Skip to main content

Advertisement

Log in

Characterization of cognitive dysfunction in Sjögren’s syndrome patients

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Sjögren’s syndrome is an autoimmune disorder primarily affecting women, with decreased saliva and tear production as the principal characteristic. Cognitive, neurological, and psychiatric disorders also are associated with Sjögren’s. The present study addressed the hypothesis that patients with Sjögren’s syndrome differ significantly from matched controls in the prevalence and impact of a number of neuropsychiatric abnormalities. Sjögren’s patients and controls (n = 37 per group) underwent medical and psychiatric evaluation, demographic assessments, quality of life and symptom evaluation, and extensive testing of cognitive function and memory. Patients and controls were closely matched for age, gender distribution, verbal IQ, marital status, educational level, employment status, and current/past medical or psychiatric history. On most subjective self-ratings, Sjögren’s patients reported greater fatigue, impaired physical functioning, feeling depressed, and autonomic symptomatology compared to controls. Impaired memory was described mainly as loss of thought continuity in the midst of a task or activity. However, the majority of objective measures of cognition, psychomotor function, and memory showed minimal differences between groups. Sjögren’s patients rate themselves as impaired on multiple ratings of emotional, cognitive, and physical function, but objective measures of cognition reveal fewer substantive differences between patients and matched controls. Sjögren’s patients perceive deteriorated physical function over time, but they achieve a level of functioning comparable to controls despite the burden of their illness.

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

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

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

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Anaya JM, Talal N (1999) Sjögren’s syndrome comes of age. Semin Arthritis Rheum 28:35–39

    Article  Google Scholar 

  2. Bayetto K, Logan RM (2010) Sjögren’s syndrome: a review of aetiology, pathogenesis, diagnosis and management. Aust Dent J 55(1Suppl):39–47

    Article  PubMed  Google Scholar 

  3. Fox RI (2005) Sjögren’s Syndrome. Lancet 366:321–331

    Article  PubMed  CAS  Google Scholar 

  4. Kassan SS, Moutsopoulos HM (2004) Clinical manifestations and early diagnosis of Sjögren’s syndrome. Arch Intern Med 164:1275–1284

    Article  PubMed  Google Scholar 

  5. Cermak JM, Papas AS, Sullivan RM, Dana MR, Sullivan DA (2003) Nutrient intake in women with primary and secondary Sjögren’s syndrome. Eur J Clin Nutr 57:328–334

    Article  PubMed  CAS  Google Scholar 

  6. Cummins MJ, Papas A, Kammer GM, Fox PC (2003) Treatment of primary Sjögren’s syndrome with low-dose human interferon alfa administered by the oromucosal route: combined phase III results. Arthritis Rheum 49:585–593

    Article  PubMed  CAS  Google Scholar 

  7. Fox PC, Bowman SJ, Segal B et al (2008) Oral involvement in primary Sjögren syndrome. J Am ENT Assoc 139:1592–1601

    Article  Google Scholar 

  8. Papas AS, Fernancez MM, Castano RA, Gallagher SC, Trivedi M, Shrotriya RC (1998) Oral pilocarpine for symptomatic relief of dry mouth and dry eyes in patient with Sjögren’s syndrome. Adv Exp Med Biol 438:973–978

    Article  PubMed  CAS  Google Scholar 

  9. Ramos-Casals M, Tzioufas AG, Stone JH, Sisó A, Bosch X (2010) Treatment of primary Sjögren Syndrome: a systematic review. JAMA 304:452–460

    Article  PubMed  CAS  Google Scholar 

  10. Barendregt PJ, Visser MRM, Smets EMA, Tulen JHM, van den Meiracker AH, Boomsma F, Markusse HM (1998) Fatigue in primary Sjögren’s syndrome. Ann Rheum Dis 57:291–295

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  11. Strombeck B, Ekdahl C, Manthorpe R, Wikstrom I, Jacobsson L (2000) Health-related quality of life in primary Sjögren’s syndrome, rheumatoid arthritis and fibromyalgia compared to normal population data using SF-36. Scand J Rheumatol 29:20–28

    Article  PubMed  CAS  Google Scholar 

  12. Ng WF, Bowman SJ (2010) Primary Sjögren’s syndrome: too dry and too tired. Rheumatology 49:844–853

    Article  PubMed  Google Scholar 

  13. Haldorsen K, Bjelland I, Bolstad AI, Jonsson R, Brun JG (2011) A five-year prospective study of fatigue in primary Sjögren’s syndrome. Arthritis Res Ther 13:R167

