Systemic lupus erythematosus (SLE) is an autoimmune disease with multisystemic involvement Pediatric systemic lupus erythematosus (pSLE) is a rare condition  representing approximately 10% of SLE cases. The nervous system involvement prevalence is estimated in 22–95% of all paediatric SLE cases  but is extremely rare as the initial clinical manifestation of the disease.  

A 11 year female child,diagonosed case of SLE with lupus nephritis outside and was on irregular treatment for last 1year presented to AMRI hospital  ED  with status epilepticus  . child had generalised edema, hypertensive, abnormal breathing pattern with low GCS. Child was intubated and shifted to PICU. Antibiotic, antiepileptic drug, antihypertensive with strict monitoring of input/output and  vitals. 2d ECHO  revealed global left ventricular hypokinesia with mild LV dysfunction with mild TR/PAH with LVH for which injection MILRINONE  and FUROSEMIDE started. In view of severe hypoalbunemia, albumin infusion was given. Inj methyl prednisolone pulse therapy started suspecting active flare up. Seizure controlled, GCS improved and maintained saturation with minimal settings in ventilator, so extubation tried but failed twice. Nephrology consultation was taken and plasmapheresis  5 cycles with IVIG at the end of each cycle was given. Inj Rituximab was started and continued monthly. Child was extubated and able to maintain saturation with oxygen support initially then room air.  MRI brain was done suggestive of  diffuse cerebral atrophy with multiple hemorrhages in cerebrum and cerebellum  with vasculitis possibly PRES. child was hemodynamically stable and oral feeds were started and discharged.