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Published in: Journal of Neurology 8/2021

01-08-2021 | Original Communication

CIDP associated with Sjögren’s syndrome

Authors: Tabea Seeliger, Stefan Gingele, Lena Bönig, Franz Felix Konen, Sonja Körner, Nils Prenzler, Thea Thiele, Diana Ernst, Torsten Witte, Martin Stangel, Thomas Skripuletz

Published in: Journal of Neurology | Issue 8/2021

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Abstract

Background

This study addresses the challenging characterisation and differentiation of CIDP versus CIDP in association with Sjögren’s syndrome to facilitate the process in clinical routine.

Methods

Patients with both CIDP and Sjögren’s syndrome and CIDP without Sjögren’s syndrome were compared concerning relevant differences in clinical, laboratory and electrophysiological findings. 154 patients who fulfilled the diagnostic EFNS/PNS criteria for CIDP were included in the analysis. 54 of these patients additionally fulfilled the ACR/EULAR classification criteria for Sjögren’s syndrome.

Results

The frequency of female patients was higher in patients with CIDP and Sjögren’s syndrome (52%) versus CIDP patients without Sjögren’s syndrome (28%). Furthermore, the occurrence of cranial nerve impairment was significantly higher in patients with Sjögren’s syndrome (39% versus 14%). There were no significant group differences in the evaluation of initial symptoms, severity of disability judged by INCAT disability scale score, presence or distribution of sensory deficits, limb weakness and the presence of ataxia, pain or dysautonomia, CSF laboratory or electrophysiological findings.

Conclusions

In conclusion, our data indicate that cranial nerve impairment and female gender might represent red flags for an additional Sjögren’s syndrome in patients with CIDP. The patterns of clinical disabilities and electrophysiological findings due to peripheral nerve damage are similar in both CIDP entities.
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Metadata
Title
CIDP associated with Sjögren’s syndrome
Authors
Tabea Seeliger
Stefan Gingele
Lena Bönig
Franz Felix Konen
Sonja Körner
Nils Prenzler
Thea Thiele
Diana Ernst
Torsten Witte
Martin Stangel
Thomas Skripuletz
Publication date
01-08-2021
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 8/2021
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-021-10459-z

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