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  14. Godaert GLR, Hartkamp A, Geenen R, Garssen A, Kruize AA, Bijlsma JMR, Derksen RHWM (2002) Fatigue in daily life in patients with primary Sjögren’s syndrome and systemic lupus erythematosus. Ann NY Acad Sci 966:320–326

    Article  PubMed  CAS  Google Scholar 

  15. Valtysdottir ST, Gudbjornsson B, Lindqvist U, Hallgren R, Hetta J (2000) Anxiety and depression in patients with primary Sjögren’s syndrome. J Rheum 27:165–169

    PubMed  CAS  Google Scholar 

  16. Valtysdottir ST, Gudbjornsson B, Hallgren R, Hetta J (2000) Psychological well-being in patients with primary Sjögren’s syndrome. Clin Exp Rheumatol 18:597–600

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  18. Govoni M, Padovan M, Rizzo N, Trotta F (2001) CNS involvement in primary Sjögren’s syndrome: prevalence, clinical aspects, diagnostic assessment and therapeutic approach. CNS Drugs 15:597–607

    Article  PubMed  CAS  Google Scholar 

  19. Mauch E, Voelk C, Kratzsch G, Krapf H et al (1994) Neurological and neuropsychiatric dysfunction in primary Sjögren’s syndrome. Acta Neurol Scand 89:31–35

    Article  PubMed  CAS  Google Scholar 

  20. Alexander EL (1991) Central nervous system disease in Sjögren’s syndrome. New insights into immunopathogenesis. Rheum Dis clin North Am 18:637–672

    Google Scholar 

  21. DeBattista C, Doghramji K, Menza MA, Rosenthal MH, Fieve RR (2003) Modafinil in Depression Study Group. Adjunct modafinil for the short-term treatment of fatigue and sleepiness in patients with major depressive disorder: a preliminary double-blind, placebo-controlled study. J Clin Psychiatry 64:1057–1064

    Article  PubMed  CAS  Google Scholar 

  22. Dinges DF, Weaver TE (2003) Effects of modafinil on sustained attention performance and quality of life in OSA patients with residual sleepiness while being treated with nCPAP. Sleep Med 4:393–402

    Article  PubMed  Google Scholar 

  23. Johnstone B, Pepmueller PH, Vieth AZ, Komatireddy G (1996) Effective treatment of neuropsychological deficits in Sjögren’s syndrome. Appl Neuropsychol 3:122–127

    Article  PubMed  CAS  Google Scholar 

  24. Le Guern V, Belin C, Henegar C et al (2010) Cognitive function and 99mTc-ECD brain SPECT are significantly correlated in patients with primary Sjögren syndrome: a case–control study. Ann Rheum Dis 69:132–137

    Article  PubMed  Google Scholar 

  25. Cox PD, Hales RE (1999) CNS Sjögren’s syndrome: an under-recognized and underappreciated neuropsychiatric disorder. J Neuropsych Clin Neurosci 11:241–247

    CAS  Google Scholar 

  26. Wyszynski AA, Wyszynski B (1993) Treatment of depression with fluoxetine in corticosteroid-dependent central nervous system Sjögren’s syndrome. Psychosomatics: J Consult Liaison Psychiatry 34:173–177

    Article  CAS  Google Scholar 

  27. Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, Schiødt M, Umehara H, Vivino F, Zhao Y, Dong Y, Greenspan D, Heidenreich AM, Helin P, Kirkham B, Kitagawa K, Larkin G, Li M, Lietman T, Lindegaard J, McNamara N, Sack K, Shirlaw P, Sugai S, Vollenweider C, Whitcher J, Wu A, Zhang S, Zhang W, Greenspan J, Daniels T (2012) Sjögren’s International Collaborative Clinical Alliance (SICCA) Research Groups. Arthritis Care Res 64:475–487

    Article  CAS  Google Scholar 

  28. Greenblatt DJ, Harmatz JS, Shapiro L, Engelhardt N, Gouthro TA, Shader RI (1991) Sensitivity to triazolam in the elderly. N Engl J Med 324:1691–1698

    Article  PubMed  CAS  Google Scholar 

  29. Kaplan GB, Greenblatt DJ, Ehrenberg BL, Goddard JE, Cotreau MM, Harmatz JS, Shader RI (1997) Dose dependent pharmacokinetics and psychomotor effects of caffeine in humans. J Clin Pharmacol 37:693–703

    Article  PubMed  CAS  Google Scholar 

  30. Scavone JM, Greenblatt DJ, Harmatz JS, Engelhardt N, Shader RI (1998) Pharmacokinetics and pharmacodynamics of diphenhydramine 25 mg in young and elderly volunteers. J Clin Pharmacol 38:603–609

