Psych · Consultation-liaison psychiatry
SLE and autoimmune neuropsychiatry
Also known as Neuropsychiatric SLE · NPSLE · Lupus psychosis · Lupus cerebritis · CNS lupus · Anti-ribosomal P · Autoimmune neuropsychiatry · SLE neuropsychiatric syndromes
Exam-exhaustive fellowship topic on neuropsychiatric systemic lupus erythematosus and related autoimmune neuropsychiatry — ACR 19 syndromes, attribution, inflammatory versus thrombotic mechanisms, anti-ribosomal P and anti-NR2 signals, EULAR-aligned treatment branches, steroid psychosis, and CL psychopharmacology. FRANZCP-primary, globally tagged.
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Target exams
Red flags
- New psychosis or acute confusional state in known or suspected SLE — attribute carefully, exclude infection, and co-manage with rheumatology urgently
- Focal neurology, seizure, or suspected stroke in SLE — activate stroke/neurology pathway and complete antiphospholipid work-up
- Do not escalate immunosuppression until infection (including opportunistic CNS infection) is actively excluded
- High-dose glucocorticoid mania or psychosis is iatrogenic until proven otherwise — coordinate steroid plan rather than automatic depot antipsychotic
- aPL-positive vascular phenotype needs antithrombotic strategy, not steroids alone
- Normal MRI does not exclude diffuse inflammatory NPSLE phenotypes
- Headache alone is rarely sufficient for aggressive cytotoxic escalation
Meet the patient
A 26-year-old woman with known SLE and lupus nephritis is admitted for a methylprednisolone pulse. On day four she is euphoric, talking rapidly, sleeping two hours a night, and has begun to believe the nursing staff are reading her notes to the government. The renal team have asked CL "to settle her." Her temperature is 37.9 and her CRP has risen from 4 to 38.[6][11]
Three questions decide this admission: is this lupus, the steroid, or an infection?, which mechanism branch owns the treatment?, and before you blast her with more immunosuppression, what have you excluded? Everything below is the consultant reasoning.[5][6][14]
References14ShowHide
- [1]ACR Ad Hoc Committee on Neuropsychiatric Lupus Nomenclature The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes Arthritis Rheum, 1999.PMID 10211873
- [2]Ainiala H, Loukkola J, Peltola J, et al. The prevalence of neuropsychiatric syndromes in systemic lupus erythematosus Neurology, 2001.PMID 11502919
- [3]Ainiala H, Hietaharju A, Loukkola J, et al. Validity of the new American College of Rheumatology criteria for neuropsychiatric lupus syndromes: a population-based evaluation Arthritis Rheum, 2001.PMID 11642640
- [4]Unterman A, Nolte JE, Boaz M, et al. Neuropsychiatric syndromes in systemic lupus erythematosus: a meta-analysis Semin Arthritis Rheum, 2011.PMID 20965549
- [5]Bertsias GK, Ioannidis JP, Aringer M, 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.PMID 20724309
- [6]Magro-Checa C, Zirkzee EJ, Huizinga TW, Steup-Beekman GM Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives Drugs, 2016.PMID 26809245
- [7]Isshi K, Hirohata S Association of anti-ribosomal P protein antibodies with neuropsychiatric systemic lupus erythematosus Arthritis Rheum, 1996.PMID 8814059
- [8]DeGiorgio LA, Konstantinov KN, Lee SC, et al. A subset of lupus anti-DNA antibodies cross-reacts with the NR2 glutamate receptor in systemic lupus erythematosus Nat Med, 2001.PMID 11689882
- [9]Hanly JG, Urowitz MB, Su L, et al. Prospective analysis of neuropsychiatric events in an international disease inception cohort of patients with systemic lupus erythematosus Ann Rheum Dis, 2010.PMID 19359262
- [10]Hanly JG, Su L, Urowitz MB, et al. Mood Disorders in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study Arthritis Rheumatol, 2015.PMID 25778456
- [11]Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus Ann Rheum Dis, 2019.PMID 30926722
- [12]Fanouriakis A, Kostopoulou M, Andersen J, et al. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update Ann Rheum Dis, 2024.PMID 37827694
- [13]Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus Arthritis Rheumatol, 2019.PMID 31385462
- [14]Govoni M, Hanly JG The management of neuropsychiatric lupus in the 21st century: still so many unmet needs? Rheumatology (Oxford), 2020.PMID 33280014