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Central nervous system lupus

MedGen UID:
156265
•Concept ID:
C0752332
•
Disease or Syndrome
Synonyms: Central Nervous System Lupus; Central Nervous System Lupus Vasculitis; Central Nervous System Systemic Lupus Erythematosis; Lupus Vasculitis, Central Nervous System; Systemic Lupus Erythematosis, Central Nervous System
 
Monarch Initiative: MONDO:0043985

Definition

Inflammation that includes the brain, spinal cord and surrounding tissues secondary to systemic lupus erythematosus (SLE); it is associated with neurological and/or psychiatric features. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCentral nervous system lupus

Professional guidelines

PubMed

Neurological deficits during treatment with tumor necrosis factor-alpha antagonists.
Nozaki K, Silver RM, Stickler DE, Abou-Fayssal NG, Giglio P, Kamen DL, Daniel R, Judson MA
Am J Med Sci 2011 Nov;342(5):352-5. doi: 10.1097/MAJ.0b013e31822b7bb8. PMID: 21876428
Central nervous system lupus--diagnosis and treatment.
Hughes GR
J Rheumatol 1980 May-Jun;7(3):405-11. PMID: 7401072
Editorial: Treatment of central nervous system lupus erythematosus.
Sergent JS, Lockshin MD
Ann Intern Med 1974 Mar;80(3):413-4. doi: 10.7326/0003-4819-80-3-413_2. PMID: 4816188

Recent clinical studies

Etiology

Central Nervous System Systemic Lupus Erythematosus: Pathophysiologic, Clinical, and Imaging Features.
Ota Y, Srinivasan A, Capizzano AA, Bapuraj JR, Kim J, Kurokawa R, Baba A, Moritani T
Radiographics 2022 Jan-Feb;42(1):212-232. doi: 10.1148/rg.210045. PMID: 34990324
Management of inflammatory neurologic and psychiatric manifestations of systemic lupus erythematosus: A systematic review.
Papachristos DA, Oon S, Hanly JG, Nikpour M
Semin Arthritis Rheum 2021 Feb;51(1):49-71. Epub 2020 Dec 17 doi: 10.1016/j.semarthrit.2020.12.004. PMID: 33360230
HMGB1 in Systemic Lupus Erythematosus.
Liu T, Son M, Diamond B
Front Immunol 2020;11:1057. Epub 2020 May 27 doi: 10.3389/fimmu.2020.01057. PMID: 32536928Free PMC Article
Diagnosis, Monitoring, and Treatment of Systemic Lupus Erythematosus: A Systematic Review of Clinical Practice Guidelines.
Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A
Arthritis Care Res (Hoboken) 2015 Oct;67(10):1440-52. doi: 10.1002/acr.22591. PMID: 25778500
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.
Bertsias GK, Ioannidis JP, Aringer M, Bollen E, Bombardieri S, Bruce IN, Cervera R, Dalakas M, Doria A, Hanly JG, Huizinga TW, Isenberg D, Kallenberg C, Piette JC, Schneider M, Scolding N, Smolen J, Stara A, Tassiulas I, Tektonidou M, Tincani A, van Buchem MA, van Vollenhoven R, Ward M, Gordon C, Boumpas DT
Ann Rheum Dis 2010 Dec;69(12):2074-82. Epub 2010 Aug 19 doi: 10.1136/ard.2010.130476. PMID: 20724309

