Clinical Features

  • Yoshiyuki Arinuma
  • Shunsei Hirohata
Chapter
First Online:

Abstract

Clinical features in neuropsychiatric systemic lupus erythematosus (NPSLE) include a variety of manifestations, which are too complicated to understand. In 1999, the American college of rheumatology (ACR) proposed nomenclature and case definitions of NPSLE as nosology for clinical descriptions and research. Clinically, NPSLE is classified into 2 different categories; one is diffuse psychiatric/neuropsychological syndromes and the other is neurologic syndromes. This discrimination is not always advantageous for clinical practice including a diagnosis and therapeutic intervention. Moreover, the severities of each manifestation differ among NPSLE. For example, acute confusional state is very severe form with poor prognosis, whereas headache, mood disorders, anxiety disorders and cognitive dysfunction are sometimes mild, and are also common in individuals without SLE. Also, we need to remember that NPSLE can be developed even in the absence of systemic disease activities. Finally, some neuropsychiatric manifestations have not been adequately defined in the ACR nomenclature.

Keywords

NPSLE ACR nomenclature Diffuse psychiatric/neuropsychological Syndromes Acute confusional state Neurologic syndromes 
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Supplementary material

451310_1_En_5_MOESM1_ESM.avi (6.1 mb)
Video 5.1 An SLE patient with acute confusional state. A 32-year-old female patient fell into a coma soon after admission. She kept opening her mouth with flattering her tongue, considered to be oral myoclonus (AVI 6254 kb)

