Skip to main content

Advertisement

SpringerLink
Log in

CIDP associated with Sjögren’s syndrome

  • Original Communication
  • Published:
Journal of Neurology Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Data availability

Data are available upon reasonable request.

References

  1. Sjögren H (1933) Zur kenntnis der keratoconjunctivitis sicca. Acta Ophthalmol 13:1–39

    Article  Google Scholar 

  2. Seror R, Ravaud P, Bowman SJ et al (2010) EULAR Sjögren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren’s syndrome. Ann Rheum Dis 69:1103–1109. https://doi.org/10.1136/ard.2009.110619

    Article  PubMed  Google Scholar 

  3. Ramos-Casals M, Brito-Zerón P, Seror R et al (2015) Characterization of systemic disease in primary Sjögren’s syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatol (Oxf) 54:2230–2238. https://doi.org/10.1093/rheumatology/kev200

    Article  Google Scholar 

  4. Jaskólska M, Chylińska M, Masiak A et al (2020) Neuro-Sjögren: uncommon or underestimated problem? Brain Behav. https://doi.org/10.1002/BRB3.1665

    Article  PubMed  PubMed Central  Google Scholar 

  5. Thiele T, Seeliger T, Witte T et al (2020) Severe polyneuropathy in primary Sjögren’s syndrome: Sjögren’s syndrome should be considered in patients with motor neuropathy. Z Rheumatol 79:707–709

    Article  CAS  Google Scholar 

  6. Seeliger T, Prenzler NK, Gingele S et al (2019) Neuro-Sjögren: peripheral neuropathy with limb weakness in Sjögren’s Syndrome. Front Immunol 10:1600. https://doi.org/10.3389/fimmu.2019.01600

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Perzyńska-Mazan J, Maślińska M, Gasik R (2020) Neurophysiological features of peripheral nervous system involvement and immunological profile of patients with primary Sjögren Syndrome. J Rheumatol. https://doi.org/10.3899/jrheum.181464 (jrheum.181464)

    Article  PubMed  Google Scholar 

  8. Delalande S, de Seze J, Fauchais A-L et al (2004) Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Med (Baltim) 83:280–291

    Article  Google Scholar 

  9. Teixeira F, Moreira I, Silva AM et al (2013) Neurological involvement in primary Sjögren syndrome. Acta Reumatol Port 38:29–36

    PubMed  Google Scholar 

  10. Van den Bergh PYK, Hadden RDM, Bouche P et al (2010) European federation of neurological societies/peripheral nerve society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European federation of neurological societies and the peripheral nerve society: first revision. Eur J Neurol 17:356–363. https://doi.org/10.1111/j.1468-1331.2009.02930.x

    Article  PubMed  Google Scholar 

  11. Shiboski CH, Shiboski SC, Seror R et al (2017) 2016 American college of rheumatology/European league against rheumatism classification criteria for primary Sjögren’s syndrome. Ann Rheum Dis 76:9–16. https://doi.org/10.1136/annrheumdis-2016-210571

    Article  PubMed  Google Scholar 

  12. Hughes R, Bensa S, Willison H et al (2001) Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 50:195–201

    Article  CAS  Google Scholar 

  13. Stålberg E, Falck B, Gilai A et al (1999) Standards for quantification of EMG and neurography. The international federation of clinical neurophysiology. Electroencephalogr Clin Neurophysiol Suppl 52:213–220

    PubMed  Google Scholar 

  14. Schwenkenbecher P, Chacko LP, Wurster U et al (2016) Intrathecal synthesis of anti-Hu antibodies distinguishes patients with paraneoplastic peripheral neuropathy and encephalitis. BMC Neurol 16:136. https://doi.org/10.1186/s12883-016-0657-5

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Seror R, Theander E, Brun JG et al (2015) Validation of EULAR primary Sjögren’s syndrome disease activity (ESSDAI) and patient indexes (ESSPRI). Ann Rheum Dis 74:859–866. https://doi.org/10.1136/annrheumdis-2013-204615

    Article  PubMed  Google Scholar 

  16. Seeliger T, Bönig L, Witte T et al (2020) Hearing dysfunction in patients with Neuro-Sjögren: a cross-sectional study. Ann Transl Med 8:1069–1069. https://doi.org/10.21037/atm-20-1856

    Article  PubMed  PubMed Central  Google Scholar 

  17. Mori K, Iijima M, Koike H et al (2005) The wide spectrum of clinical manifestations in Sjögren’s syndrome-associated neuropathy. Brain 128:2518–2534. https://doi.org/10.1093/brain/awh605

    Article  PubMed  Google Scholar 

  18. Carvajal Alegria G, Guellec D, Mariette X et al (2016) Epidemiology of neurological manifestations in Sjögren’s syndrome: data from the French ASSESS Cohort. RMD open 2:e000179. https://doi.org/10.1136/rmdopen-2015-000179

    Article  PubMed  PubMed Central  Google Scholar 

  19. Phuljhele S, Pujari A, Obedulla H et al (2019) Atypical presentation of primary Sjögren’s syndrome as optic neuritis. Can J Ophthalmol 54:e30–e32