Diagnosis

Neuropsychiatric involvement in systemic lupus erythematosus: A review.
Carrión-Barberà I, Salman-Monte TC, Vílchez-Oya F, Monfort J
Autoimmun Rev 2021 Apr;20(4):102780. Epub 2021 Feb 18 doi: 10.1016/j.autrev.2021.102780. PMID: 33609799
Update οn the diagnosis and management of systemic lupus erythematosus.
Fanouriakis A, Tziolos N, Bertsias G, Boumpas DT
Ann Rheum Dis 2021 Jan;80(1):14-25. Epub 2020 Oct 13 doi: 10.1136/annrheumdis-2020-218272. PMID: 33051219
Lupus Vasculitis.
Calle-Botero E, Abril A
Curr Rheumatol Rep 2020 Aug 26;22(10):71. doi: 10.1007/s11926-020-00937-0. PMID: 32845454
Neuropsychiatric lupus: new mechanistic insights and future treatment directions.
Schwartz N, Stock AD, Putterman C
Nat Rev Rheumatol 2019 Mar;15(3):137-152. doi: 10.1038/s41584-018-0156-8. PMID: 30659245Free PMC Article
Diagnosis, Monitoring, and Treatment of Systemic Lupus Erythematosus: A Systematic Review of Clinical Practice Guidelines.
Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A
Arthritis Care Res (Hoboken) 2015 Oct;67(10):1440-52. doi: 10.1002/acr.22591. PMID: 25778500

Therapy

Management of inflammatory neurologic and psychiatric manifestations of systemic lupus erythematosus: A systematic review.
Papachristos DA, Oon S, Hanly JG, Nikpour M
Semin Arthritis Rheum 2021 Feb;51(1):49-71. Epub 2020 Dec 17 doi: 10.1016/j.semarthrit.2020.12.004. PMID: 33360230
Update οn the diagnosis and management of systemic lupus erythematosus.
Fanouriakis A, Tziolos N, Bertsias G, Boumpas DT
Ann Rheum Dis 2021 Jan;80(1):14-25. Epub 2020 Oct 13 doi: 10.1136/annrheumdis-2020-218272. PMID: 33051219
Lupus myelopathy.
Raciborska DD, Noyce AJ, Pyne D, Turner BP
Pract Neurol 2018 Feb;18(1):66-69. Epub 2017 Nov 2 doi: 10.1136/practneurol-2017-001756. PMID: 29097555
The role of infections in neuropsychiatric lupus.
Ribeiro FM, Signorelli F
Lupus 2017 Apr;26(5):490-496. doi: 10.1177/0961203317691375. PMID: 28394236
Corticosteroids in Lupus Nephritis and Central Nervous System Lupus.
Kamen DL, Zollars ES
Rheum Dis Clin North Am 2016 Feb;42(1):63-73, viii. Epub 2015 Oct 24 doi: 10.1016/j.rdc.2015.08.008. PMID: 26611551

Prognosis

Update οn the diagnosis and management of systemic lupus erythematosus.
Fanouriakis A, Tziolos N, Bertsias G, Boumpas DT
Ann Rheum Dis 2021 Jan;80(1):14-25. Epub 2020 Oct 13 doi: 10.1136/annrheumdis-2020-218272. PMID: 33051219
Advances in the diagnosis, pathogenesis and treatment of neuropsychiatric systemic lupus erythematosus.
Moore E, Huang MW, Putterman C
Curr Opin Rheumatol 2020 Mar;32(2):152-158. doi: 10.1097/BOR.0000000000000682. PMID: 31895125Free PMC Article
Neuropsychiatric lupus: new mechanistic insights and future treatment directions.
Schwartz N, Stock AD, Putterman C
Nat Rev Rheumatol 2019 Mar;15(3):137-152. doi: 10.1038/s41584-018-0156-8. PMID: 30659245Free PMC Article
Diagnosis, Monitoring, and Treatment of Systemic Lupus Erythematosus: A Systematic Review of Clinical Practice Guidelines.
Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A
Arthritis Care Res (Hoboken) 2015 Oct;67(10):1440-52. doi: 10.1002/acr.22591. PMID: 25778500
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.
Bertsias GK, Ioannidis JP, Aringer M, Bollen E, Bombardieri S, Bruce IN, Cervera R, Dalakas M, Doria A, Hanly JG, Huizinga TW, Isenberg D, Kallenberg C, Piette JC, Schneider M, Scolding N, Smolen J, Stara A, Tassiulas I, Tektonidou M, Tincani A, van Buchem MA, van Vollenhoven R, Ward M, Gordon C, Boumpas DT
Ann Rheum Dis 2010 Dec;69(12):2074-82. Epub 2010 Aug 19 doi: 10.1136/ard.2010.130476. PMID: 20724309