    Article  PubMed  CAS  Google Scholar 

  31. Spitzer RL, Williams JB, Gibbon M, First MB (1992) The structured clinical interview for DSM-III-R (SCID). I: History, rationale, and description. Arch Gen Psychiatry 49:624–629

    Article  PubMed  CAS  Google Scholar 

  32. Spitzer RL, Williams JBW, Gibbon M, First MB. Structured clinical interview for DSM-III-R, patient edition/non-patient edition, (SCID-P/SCID-NP), Washington, D.C.: American Psychiatric Press, Inc., 1990

  33. Small GW (2002) The Memory Bible: an innovative strategy for keeping the brain young. Penguin, London

    Google Scholar 

  34. Holmes TH, Rahe RH (1967) Holmes-Rahe social readjustment rating scale. J Psychosom Res 11:213–218

    Article  PubMed  CAS  Google Scholar 

  35. Hollingshead AB (1975) Four factor index of social status. Yale University, New Haven

    Google Scholar 

  36. Krupp LB, LaRocca NG, Muir-Nash J, Steinberg AD (1989) The fatigue severity scale: application to patients with multiple sclerosis and systemic lupus erythematosus. Arch Neurol 46:1121–1123

    Article  PubMed  CAS  Google Scholar 

  37. Ware JEJ, Snow KK, Kosinski M, Gandek B (1993) SF-36 Health survey: manual and interpretation guide. The Health Institute, New England Medical Center

    Google Scholar 

  38. Radloff LS, Teri L (1986) Use of the center for epidemiological studies—depression scale with older adults. Clin Gerontol 5:119–136

    Article  Google Scholar 

  39. Greenblatt DJ, Harmatz JS, Engelhardt N, Shader RI (1989) Pharmacokinetic determinants of dynamic differences among three benzodiazepine hypnotics: flurazepam, temazepam, and triazolam. Arch Gen Psychiatry 46:326–332

    Article  PubMed  CAS  Google Scholar 

  40. Greeenblatt DJ, Harmatz J, Dorsey C, Shader RI (1988) Comparative single-dose kinetics and dynamics of lorazepam, alprazolam, prazepam, and placebo. Clin Pharmacol Ther 44:326–334

    Article  Google Scholar 

  41. Curtis-Prior PB (1996) Computerized methods of neuropsychological assessment. Br J Hosp Med 56:445–449

    PubMed  CAS  Google Scholar 

  42. Sahakian BJ, Owen AM (1992) Computerized assessment in neuropsychiatry using CANTAB. J R Soc Med 85:399–402

    PubMed Central  PubMed  CAS  Google Scholar 

  43. Fray PJ, Robbins TW (1996) CANTAB battery: proposed utility in neurotoxicology. Neurotoxicol Teratol 18:499–504

    Article  PubMed  CAS  Google Scholar 

  44. Bright P, Jaldow E, Kopelman MD (2002) The National Adult Reading Test as a measure of premorbid intelligence: a comparison with estimates derived from demographic variables. J Int Neuropsychol Soc 8:847–854

    Article  PubMed  Google Scholar 

  45. Gladsjo JA, Heaton RK, Palmer BW, Taylor MJ, Jeste DV (1999) Use of oral reading to estimate premorbid intellectual and neuropsychological functioning. J Int Neuro Psychol Soc 5:247–254

    CAS  Google Scholar 

  46. Birnbaum J (2010) Peripheral nervous system manifestations of Sjögren’s syndrome: clinical patterns, diagnostic paradigms, etiopathogenesis, and therapeutic strategies. Neurologist 16:287–297

    Article  PubMed  Google Scholar 

  47. Segal BM, Pogatchnik B, Holker E, Liu H, Sloan J, Rhodus N, Moser KL (2012) Primary Sjögren's syndrome: cognitive symptoms, mood, and cognitive performance. Acta Neurol Scand 125:272–278

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  48. Segal B, Bowman SJ, Fox PC et al (2009) Primary Sjögren’s syndrome: health experiences and predictors of health quality among patients in the United States. Health Qual Life Outcomes 7:46

    Article  PubMed Central  PubMed  Google Scholar 

Download references

Acknowledgments

This work was supported by a grant from the Sjögren’s Syndrome Foundation.

Disclosures

None.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to David J. Greenblatt.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Epstein, L.C., Masse, G., Harmatz, J.S. et al. Characterization of cognitive dysfunction in Sjögren’s syndrome patients. Clin Rheumatol 33, 511–521 (2014). https://doi.org/10.1007/s10067-013-2453-6

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-013-2453-6

Keywords

Navigation