Skip to main content

Epidemiology of Neuropsychiatric Systemic Lupus Erythematosus

  • Chapter
  • First Online:

Abstract

A variety of neuropsychiatric manifestations are observed in patients with SLE. The American College of Rheumatology (ACR) developed standardized nomenclature and case definitions for neuropsychiatric involvement in SLE (NPSLE) in 1999. One of the problems in the 1999 ACR classification is the inclusion of milder, less specific and more subjective manifestations such as headache, mild cognitive dysfunction and mood disorders, which resulted in an enormous variation in the prevalence between studies. Another critical point of the ACR classification is the lack of a number of other neurological manifestations, such as neuromyelitis optica spectrum disorders and reversible focal neurological deficits mimicking cerebrovascular disease. Steroid psychosis is sometimes a difficult differential diagnosis, but not necessarily an exclusion, of lupus psychosis. CSF IL-6 might be one of the surrogate markers to detect patients with headache, cognitive dysfunction and mood disorders, requiring immunosuppressive therapy.

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

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Gibson T, Myers AR. Nervous system involvement in systemic lupus erythematosus. Ann Rheum Dis. 1975;35:398–406.

    Article  CAS  PubMed  Google Scholar 

  2. Harris EN, Hughes GR. Cerebral disease in systemic lupus erythematosus. Springer Semin Immunopathol. 1985;8:251–66.

    Article  CAS  PubMed  Google Scholar 

  3. ACR Ad Hoc Committee on Neuropsychiatric Lupus Nomenclature. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum. 1999;42:599–608.

    Article  Google Scholar 

  4. Cohen AS, Canoso JJ. Criteria for the classification of systemic lupus erythematosus-status 1972. Arthritis Rheum. 1972;15:540–3.

    Article  CAS  PubMed  Google Scholar 

  5. Tan EM, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25:1271–7.

    Article  CAS  PubMed  Google Scholar 

  6. Grigor R, et al. Systemic lupus erythematosus. Ann Rheum Dis. 1978;37:121–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Kremer JM, et al. Non-organic, non-psychotic psychopathology (NONPP) in patients with systemic lupus erythematosus. Semin Arthritis Rheum. 1981;11:182–9.

    Article  CAS  PubMed  Google Scholar 

  8. Zvaifler NJ, Bluestein HG. The pathogenesis of central nervous system manifestations of systemic lupus erythematosus. Arthritis Rheum. 1982;25:862–6.

    Article  CAS  PubMed  Google Scholar 

  9. Arinuma Y, et al. Association of cerebrospinal fluid anti-NR2 glutamate receptor antibodies with diffuse neuropsychiatric systemic lupus erythematosus. Arthritis Rheum. 2008;58:1130–5.

    Article  CAS  PubMed  Google Scholar 

  10. Bertsias GK, Iet a. EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs. Ann Rheum Dis. 2010;69:2074–82.

    Article  CAS  PubMed  Google Scholar 

  11. Steup-Beekman GM, et al. Neuropsychiatric manifestations in patients with systemic lupus erythematosus: epidemiology and radiology pointing to an immune-mediated cause. Ann Rheum Dis. 2013;72(Suppl 2):ii76–9.

    Article  CAS  PubMed  Google Scholar 

  12. Govoni M, et al. The diagnosis and clinical management of the neuropsychiatric manifestations of lupus. J Autoimmun. 2016;74:41–72.

    Article  CAS  PubMed  Google Scholar 

  13. Ainiala H, et al. Validity of the new American College of Rheumatology criteria for neuropsychiatric lupus syndromes: a population-based evaluation. Arthritis Rheum. 2001;45:419–23.

    Article  CAS  PubMed  Google Scholar 

  14. Brey RL, et al. Neuropsychiatric syndromes in lupus: prevalence using standardized definitions. Neurology. 2002;58:1214–20.

