: drug therapy / Female / Male / Middle Aged /
Central Nervous System Diseases: cerebrospinal fluid /
Central Nervous System Diseases: diagnostic imaging /
Central Nervous System Diseases: epidemiology / Central
Nervous System Diseases: drug therapy / Central Nervous
System Diseases: diagnosis / Adult / Retrospective Studies /
Recurrence / Risk Factors / Magnetic Resonance Imaging /
Cohort Studies / Disease Progression / Immunosuppressive
Agents: therapeutic use / Immunosuppressive Agents (NLM
Chemicals)},
cin = {AG Pröbstel},
ddc = {610},
cid = {I:(DE-2719)1013045},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40373242},
doi = {10.1212/WNL.0000000000213705},
url = {https://pub.dzne.de/record/278784},
}