E50 Central Nervous System manifestation of Systemic Lupus Erythematosus: A case presentation

Abstract

Abstract Background Systemic Lupus Erythematosus (SLE) is a complex, chronic, multisystem autoimmune disease with clinical manifestations in all organ systems. It is characterized by production of autoantibodies to components of the cell nucleus. Pediatric patients tend to have more severe disease and widespread organ involvement. If untreated, the 5-year mortality rate is almost 95%. Literature on SLE, more so pediatric SLE in Sub-Saharan Africa is scarce. Aim To describe the diagnostic journey of a child with CNS lupus in our setting. Method Retrospective chart review of a child with CNS Lupus at a tertiary referral teaching hospital in Nairobi, Kenya. Results We present a case of a 14-year-old boy who was diagnosed with CNS Lupus. He was known to have a convulsive disorder, with a recent history of mutism and was on Lamictal and rivotril for approximately one year. He presented to our facility in status epilepticus, having complained of a sudden onset of headache prior to the onset of the convulsions. He had had an uneventful birth and developmental history with no family history of chronic ailments. On examination, he was found to have bilateral ptosis with no other neurological deficits and otherwise normal systemic examination. Complete blood count initially showed a slight neutrophilia and thrombocytosis. Kidney function was normal and the CSF workup was negative for infection. Chest X ray showed features of aspiration pneumonia for which he received Augmentin, nebulization with hypertonic saline and chest physiotherapy. EEG showed generalized electrical seizures. CT/MRI brain showed acute infarcts in the superior frontal gyrus, post central gyrus and in the right insular cortex. He was managed in the Intensive Care Unit until cessation of the seizures. Post extubation, he was noted to have reduced lower limb power and bulbar dysfunction. A rheumatology consult was requested and revealed speech regression, a prior episode of conjunctivitis, a vasculitic palmar rash, palatial petechiae and suspected right knee arthritis. Urinalysis was suggestive of glomerulonephritis. ANA Antibodies were positive (titers of 1:160), positive anti-Ro antibodies. Anti-Ds DNA, P and C ANCA were negative. Renal histopathology was unremarkable. He was pulsed with IV methylprednisolone for 3 days with improvement and thereafter on follow up as an outpatient in the Pediatric Rheumatology clinic. Conclusion Pediatric SLE has significant morbidity and mortality therefore early intervention is crucial to retard disease progression. Given the vast array of clinical manifestations, it requires a high index of suspicion to enable early, accurate diagnosis and prompt treatment.