Clinical prediction guides

Microglia activation in the presence of intact blood-brain barrier and disruption of hippocampal neurogenesis via IL-6 and IL-18 mediate early diffuse neuropsychiatric lupus.
Nikolopoulos D, Manolakou T, Polissidis A, Filia A, Bertsias G, Koutmani Y, Boumpas DT
Ann Rheum Dis 2023 May;82(5):646-657. Epub 2023 Mar 10 doi: 10.1136/ard-2022-223506. PMID: 36898766Free PMC Article
Management of inflammatory neurologic and psychiatric manifestations of systemic lupus erythematosus: A systematic review.
Papachristos DA, Oon S, Hanly JG, Nikpour M
Semin Arthritis Rheum 2021 Feb;51(1):49-71. Epub 2020 Dec 17 doi: 10.1016/j.semarthrit.2020.12.004. PMID: 33360230
Advances in the diagnosis, pathogenesis and treatment of neuropsychiatric systemic lupus erythematosus.
Moore E, Huang MW, Putterman C
Curr Opin Rheumatol 2020 Mar;32(2):152-158. doi: 10.1097/BOR.0000000000000682. PMID: 31895125Free PMC Article
Corticosteroids in Lupus Nephritis and Central Nervous System Lupus.
Kamen DL, Zollars ES
Rheum Dis Clin North Am 2016 Feb;42(1):63-73, viii. Epub 2015 Oct 24 doi: 10.1016/j.rdc.2015.08.008. PMID: 26611551
Diagnosis, Monitoring, and Treatment of Systemic Lupus Erythematosus: A Systematic Review of Clinical Practice Guidelines.
Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A
Arthritis Care Res (Hoboken) 2015 Oct;67(10):1440-52. doi: 10.1002/acr.22591. PMID: 25778500

Recent systematic reviews

Suicidal behavior in patients with systematic lupus erythematosus: Systematic literature review and genetic linkage disequilibrium analysis.
Parperis K, Kyriakou A, Voskarides K, Chatzittofis A
Semin Arthritis Rheum 2022 Jun;54:151997. Epub 2022 Mar 19 doi: 10.1016/j.semarthrit.2022.151997. PMID: 35344734
Management of inflammatory neurologic and psychiatric manifestations of systemic lupus erythematosus: A systematic review.
Papachristos DA, Oon S, Hanly JG, Nikpour M
Semin Arthritis Rheum 2021 Feb;51(1):49-71. Epub 2020 Dec 17 doi: 10.1016/j.semarthrit.2020.12.004. PMID: 33360230
Neuropsychiatric Lupus Erythematosus: Future Directions and Challenges; a Systematic Review and Survey.
Zhang Y, Han H, Chu L
Clinics (Sao Paulo) 2020;75:e1515. Epub 2020 Apr 17 doi: 10.6061/clinics/2020/e1515. PMID: 32321114Free PMC Article
Diagnosis, Monitoring, and Treatment of Systemic Lupus Erythematosus: A Systematic Review of Clinical Practice Guidelines.
Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A
Arthritis Care Res (Hoboken) 2015 Oct;67(10):1440-52. doi: 10.1002/acr.22591. PMID: 25778500
Central nervous system manifestations of neonatal lupus: a systematic review.
Chen CC, Lin KL, Chen CL, Wong AM, Huang JL
Lupus 2013 Dec;22(14):1484-8. Epub 2013 Oct 18 doi: 10.1177/0961203313509294. PMID: 24142583

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