Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) Case presentations and topic discussion The Rheumatology Unit UMMC experience

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1 Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) Case presentations and topic discussion The Rheumatology Unit UMMC experience

2 References Sanna G, Bertolaccini ML. Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies. J Rheumatology 2003; 30: The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999;42: Bruyn GA. Controversies in lupus: Nervous system involvement. Rheum Dis 1995: 54:

3 CNS Lupus Current scenario in UMMC From the beginning of 2005 an average of 1 admission a month (new case). Varied presentations closely following the revised ACR criteria ( 19 NPSLE definitions) Problems with establishing a concrete diagnosis and we lack a clear treatment protocol.

4 CNS Lupus Nervous system manifestations are present in up to 70% of patients with SLE. There are 19 definitions which are components of NPSLE. The earlier classifications only recognized 2 clinical entities seizures and psychoses

5 NPSLE based on the revised ACR criteria Acute confusional state Anxiety disorders Cognitive function impairment Affective disorders psychoses Cranial nerve disorders Mononeuropathy Plexus neuropathy Vegetative neuropathy Myasthenia AIDP Polyneuropathy

6 NPSLE component entities Epileptic attacks Headaches and migraines Cereberovascular diseases Demyelinating syndromes Aseptic meningitis Chorea myelopathy

7 CNS Lupus problems in diagnosis Subtle presentations wouldn t I be depressed or anxious (or both) if I was diagnosed with SLE? Effect of corticosteroids Other differential diagnoses? Infections and metabolic diseases access to imaging facilities

8 NPSLE specific antibodies Anti ribosomal P antibody Anti neuronal antibodies

9 Imaging MRI by far the most superior SPECT scans have also been found to be useful In the UMMC experience the MRI has shown to be of great diagnostic value

10 MRI Images of 2 patients Case 1 14 years old Indian girl who presented to Kuantan with dense right sided hemiplegia who showed remarkable recovery with intravenous Methyl Prednisolone. Low complements, anti dsdna 276 iu/ml, Ig ACL 4 units, LA negative

11 MRI Images of 2 patients 12 years old Chinese girl presented with seizures while under treatment for class IV lupus nephritis. Low complements anti dsdna 167 iu/ml, IgG ACL 4 units, LA negative. Cases presented today had CT scans with contrast which were normal but with low complements and anti ds DNA in the thousands

12 NPSLE - CSF Varied findings among samples from our cases, an indicator which we use is the CSF proteins (tend to be higher then 0.45) More useful to rule out infections. Always send for TB culture.

13 Therapeutic approach in CNS lupus mild disease Symptomatic therapy Analgesics Anxiolytics Antidepressants Tricyclics Fluoxetine Anti convulsants Anti psychotics Low dose corticosteroids

14 Therapeutic approach in CNS Lupus severe CNS disease: diffuse/nonthrombotic disease Acute treatment High dose corticosteroids Iv pulse methylprednisolone Iv pulse cyclophosphamide Plasmapharesis Iv immunoglobulins Intrathecal methotrexate Azathioprine Mycophenolate mofetil

15 Therapeutic approach in CNS Lupus severe CNS disease: diffuse/nonthrombotic disease Chronic treatment Taper corticosteroids Iv pulse cyclophosphamide Methotrexate Azathioprine Mycophenolate mofetil

16 Current practice at UMMC Intravenous methylprednisolone 500mg daily for 3 days followed by one of 2 cyclophosphamide regimes 1) monthly courses of 1.0grams/BSA for 6 months followed by 3 monthly courses. 2) 2 weekly courses of 500mg total of 6 doses (3 grams in total)

17 Current practice at UMMC 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. Hydroxychloroquine Role for cyclophosphamide in such patients? At which doses 1 or 2?

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