Reference

  1. 1.
    Hebra F, et al. On diseases of the skin, including the exanthemata. London: The New Sydenham Society; 1874. p. 14–47.Google Scholar
  2. 2.
    Unterman A, et al. Neuropsychiatric syndromes in systemiclupuserythematosus: a meta-analysis. Semin Arthritis Rheum. 2011;41:1–11.CrossRefPubMedGoogle Scholar
  3. 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.CrossRefGoogle Scholar
  4. 4.
    Zirkzee E, et al. Mortality in neuropsychiatric systemic lupus erythematosus (NPSLE). Lupus. 2014;23:31–8.CrossRefPubMedGoogle Scholar
  5. 5.
    Tay S, et al. Active disease is independently associated with more severe anxiety rather than depressive symptoms in patients with systemic lupus erythematosus. Lupus. 2015;24:1392–9.CrossRefPubMedGoogle Scholar
  6. 6.
    Maneeton B, et al. Prevalence and predictors of depression in patients with systemic lupus erythematosus: a cross-sectional study. Neuropsychiatr Dis Treat. 2013;9:799–804.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Bertsias GK, et al. 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.CrossRefPubMedGoogle Scholar
  8. 8.
    Ainiala H, et al. The prevalence of neuropsychiatric syndromes in systemic lupus erythematosus. Neurology. 2001;57:496–500.CrossRefPubMedGoogle Scholar
  9. 9.
    Sung SC, et al. The impact of chronic depression on acute and long-term outcomes in a randomized trial comparing selective serotonin reuptake inhibitor Monotherapy versus each of 2 different antidepressant medication combinations. J Clin Psychiatry. 2012;73:967–76.CrossRefPubMedGoogle Scholar
  10. 10.
    Meszaros ZS, et al. Psychiatric symptoms in systemic lupus erythematosus: a systematic review. J Clin Psychiatry. 2012;73:993–1001.CrossRefPubMedGoogle Scholar
  11. 11.
    Hanly JG, et al. Cognitive function in systemic lupus erythematosus: results of a 5-year prospective study. Arthritis Rheum. 1997;40:1542–3.CrossRefPubMedGoogle Scholar
  12. 12.
    Waterloo K, et al. Neuropsychological function in systemic lupus erythematosus: a five-year longitudinal study. Rheumatology (Oxford). 2002;41:411–5.CrossRefGoogle Scholar
  13. 13.
    Hanly JG, et al. Management of neuropsychiatric lupus. Best Pract Res Clin Rheumatol. 2005;19:799–821.CrossRefPubMedGoogle Scholar
  14. 14.
    Brey RL, et al. Neuropsychiatric syndromes in lupus: prevalence using standardized definitions. Neurology. 2002;58:1214–20.CrossRefPubMedGoogle Scholar
  15. 15.
    Sanna G, et al. Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies. J Rheumatol. 2003;30:985–92.PubMedGoogle Scholar
  16. 16.
    Hanly JG, et al. Neuropsychiatric events in systemic lupus erythematosus: attribution and clinical significance. J Rheumatol. 2004;31:2156–62.PubMedGoogle Scholar
  17. 17.
    Mok CC, et al. Neuropsychiatric manifestations and their clinical associations in southern Chinese patients with systemic lupus erythematosus. J Rheumatol. 2001;28:766–71.PubMedGoogle Scholar
  18. 18.
    Tomietto P, et al. General and specific factors associated with severity of cognitive impairment in systemic lupus erythematosus. Arthritis Rheum. 2007;57:1461–72.CrossRefPubMedGoogle Scholar
  19. 19.
    Lim L, et al. Psychiatric and neurological manifestations in systemic lupus erythematosus. Q J Med. 1988;66:27–38.PubMedGoogle Scholar
  20. 20.
    Harboe E, et al. Fatigue is associated with cerebral white matter hyperintensities in patients with systemic lupus erythematosus. J Neurol Neurosurg Psychiatry. 2008;79:199–201.CrossRefPubMedGoogle Scholar
  21. 21.
    Kozora E, et al. Major life stress, coping styles, and social support in relation to psychological distress in patients with systemic lupus erythematosus. Lupus. 2005;14:363–72.CrossRefPubMedGoogle Scholar
  22. 22.
    Wekking EM. Psychiatric symptoms in systemic lupus erythematosus: an update. Psychosom Med. 1993;55:219–28.CrossRefPubMedGoogle Scholar
  23. 23.
    Bachen EA, et al. Prevalence of mood and anxiety disorders in women with systemic lupus erythematosus. Arthritis Rheum. 2009;61:822–9.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Líndal E, et al. Psychiatric disorders among subjects with systemic lupus erythematosus in an unselected population. Scand J Rheumatol. 1995;24:346–51.CrossRefPubMedGoogle Scholar
  25. 25.
    Pego-Reigosa JM, et al. Psychosis due to systemic lupus erythematosus: characteristics and long-term outcome of this rare manifestation of the disease. Rheumatology (Oxford). 2008;47:1498–502.CrossRefGoogle Scholar
  26. 26.
    Kohen M, et al. Lupus psychosis: differentiation from the steroid-induced state. Clin Exp Rheumatol. 1993;11:323–6.PubMedGoogle Scholar
  27. 27.
    Denburg SD, et al. Corticosteroids and neuropsychological functioning in patients with systemic lupus erythematosus. Arthritis Rheum. 1994;37:1311–20.CrossRefPubMedGoogle Scholar
  28. 28.
    Wysenbeek AJ, et al. Acute central nervous system complications after pulse steroid therapy in patients with systemic lupus erythematosus. J Rheumatol. 1990;17:1695–6.PubMedGoogle Scholar
