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Neuropsychiatric Lupus

Neuropsychiatric Lupus. Seuli Bose Brill, MD Medicine AM Report 2/9/10. Historical perspective. Initially described by Mortiz Kaposi in 1870s (delirium) Further description by Osler in early 1903 Prior to this, lupus thought to be primarily cutaneous disease

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Neuropsychiatric Lupus

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  1. Neuropsychiatric Lupus Seuli Bose Brill, MD Medicine AM Report 2/9/10

  2. Historical perspective • Initially described by Mortiz Kaposi in 1870s (delirium) • Further description by Osler in early 1903 • Prior to this, lupus thought to be primarily cutaneous disease • The term “lupus” used as early as the 13th century to describe a wolf-like rash

  3. 25-50% of all patients with SLE have some neuropsychiatric involvement.

  4. Common Clinical Manifestations • Cognitive dysfunction (55-80%) • Headache (24-72%) • Mood disorders and psychosis (14-57%) • Cerebrovascular disease • Acute confusional state • Peripheral nervous system involvement

  5. SLE Related Cognitive Dysfunction • Mild cognitive impairment (detected through neuropsychiatric testing) estimated to be about 80% • Variable presentation • Overall cognitive slowing • Decreased attention • Impaired working memory • Executive dysfunction (e.g. difficulty multitasking)

  6. SLE Related Cognitive Dysfunction • More prevalent in those with active compared to inactive SLE • Decline is not inevitable • Waxing and waning course • Difficult to distinguish from other causes of cognitive dysfunction • Often diagnosis of exclusion due to lack of definitive diagnostic testing

  7. Pathogenesis • Increased permeability of blood brain barrier • Pro-inflammatory cytokine mediated disruption of global function • Vascular injury of small and large caliber vessels • Microangiopathic • Anti-phospholipid antibodies, immune complexes, and leukoagglutination • May cause focal or global events

  8. Disease Mechanism

  9. Diagnostic Evaluation

  10. Biomarkers • Area of aggressive investigation • Many with low specificity • Many are experimental • Currently with limited clinical application

  11. Neuroimaging • Several possible modalities • Computerized tomography (CT) • Magnetic resonance imaging/angiography (MRI/MRA) • Positron electron tomography (PET) • Single photon emission computed tomography (SPECT) • Choice depends on focal versus global dysfunction • Supplementation with EEG • Normal study does not rule out disease • Cerebral vasculitis generally not detected on MRI/MRA or even autopsy

  12. Treatment • Symptomatic Therapy • Anti-epileptics • Anti-psychotics • Anti-coagulation when anti-phospholipid antibodies implicated • Immunosuppression (prolonged course) • High dose oral corticosteroids • May be coupled with cyclophosphamide or rituximab • Regimens derived from uncontrolled clinical studies with small numbers • Cognitive Rehabilitation • In developmental stages

  13. Ongoing NP-SLE Research • Role of auto-antibodies and inflammatory mediators • Long term patient outcomes • Clinical significance in context of overall disease activity • Correlation of neuroimaging in patients who meet diagnostic criteria • Controlled trials of treatment modalities

  14. Take Home Points • Neuropsychiatric manifestations of SLE are very common. • Clinical diagnosis can be elusive. • Presentations are varied. • Diagnostic testing is often unreliable. • Prolonged immune suppression is the mainstay of therapy.

  15. Bibliography • History of Lupus; http://www.lupus.org/ • Colasanti T, Delunardo F, Margutti P, Vacirca D, Piro E, Siracusano A, Ortona E. Autoantibodies involved in neuropsychiatric manifestations associated with systemic lupus erythematosus. J Neuroimmunol. 2009 Jul 25;212(1-2):3-9. • Efthimiou P, Blanco M. Pathogenesis of neuropsychiatric systemic lupus erythematosus and potential biomarkers. Mod Rheumatol. 2009;19(5):457-68. • Hanly JG. Demystifying neuropsychiatric lupus--is it possible? Bull NYU Hosp Jt Dis. 2009;67(3):276-80. • Hanly JG, Harrison MJ. Management of neuropsychiatric lupus. Best Pract Res Clin Rheumatol. 2005 Oct;19(5):799-821. • Hirohata S, Kanai Y, Mitsuo A, Tokano Y, Hashimoto H; Accuracy of cerebrospinal fluid IL-6 testing for diagnosis of lupus psychosis. A multicenter retrospective study. Clin Rheumatol. 2009 Nov;28(11):1319-23. • Holubar K, Fatović-Ferencić S. Cazenave, Kaposi and lupus erythematosus. A centennial and a sesquicentennial. Dermatology. 2001;203(2). • Kajs-Wyllie M. Lupus cerebritis: a case study. J Neurosci Nurs. 2002 Aug;34(4):176-83. • Muscal E, Brey RL. Neurologic manifestations of systemic lupus erythematosus in children and adults.; Neurol Clin. 2010 Feb;28(1):61-73. • Mallavarapu RK, Grimsley EW. The history of lupus erythematosus. South Med J. 2007 Sep;100(9):896-8.

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