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
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 2013 2
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 2013 3
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 2013 4
Obs/Gynae history Para 2 + 1 (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 2013 5
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 2013 6
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 2013 7
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 2013 8
Initial investigations Imaging Special chemistry Biochemistry Microbiology Haematology 11-May-13 KAP Annual Scientific Conference 2013 9
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 2013 10
Results of lab investigations Special chemistry Anti - DS DNA +, 1101iu/ml (N = 0-200iu/ml) Anti SSA/Ro +, Anti RNP +, CA 125 + Anti APL negative Haematology anaemia (Hb 9g/dl) Biochemistry Urea 8.8 mmol (N = 2.5 6.4), Creatinine Normal LFTs Moderately enzymes 11-May-13 KAP Annual Scientific Conference 2013 11
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 2013 12
Management timeline Admitted ICU MTX/DM PMP Ward 17 19 21 23 25 27 29 31 Dates in August 2012 11-May-13 KAP Annual Scientific Conference 2013 13
Discussion Corticosteroids Antimalarial agents Cyclophosphamide Azathioprine Methotrexate Mycophenolate Mofetil Cyclosporin A 11-May-13 KAP Annual Scientific Conference 2013 14
RX of neuropsychiatric SLE I Inflammation Thrombosis 11-May-13 KAP Annual Scientific Conference 2013 15
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 2013 16
Rx of Neuropsychiatric SLE Thrombotic mechanisms (Positive APL) Induction oral anticoagulant, INR 2.5 3 Maintenance - oral anticoagulant, INR 2.5 3/antimalarials 11-May-13 KAP Annual Scientific Conference 2013 17
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 2013 18
Acknowledgments Dr Melvin D lima for editorial comments Nairobi Hospital management and nursing staff 11-May-13 KAP Annual Scientific Conference 2013 19