  29. 29.
    Chau SY, et al. Factors predictive of corticosteroid psychosis in patients with systemic lupus erythematosus. Neurology. 2003;61:104–7.CrossRefPubMedGoogle Scholar
  30. 30.
    Shimizu Y, et al. Post-steroid neuropsychiatric manifestations are significantly more frequent in SLE compared with other systemic autoimmune diseases and predict better prognosis compared with de novo neuropsychiatric SLE. Autoimmun Rev. 2016;15:786–94.CrossRefPubMedGoogle Scholar
  31. 31.
    Sergent JS, et al. Central nervous system disease in systemic lupus erythematosus. Therapy and prognosis. Am J Med. 1975;58:644–54.CrossRefPubMedGoogle Scholar
  32. 32.
    Baizabal-Carvallo JF, et al. Clinical characteristics and outcomes of the meningitides in systemic lupus erythematosus. Eur Neurol. 2009;61:143–8.CrossRefPubMedGoogle Scholar
  33. 33.
    Sands ML, et al. Recurrent aseptic meningitis followed by transverse myelitis as a presentation of systemic lupus erythematosus. J Rheumatol. 1988;15:862–4.PubMedGoogle Scholar
  34. 34.
    Suzuki Y, et al. Severe cerebral and systemic necrotizing vasculitis developing during pregnancy in a case of systemic lupus erythematosus. J Rheumatol. 1990;17:1408–11.PubMedGoogle Scholar
  35. 35.
    Marin OS, et al. Cerebral vasculitis. Presenting as a meningoencephalitis. A case of systemic lupus erythematosus. Del Med J. 1973;45:315–9.PubMedGoogle Scholar
  36. 36.
    Ostensen M, et al. Nonsteroidal anti-inflammatory drugs in systemic lupus erythematosus. Lupus. 2000;9:566–72.CrossRefPubMedGoogle Scholar
  37. 37.
    Escalante A, et al. Trimethoprim-sulfamethoxasole induced meningitis in systemic lupus erythematosus. J Rheumatol. 1992;19:800–2.PubMedGoogle Scholar
  38. 38.
    Bruner KE, et al. Trimethoprim-sulfamethoxazole-induced aseptic meningitis-not just another sulfa allergy. Ann Allergy Asthma Immunol. 2014;113:520–6.CrossRefPubMedGoogle Scholar
  39. 39.
    Sciascia S, et al. The estimated frequency of antiphospholipid antibodies in young adults with cerebrovascular events: a systematic review. Ann Rheum Dis. 2015;74:2028–33.CrossRefPubMedGoogle Scholar
  40. 40.
    Petri M, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64:2677–86.CrossRefPubMedPubMedCentralGoogle Scholar
  41. 41.
    Holmqvist M, et al. Stroke in systemic lupus erythematosus: a meta-analysis of population-based cohort studies. RMD Open. 2015;1:e000168.CrossRefPubMedPubMedCentralGoogle Scholar
  42. 42.
    Kitagawa Y, et al. Stroke in systemic lupus erythematosus. Stroke. 1990;21:1533–9.CrossRefPubMedGoogle Scholar
  43. 43.
    Mikdashi J, et al. Baseline disease activity, hyperlipidemia, and hypertension are predictive factors for ischemic stroke and stroke severity in systemic lupus erythematosus. Stroke. 2007;38:281–5.CrossRefPubMedGoogle Scholar
  44. 44.
    Toloza SMA, et al. Systemic lupus erythematosus in a multiethnic US cohort (LUMINA). XXIII. Baseline predictors of vascular events. Arthritis Rheum. 2004;50:3947–57.CrossRefPubMedGoogle Scholar
  45. 45.
    Csépány T, et al. MRI findings in central nervous system systemic lupus erythematosus are associated with immunoserological parameters and hypertension. J Neurol. 2003;250:1348–54.CrossRefPubMedGoogle Scholar
  46. 46.
    Morelli S, et al. Left-sided heart valve abnormalities and risk of ischemic cerebrovascular accidents in patients with systemic lupus erythematosus. Lupus. 2003;12:805–12.CrossRefPubMedGoogle Scholar
  47. 47.
    Petri M, et al. Plasma homocysteine as a risk factor for atherothrombotic events in systemic lupus erythematosus. Lancet. 1996;348:1120–4.CrossRefPubMedGoogle Scholar
  48. 48.
    Esdaile JM, et al. Traditional Framingham risk factors fail to fully account for accelerated atherosclerosis in systemic lupus erythematosus. Arthritis Rheum. 2001;44:2331–7.CrossRefPubMedGoogle Scholar
  49. 49.
    Bessant R, et al. Risk of coronary heart disease and stroke in a large British cohort of patients with systemic lupus erythematosus. Rheumatology (Oxford). 2004;43:924–9.CrossRefGoogle Scholar
  50. 50.
    Urowitz MB, et al. Atherosclerotic vascular events in a multinational inception cohort of systemic lupus erythematosus. Arthritis Care Res (Hoboken). 2010;62:881–7.CrossRefGoogle Scholar
  51. 51.
    Roldan CA, et al. Libman-sacks endocarditis and embolic cerebrovascular disease. JACC Cardiovasc Imaging. 2013;6:973–83.CrossRefPubMedPubMedCentralGoogle Scholar
  52. 52.
    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.CrossRefPubMedGoogle Scholar
  53. 53.
    Magro Checa C, et al. Demyelinating disease in SLE: is it multiple sclerosis or lupus? Best Pract Res Clin Rheumatol. 2013;27:405–24.CrossRefPubMedGoogle Scholar
  54. 54.
    Bertsias GK, et al. Pathogenesis, diagnosis and management of neuropsychiatric SLE manifestations. Nat Rev Rheumatol. 2010;6:358–67.CrossRefPubMedGoogle Scholar
  55. 55.
    Wingerchuk DM, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85:177–89.CrossRefPubMedPubMedCentralGoogle Scholar
