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21.02.2021 | Original Communication

CIDP associated with Sjögren’s syndrome

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

Erschienen in: Journal of Neurology | Ausgabe 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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Titel: CIDP associated with Sjögren’s syndrome
verfasst von: Tabea Seeliger
Stefan Gingele
Lena Bönig
Franz Felix Konen
Sonja Körner
Nils Prenzler
Thea Thiele
Diana Ernst
Torsten Witte
Martin Stangel
Thomas Skripuletz
Publikationsdatum: 21.02.2021
Verlag: Springer Berlin Heidelberg
Erschienen in: Journal of Neurology / Ausgabe 8/2021
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-021-10459-z

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Abstract

Background

Methods

Results

Conclusions

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