(746) Neuropsychiatric SLE in a Filipino Child Presenting as Fulminant Guillain-Barré Syndrome

Introduction: Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) and Guillain-Barré Syndrome (GBS) are common diagnoses in children but their association and simultaneous occurrence is rare, as peripheral nervous system involvement only accounts for 3-5% of NPSLE. Whether GBS unmasks SLE, or lupus flare triggers GBS is currently unknown, and the best treatment approach has not yet been established.

Description: An 8-year old female was admitted in the PICU of University of Santo Tomas Hospital, Manila in 2023 due to symmetrical ascending weakness. She had bulbar involvement, hypotonia, and areflexia, and CSF analysis showed cytoalbuminologic dissociation hence diagnosed with GBS. She was given IVIG 0.4g/kg for 5 days. During treatment, she was intubated due to respiratory failure and paralysis further progressed with absence of spontaneous breathing. Two days after, she went into a brain death-like state with no spontaneous eye opening, anisocoria, and no brainstem reflexes. Plain Cranial CT scan was normal. An assessment of Fulminant GBS was made. Moreover, she also had hypertension, edema, oliguria, low C3, and elevated UPCR. Further workups showed (+) ANA and anti-dsDNA, hence diagnosed with Neuropsychiatric Systemic Lupus Erythematosus. A renal biopsy showed Lupus Nephritis Class IV. She was given Pulse Methylprednisolone, Cyclophosphamide, and immunosuppressants but had no improvement hence underwent 5 cycles of Therapeutic Plasma Exchange (TPE). Tracheostomy tube was also inserted. Other diagnostic investigations revealed myocarditis, Grade I right ventricular diastolic dysfunction, and subacute infarcts, white matter hyperintensities and mild hydrocephalus. She regained consciousness and spontaneous breathing immediately after TPE, with gradual improvement of bulbar muscles and motor power. She achieved remission 2 months after discharge and with continued physical therapy, she is now able to walk with assistance and no other deficits.

Discussion: To our knowledge, this is the first case of a pediatric patient with NPSLE-GBS who progressed into a brain death-like state and was successfully treated with TPE, IVIG, and immunosuppressants. A high index of suspicion with prompt recognition is of utmost importance since management is complicated and treatment guidelines are still lacking.