    Article  CAS  PubMed  Google Scholar 

  15. Hanly JG, et al. Systemic lupus international collaborating clinics, neuropsychiatric events at the time of diagnosis of systemic lupus erythematosus: an international inception cohort study. Arthritis Rheum. 2007;56:265–73.

    Article  CAS  PubMed  Google Scholar 

  16. Bertsias GK, Boumpas DT. Pathogenesis, diagnosis and management of neuropsychiatric SLE manifestations. Nat Rev Rheumatol. 2010;6:358–67.

    Article  PubMed  Google Scholar 

  17. Sanna G, et al. Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies. J Rheumatol. 2003;30:985–92.

    PubMed  Google Scholar 

  18. Cervera R, et al. European working party on systemic lupus erythematosus, lessons from the “euro-lupus cohort”. Ann Med Interne. 2002;153:530–6.

    Google Scholar 

  19. Unterman A, et al. Neuropsychiatric syndromes in systemic lupus erythematosus: a meta-analysis. Semin Arthritis Rheum. 2011;41:1–11.

    Article  PubMed  Google Scholar 

  20. Borowoy AM, et al. Neuropsychiatric lupus: the prevalence and autoantibody associations depend on the definition: results from the 1000 faces of lupus cohort. Semin Arthritis Rheum. 2012;42:179–85.

    Article  PubMed  Google Scholar 

  21. Amur S, et al. Sex differences and genomics in autoimmune diseases. J Autoimmun. 2012;38:J254–65.

    Article  CAS  PubMed  Google Scholar 

  22. Thumboo J, et al. A comparative study of the clinical manifestations of systemic lupus erythematosus in Caucasians in Rochester, Minnesota, and Chinese in Singapore, from 1980 to 1992. Arthritis Rheum. 2001;45:494–500.

    Article  CAS  PubMed  Google Scholar 

  23. Uribe AG, Alarcon GS. Ethnic disparities in patients with systemic lupus erythematosus. Curr Rheumatol Rep. 2003;5:364–9.

    Article  PubMed  Google Scholar 

  24. Fernandez M, et al., LUMINA Study Group. A multiethnic, multicenter cohort of patients with systemic lupus erythematosus (SLE) as a model for the study of ethnic disparities in SLE. Arthritis Rheum 2007; 57:576–584.

    Google Scholar 

  25. Gonzalez LA, et al. Impact of race and ethnicity in the course and outcome of systemic lupus erythematosus. Rheum Dis Clin N Am. 2014;40:433–54.

    Article  Google Scholar 

  26. Rivest C, et al. Association between clinical factors, socioeconomic status, and organ damage in recent onset systemic lupus erythematosus. J Rheumatol. 2000;27:680–4.

    CAS  PubMed  Google Scholar 

  27. Govoni M, et al., Italian Society of Rheumatology. Factors and comorbidities associated with first neuropsychiatric event in systemic lupus erythematosus: does a risk profile exist? A large multicentre retrospective cross-sectional study on 959 Italian patients. Rheumatology. 2012; 51:157–168.

    Google Scholar 

  28. Andrade RM, et al., LUMINA Study Group. Seizures in patients with systemic lupus erythematosus: data from LUMINA, a multiethnic cohort (LUMINA LIV). Ann Rheum Dis 2008; 67:829–834,

    Google Scholar 

  29. Mikdashi J, Handwerger B. Predictors of neuropsychiatric damage in systemic lupus erythematosus: data from the Maryland lupus cohort. Rheumatology. 2004;43:1555–60.

    Article  CAS  PubMed  Google Scholar 

  30. Tomietto P, et al. General and specific factors associated with severity of cognitive impairment in systemic lupus erythematosus. Arthritis Rheum. 2007;57:1461–72.

