Hussein. S, Kioy. PG, Simani. P The Nairobi Hospital. KPA Annual Scientific Conference 2013 Safari Park Hotel, Nairobi
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1 Treatment of Neuropsychiatric Systemic Lupus Erythematosis with Intra-thecal Methotrexate - a case report Hussein. S, Kioy. PG, Simani. P The Nairobi Hospital KPA Annual Scientific Conference 2013 Safari Park Hotel, Nairobi
2 Introduction to SLE Definition: Multisystem autoimmune connective tissue disorder Epidemiology:20 70 cases per 100,000 population Presentation 4 commonest systems affected skin, Musculoskeletal, kidneys and CNS 11-May-13 KAP Annual Scientific Conference
3 Patient 35 yr old female of Asian origin History of SLE Evacuated by air from Arusha after acute flare up worsened 11-May-13 KAP Annual Scientific Conference
4 Married Family history Father suffered from diabetes and rheumatoid arthritis. Died at 65yrs from heart disease Mother, now 68yrs with hypertension and osteoporosis 3 siblings are alive and well. 11-May-13 KAP Annual Scientific Conference
5 Obs/Gynae history Para (spontaneous abortion in first trimester of 3 rd pregnancy) No mammogram done Last pap smear done 8 months ago 11-May-13 KAP Annual Scientific Conference
6 Systemic review Recent weight loss Fatigue and morning stiffness Nose bleeds Mouth sores with difficult swallowing Sun-sensitive skin Hair loss Color change in hands during cold Memory problems 11-May-13 KAP Annual Scientific Conference
7 General examination on admission Very sick looking young lady with fluctuating levels of consciousness Hypotensive - BP 90/60 Watery diarrhoea Moderate dehydration 11-May-13 KAP Annual Scientific Conference
8 Systemic examination CNS Drowsy, disoriented, confused soft neck Kernigs negative Paraparesis with exaggerated knee jerks, equivocal plantar reflex GIT Mouth ulcers Epigastric tenderness Hepatosplenomegaly with shifting dullness Skin Hair loss 11-May-13 KAP Annual Scientific Conference
9 Initial investigations Imaging Special chemistry Biochemistry Microbiology Haematology 11-May-13 KAP Annual Scientific Conference
10 Results of imaging investigations CT Head brain volume loss US abdomen HSM with ascites + no other masses noted CXR,MRI (Thorax/lumbar) - Normal Doppler US of lower limbs - Normal 11-May-13 KAP Annual Scientific Conference
11 Results of lab investigations Special chemistry Anti - DS DNA +, 1101iu/ml (N = 0-200iu/ml) Anti SSA/Ro +, Anti RNP +, CA Anti APL negative Haematology anaemia (Hb 9g/dl) Biochemistry Urea 8.8 mmol (N = ), Creatinine Normal LFTs Moderately enzymes 11-May-13 KAP Annual Scientific Conference
12 Diagnosis and co-morbidities 1. Neuropsychiatric SLE presenting as an acute confusional state with paraparesis 2. Co-Morbidities? Infection 11-May-13 KAP Annual Scientific Conference
13 Management timeline Admitted ICU MTX/DM PMP Ward Dates in August May-13 KAP Annual Scientific Conference
14 Discussion Corticosteroids Antimalarial agents Cyclophosphamide Azathioprine Methotrexate Mycophenolate Mofetil Cyclosporin A 11-May-13 KAP Annual Scientific Conference
15 RX of neuropsychiatric SLE I Inflammation Thrombosis 11-May-13 KAP Annual Scientific Conference
16 Rx of neuropsychiatric SLE II Inflammatory mechanisms (negative APL) Induction High dose Pulse CS and Cycl MTX intra-thecal Plasma exchange Ritux in refractory cases Maintenance AZA,MTX,MMF 11-May-13 KAP Annual Scientific Conference
17 Rx of Neuropsychiatric SLE Thrombotic mechanisms (Positive APL) Induction oral anticoagulant, INR Maintenance - oral anticoagulant, INR 2.5 3/antimalarials 11-May-13 KAP Annual Scientific Conference
18 New therapies B cell targeted therapies Rituximab LJP 394 Anti Blys (Belimumab) TNF blocking agents (infliximab) Haemopoietic stem cell transplantation 11-May-13 KAP Annual Scientific Conference
19 Acknowledgments Dr Melvin D lima for editorial comments Nairobi Hospital management and nursing staff 11-May-13 KAP Annual Scientific Conference
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