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  1. 1. Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) Case presentations and topic discussion The Rheumatology Unit UMMC experience
  2. 2. References <ul><li>Sanna G, Bertolaccini ML. Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies. J Rheumatology 2003; 30: 985-992. </li></ul><ul><li>The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999;42:599-608 </li></ul><ul><li>Bruyn GA. Controversies in lupus: Nervous system involvement. Rheum Dis 1995: 54: 159-167 </li></ul>
  3. 3. CNS Lupus – Current scenario in UMMC <ul><li>From the beginning of 2005 an average of 1 admission a month (new case). </li></ul><ul><li>Varied presentations closely following the revised ACR criteria ( 19 NPSLE definitions) </li></ul><ul><li>Problems with establishing a concrete diagnosis and we lack a clear treatment protocol. </li></ul>
  4. 4. CNS Lupus <ul><li>Nervous system manifestations are present in up to 70% of patients with SLE. </li></ul><ul><li>There are 19 definitions which are components of NPSLE. The earlier classifications only recognized 2 clinical entities – seizures and psychoses </li></ul>
  5. 5. NPSLE based on the revised ACR criteria <ul><li>Acute confusional state </li></ul><ul><li>Anxiety disorders </li></ul><ul><li>Cognitive function impairment </li></ul><ul><li>Affective disorders </li></ul><ul><li>psychoses </li></ul><ul><li>Cranial nerve disorders </li></ul><ul><li>Mononeuropathy </li></ul><ul><li>Plexus neuropathy </li></ul><ul><li>Vegetative neuropathy </li></ul><ul><li>Myasthenia </li></ul><ul><li>AIDP </li></ul><ul><li>Polyneuropathy </li></ul>
  6. 6. NPSLE component entities <ul><li>Epileptic attacks </li></ul><ul><li>Headaches and migraines </li></ul><ul><li>Cereberovascular diseases </li></ul><ul><li>Demyelinating syndromes </li></ul><ul><li>Aseptic meningitis </li></ul><ul><li>Chorea </li></ul><ul><li>myelopathy </li></ul>
  7. 7. CNS Lupus – problems in diagnosis <ul><li>Subtle presentations – wouldn’t I be depressed or anxious (or both) if I was diagnosed with SLE ? </li></ul><ul><li>Effect of corticosteroids </li></ul><ul><li>Other differential diagnoses? Infections and metabolic diseases </li></ul><ul><li>access to imaging facilities </li></ul>
  8. 8. NPSLE specific antibodies <ul><li>Anti ribosomal P antibody </li></ul><ul><li>Anti neuronal antibodies </li></ul>
  9. 9. Imaging <ul><li>MRI by far the most superior </li></ul><ul><li>SPECT scans have also been found to be useful </li></ul><ul><li>In the UMMC experience the MRI has shown to be of great diagnostic value </li></ul>
  10. 10. MRI Images of 2 patients <ul><li>Case 1 – 14 years old Indian girl who presented to Kuantan with dense right sided hemiplegia who showed remarkable recovery with intravenous Methyl Prednisolone. </li></ul><ul><li>Low complements, anti dsDNA 276 iu/mL, Ig ACL 4 units, LA negative </li></ul>
  11. 11. MRI Images of 2 patients <ul><li>12 years old Chinese girl presented with seizures while under treatment for class IV lupus nephritis. </li></ul><ul><li>Low complements anti dsDNA 167 iu/mL, IgG ACL 4 units, LA negative. </li></ul><ul><li>Cases presented today had CT scans with contrast which were normal but with low complements and anti ds DNA in the thousands </li></ul>
  12. 12. NPSLE - CSF <ul><li>Varied findings among samples from our cases, an indicator which we use is the CSF proteins (tend to be higher then 0.45) </li></ul><ul><li>More useful to rule out infections. Always send for TB culture. </li></ul>
  13. 13. Therapeutic approach in CNS lupus – mild disease <ul><li>Symptomatic therapy </li></ul><ul><li>Analgesics </li></ul><ul><li>Anxiolytics </li></ul><ul><li>Antidepressants </li></ul><ul><li>Tricyclics </li></ul><ul><li>Fluoxetine </li></ul><ul><li>Anti convulsants </li></ul><ul><li>Anti psychotics </li></ul><ul><li>Low dose corticosteroids </li></ul>
  14. 14. Therapeutic approach in CNS Lupus – severe CNS disease: diffuse/nonthrombotic disease <ul><li>Acute treatment </li></ul><ul><li>High dose corticosteroids </li></ul><ul><li>Iv pulse methylprednisolone </li></ul><ul><li>Iv pulse cyclophosphamide </li></ul><ul><li>Plasmapharesis </li></ul><ul><li>Iv immunoglobulins </li></ul><ul><li>Intrathecal methotrexate </li></ul><ul><li>Azathioprine </li></ul><ul><li>Mycophenolate mofetil </li></ul>
  15. 15. Therapeutic approach in CNS Lupus – severe CNS disease: diffuse/nonthrombotic disease <ul><li>Chronic treatment </li></ul><ul><li>Taper corticosteroids </li></ul><ul><li>Iv pulse cyclophosphamide </li></ul><ul><li>Methotrexate </li></ul><ul><li>Azathioprine </li></ul><ul><li>Mycophenolate mofetil </li></ul>
  16. 16. Current practice at UMMC <ul><li>Intravenous methylprednisolone 500mg daily for 3 days followed by one of 2 cyclophosphamide regimes </li></ul><ul><li>1) monthly courses of 1.0grams/BSA for 6 months followed by 3 monthly courses. </li></ul><ul><li>2) 2 weekly courses of 500mg total of 6 doses (3 grams in total) </li></ul>
  17. 17. Current practice at UMMC <ul><li>Symptomatic treatment is accompanied by intravenous methylprednisolone and high dose 1mg/kg of oral prednisolone daily which is tapered. Usually a steroid sparing agent such as azathioprine is added. </li></ul><ul><li>Hydroxychloroquine </li></ul><ul><li>Role for cyclophosphamide in such patients? At which doses – 1 or 2? </li></ul>