    Article  CAS  PubMed  Google Scholar 

  31. Hanly JG, et al. Seizure disorders in systemic lupus erythematosus results from an international, prospective, inception cohort study. Ann Rheum Dis. 2012;71:1502–9.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Bujan S, et al. Contribution of the initial features of systemic lupus erythematosus to the clinical evolution and survival of a cohort of Mediterranean patients. Ann Rheum Dis. 2003;62:859–65.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Mikdashi J, et al. Factors at diagnosis predict subsequent occurrence of seizures in systemic lupus erythematosus. Neurology. 2005;64:2102–7.

    Article  PubMed  Google Scholar 

  34. Appenzeller S, et al. Epileptic seizures in systemic lupus erythematosus. Neurology. 2004;63:1808–12.

    Article  PubMed  Google Scholar 

  35. Mok MY, et al. Antiphospholipid antibody profiles and their clinical associations in Chinese patients with systemic lupus erythematosus. J Rheumatol. 2005;32:622–8.

    CAS  PubMed  Google Scholar 

  36. McLaurin EY, et al. Predictors of cognitive dysfunction in patients with systemic lupus erythematosus. Neurology. 2005;64:297–303.

    Article  CAS  PubMed  Google Scholar 

  37. Hanly JG, et al. Autoantibodies as biomarkers for the prediction of neuropsychiatric events in systemic lupus erythematosus. Ann Rheum Dis. 2011;70:1726–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. Narvaez J, et al. Rituximab therapy in refractory neuropsychiatric lupus: current clinical evidence. Semin Arthritis Rheum. 2011;41:364–72.

    Article  CAS  PubMed  Google Scholar 

  39. Hirohata S, et al. Blood-brain barrier damages and intrathecal synthesis of anti-N-methyl-D-aspartate receptor NR2 antibodies in diffuse psychiatric/neuropsychological syndromes in systemic lupus erythematosus. Arthritis Res Ther. 2014;16:R77.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Gladman DD, et al. Systemic lupus erythematosus disease activity index 2000. J Rheumatol. 2002;29:288–91.

    PubMed  Google Scholar 

  41. Isenberg DA, et al. 2004 BILAG, development and initial validation of an updated version of the British isles lupus assessment Group’s disease activity index for patients with systemic lupus erythematosus. Rheumatologu. 2005;44:902–6.

    Article  CAS  Google Scholar 

  42. Brandt KD, Lessell S. Migrainous phenomena in systemic lupus erythematosus. Arthritis Rheum. 1978;21:7–16.

    Article  CAS  PubMed  Google Scholar 

  43. Toubi E, et al. Association of antiphospholipid antibodies with central nervous system disease in systemic lupus erythematosus. Am J Med. 1995;99:397–401.

    Article  CAS  PubMed  Google Scholar 

  44. Kimura M, et al. Reversible focal neurological deficits in systemic lupus erythematosus: report of 2 cases and review of the literature. J Neurol Sci. 2008;272:71–6.

    Article  PubMed  Google Scholar 

  45. Birnbaum J, et al. Distinct subtypes of myelitis in systemic lupus erythematosus. Arthritis Rheum. 2009;60:3378–87.

    Article  CAS  PubMed  Google Scholar 

  46. Sibbitt WL Jr, et al. The incidence and prevalence of neuropsychiatric syndromes in pediatric onset systemic lupus erythematosus. J Rheumatol. 2002;29:1536–42.

    PubMed  Google Scholar 

  47. Muscal E, Myones BL. The role of autoantibodies in pediatric neuropsychiatric systemic lupus erythematosus. Autoimmun Rev. 2007;6:215–7.

    Article  PubMed  Google Scholar 

  48. Brunner HI. Risk factors for damage in childhood-onset systemic lupuserythematosus: cumulative disease activity and medication use predict disease damage. Arthritis Rheum. 2002;46:436–44.

    Article  PubMed  Google Scholar 

  49. Brunner HI, et al. Difference in disease features between childhood-onsetand adult-onset systemic lupus erythematosus. Arthritis Rheum. 2008;58:556–62.