    Article  Google Scholar 

  20. Pars K, Pul R, Schwenkenbecher P et al (2017) Cerebrospinal fluid findings in neurological diseases associated with Sjögren’s syndrome. Eur Neurol 77:91–102. https://doi.org/10.1159/000454765

    Article  CAS  PubMed  Google Scholar 

  21. Hughes RAC, Bouche P, Cornblath DR et al (2006) European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European federation of neurological societies and the peripher. Eur J Neurol 13:326–332. https://doi.org/10.1111/j.1468-1331.2006.01278.x

    Article  CAS  PubMed  Google Scholar 

  22. Beeson PB (1994) Age and sex associations of 40 autoimmune diseases. Am J Med 96:457–462

    Article  CAS  Google Scholar 

  23. Alamanos Y, Tsifetaki N, Voulgari PV et al (2006) Epidemiology of primary Sjögren’s syndrome in north-west Greece, 1982–2003. Rheumatology 45:187–191. https://doi.org/10.1093/rheumatology/kei107

    Article  CAS  PubMed  Google Scholar 

  24. Izmirly PM, Buyon JP, Wan I et al (2018) The incidence and prevalence of adult primary Sjögren’s syndrome in New York county. Arthritis Care Res (Hoboken). https://doi.org/10.1002/acr.23707

    Article  Google Scholar 

  25. Lunn MP, Manji H, Choudhary PP et al (1999) Chronic inflammatory demyelinating polyradiculoneuropathy: a prevalence study in south east England. J Neurol Neurosurg Psychiatry 66:677–680

    Article  CAS  Google Scholar 

  26. Chio A, Cocito D, Bottacchi E et al (2007) Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy. J Neurol Neurosurg Psychiatry 78:1349–1353. https://doi.org/10.1136/jnnp.2007.114868

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Laughlin RS, Dyck PJ, Melton LJ et al (2009) Incidence and prevalence of CIDP and the association of diabetes mellitus. Neurology 73:39–45. https://doi.org/10.1212/WNL.0b013e3181aaea47

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Sivadasan A, Muthusamy K, Patel B et al (2017) Clinical spectrum, therapeutic outcomes, and prognostic predictors in Sjogren’s syndrome-associated neuropathy. Ann Indian Acad Neurol 20:278–283. https://doi.org/10.4103/aian.AIAN_116_17

    Article  PubMed  PubMed Central  Google Scholar 

  29. Rajabally YA, Attarian S, Delmont E (2020) Evolving immunologic perspectives in chronic inflammatory demyelinating polyneuropathy. J Inflamm Res 13:543–549

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany

    Tabea Seeliger, Stefan Gingele, Lena Bönig, Franz Felix Konen, Sonja Körner, Martin Stangel & Thomas Skripuletz

  2. Department of Otolaryngology, Hannover Medical School, Hannover, Germany

    Nils Prenzler

  3. Department of Rheumatology and Immunology, Hannover Medical School, Hannover, Germany

    Thea Thiele, Diana Ernst & Torsten Witte

Authors
  1. Tabea Seeliger
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Stefan Gingele
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Lena Bönig
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Franz Felix Konen
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Sonja Körner
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Nils Prenzler
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Thea Thiele
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Diana Ernst
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Torsten Witte
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Martin Stangel
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Thomas Skripuletz
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Substantial contributions were made by TSk, TW, MS, DE, TT, NP and SK to conception and design of the work, by FK, LB, SG, NP and TSe to data acquisition, by TSe, SG, TT, DE and LB to data analysis and by TSe, TSk, TW, MS, SK to data interpretation.

Corresponding author

Correspondence to Thomas Skripuletz.

Ethics declarations

Conflicts of interest

The authors declare that there are no conflicts of interest or competing interests regarding the publication of this paper.

Ethics approval

The local Ethics Committee of the Hannover Medical School approved of this study 8172_BO_K_2018. The study was undertaken according to the Declaration of Helsinki.

Consent to participate

All patients gave their informed consent to participate.

Consent for publication

All patients gave their informed consent for publication.

Supplementary Information

Below is the link to the electronic supplementary material.

415_2021_10459_MOESM1_ESM.tif

Supplementary file1 (TIF 725 KB) Supplementary Figure 1 Initial symptoms reported by CIDP patients with (A) and without Sjögren’s syndrome (B).

415_2021_10459_MOESM2_ESM.xlsx

Supplementary file2 (XLSX 14 KB) Supplementary Table 1 Nerve conduction studies in absolute values and significance levels for CIDP patients with and without Sjögren’s syndrome.

415_2021_10459_MOESM3_ESM.xlsx

Supplementary file3 (XLSX 11 KB) Supplementary Table 2 Cerebrospinal fluid (CSF) analysis compared in patients with and without Sjögren’s syndrome.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Seeliger, T., Gingele, S., Bönig, L. et al. CIDP associated with Sjögren’s syndrome. J Neurol 268, 2908–2912 (2021). https://doi.org/10.1007/s00415-021-10459-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-021-10459-z

Keywords

Search

Navigation