  56. 56.
    Hanly JG, et al. Headache in systemic lupus erythematosus: results from a prospective, international inception cohort study. Arthritis Rheum. 2013;65:2887–97.CrossRefPubMedGoogle Scholar
  57. 57.
    Mitsikostas DD, et al. A meta-analysis for headache in systemic lupus erythematosus: the evidence and the myth. Brain. 2004;127:1200–9.CrossRefPubMedGoogle Scholar
  58. 58.
    Omdal R, et al. Somatic and psychological features of headache in systemic lupus erythematosus. J Rheumatol. 2001;28:772–9.PubMedGoogle Scholar
  59. 59.
    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.CrossRefGoogle Scholar
  60. 60.
    Baizabal-Carvallo JF, et al. Movement disorders in systemic lupus erythematosus and the antiphospholipid syndrome. J Neural Transm. 2013;120:1579–89.CrossRefPubMedGoogle Scholar
  61. 61.
    Cervera R, et al. Chorea in the antiphospholipid syndrome. Clinical, radiologic, and immunologic characteristics of 50 patients from our clinics and the recent literature. Medicine (Baltimore). 1997;76:203–12.CrossRefGoogle Scholar
  62. 62.
    Asherson RA, et al. Antiphospholipid antibodies and chorea. J Rheumatol. 1988;15:377–9.PubMedGoogle Scholar
  63. 63.
    Orzechowski NM, et al. Antiphospholipid antibody-associated chorea. J Rheumatol. 2008;35:2165–70.CrossRefPubMedGoogle Scholar
  64. 64.
    González-Duarte A, et al. Clinical description of seizures in patients with systemic lupus erythematosus. Eur Neurol. 2008;59:320–3.CrossRefPubMedGoogle Scholar
  65. 65.
    Appenzeller S, et al. Epileptic seizures in systemic lupus erythematosus. Neurology. 2004;63:1808–12.CrossRefPubMedGoogle Scholar
  66. 66.
    Andrade RM, et al. Seizures in patients with systemic lupus erythematosus: data from LUMINA, a multiethnic cohort (LUMINA LIV). Ann Rheum Dis. 2008;67:829–34.CrossRefPubMedGoogle Scholar
  67. 67.
    Herranz MT, et al. Association between antiphospholipid antibodies and epilepsy in patients with systemic lupus erythematosus. Arthritis Rheum. 1994;37:568–71.CrossRefPubMedGoogle Scholar
  68. 68.
    Mackworth-Young CG, et al. Epilepsy: an early symptom of systemic lupus erythematosus. J Neurol Neurosurg Psychiatry. 1985;48:185.CrossRefPubMedPubMedCentralGoogle Scholar
  69. 69.
    Florica B, et al. Peripheral neuropathy in patients with systemic lupus Erythematosus. Semin Arthritis Rheum. 2011;41:203–11.CrossRefPubMedGoogle Scholar
  70. 70.
    Oomatia A, et al. Peripheral neuropathies in systemic lupus erythematosus: clinical features, disease associations, and immunologic characteristics evaluated over a twenty-five-year study period. Arthritis Rheumatol. 2014;66:1000–9.CrossRefPubMedGoogle Scholar
  71. 71.
    Xianbin W, et al. Peripheral neuropathies due to systemic lupus erythematosus in China. Medicine (Baltimore). 2015;94:e625.CrossRefGoogle Scholar
  72. 72.
    Toledano P, et al. Peripheral nervous system involvement in systemic lupus erythematosus: prevalence, clinical and immunological characteristics, treatment and outcome of a large cohort from a single centre. Autoimmun Rev. 2017;16:750–5.CrossRefPubMedGoogle Scholar
  73. 73.
    Keane JR. Eye movement abnormalities in systemic lupus erythematosus. Arch Neurol. 1995;52:1145–9.CrossRefPubMedGoogle Scholar
  74. 74.
    Saleh Z, et al. Cranial nerve VI palsy as a rare initial presentation of systemic lupus erythematosus: case report and review of the literature. Lupus. 2010;19:201–5.CrossRefPubMedGoogle Scholar
  75. 75.
    Sivaraj RR, et al. Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford). 2007;46:1757–62.CrossRefGoogle Scholar
  76. 76.
    Feinglass EJ, et al. Neuropsychiatric manifestations of systemic lupus erythematosus: diagnosis, clinical spectrum, and relationship to other features of the disease. Medicine (Baltimore). 1976;55:323–39.CrossRefGoogle Scholar
  77. 77.
    Omdal R, et al. Autonomic function in systemic lupus erythematosus. Lupus. 1994;3:413–7.CrossRefPubMedGoogle Scholar
  78. 78.
    Gamez-Nava JI, et al. Autonomic dysfunction in patients with systemic lupus erythematosus. J Rheumatol. 1998;25:1092–6.PubMedGoogle Scholar
  79. 79.
    Hogarth MB, et al. Cardiovascular autonomic function in systemic lupus erythematosus. Lupus. 2002;11:308–12.CrossRefPubMedGoogle Scholar
  80. 80.
    Shalimar, et al. Autonomic dysfunction in systemic lupus erythematosus. Rheumatol Int. 2006;26:837–40.CrossRefPubMedGoogle Scholar
  81. 81.
    Bekircan-Kurt CE, et al. The course of myasthenia gravis with systemic lupus erythematosus. Eur Neurol. 2014;72:326–9.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Yoshiyuki Arinuma
    • 1
  • Shunsei Hirohata
    • 2
    • 3
  1. 1.Department of Rheumatology and Infectious diseasesKitasato University School of MedicineSagamiharaJapan
  2. 2.Department of RheumatologyNobuhara HospitalTatsunoJapan
  3. 3.Department of Rheumatology & Infectious DiseasesKitasato University School of MedicineSagamiharaJapan

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