    Article  PubMed  Google Scholar 

  50. Tucker LB, et al. Adolescent onset of lupus results in more aggressive disease and worse outcomes: results of a nested matched case-control study within LUMINA, a multiethnic US cohort (LUMINA LVII). Lupus. 2008;17:314–22.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  51. Hoffman IEA, et al. Juvenile-onset systemic lupus erythemotosis: different clinical and serological pattern than adult-onset systemic lupus erythematosus. Ann Rheum Dis. 2009;68:412–5.

    Article  CAS  PubMed  Google Scholar 

  52. Benseler SM, Silverman ED. Neuropsychiatric involvement in pediatric systemic lupus erythematosus. Lupus. 2007;16:564–71.

    Article  CAS  PubMed  Google Scholar 

  53. Harel L, et al. Neuropsychiatric manifestations in pediatric systemic lupus erythematosus and association with antiphospholipid antibodies. J Rheumatol. 2006;33:1873–7.

    CAS  PubMed  Google Scholar 

  54. Brunner HI, et al. Initial validation of the pediatric automated neuropsychological assessment metrics for childhood-onset systemic lupus erythematosus. Arthritis Rheum. 2007;57:1174–82.

    Article  PubMed  Google Scholar 

  55. Muscal E, Brey RL. Neurological manifestations of systemic lupus erythematosus in children and adults. Neurol Clin. 2010;28:61–73.

    Article  PubMed  PubMed Central  Google Scholar 

  56. Appenzeller S, et al. Acute psychosis in systemic lupus erythematosus. Rheumatol Int. 2008;28:237–43.

    Article  PubMed  Google Scholar 

  57. Chau SY, Mok CC. Factors predictive of corticosteroid psychosis in patients with systemic lupus erythematosus. Neurology. 2003;61:104–7.

    Article  PubMed  Google Scholar 

  58. Nishimura K, et al. Blood-brain barrier damage as a risk factor for corticosteroid-induced psychiatric disorders in systemic lupus erythematosus. Psychoneuroendocrinology. 2008;33:395–403.

    Article  CAS  PubMed  Google Scholar 

  59. Yurkovich M, et al. Overall and cause-specific mortality in patients with systemic lupus erythematosus: a meta-analysis of observational studies. Arthritis Care Res (Hoboken). 2014;66:608–16.

    Article  Google Scholar 

  60. Zirkzee EJ, et al. Mortality in neuropsychiatric systemic lupus erythematosus (NPSLE). Lupus. 2014;23:31–8.

    Article  CAS  PubMed  Google Scholar 

  61. Abe G, et al. Brain MRI in patients with acute confusional state of diffuse psychiatric/neuropsychological syndromes in systemic lupus erythematosus. Mod Rheumatol. 2017;27:278–83.

    Article  CAS  PubMed  Google Scholar 

  62. Unterman A, et al. Neuropsychiatric syndromes in systemic lupus erythematosus: a meta-analysis. Semin Arthritis Rheum. 2011;41:1–11.

    Article  PubMed  Google Scholar 

  63. Fragoso-Loyo H, et al. Inflammatory profile in cerebrospinal fluid of patients with headache as a manifestation of neuropsychiatric systemic lupus erythematosus. Rheumatology (Oxford). 2013;52:2218–22.

    Article  CAS  Google Scholar 

  64. Hirohata S, et al. Accuracy of cerebrospinal fluid IL-6 testing for diagnosis of lupus psychosis. A multicenter retrospective study. Clin Rheumatol. 2009;28:1319–23.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Hirohata, S. (2018). Epidemiology of Neuropsychiatric Systemic Lupus Erythematosus. In: Hirohata, S. (eds) Neuropsychiatric Systemic Lupus Erythematosus. Springer, Cham. https://doi.org/10.1007/978-3-319-76496-2_1

Download citation

Publish with us

Policies and ethics