(Kiosk 6) Functional Improvement in Pediatric Patients with Lupus Myelitis: A Case Series

Case Diagnosis: Lupus Myelitis

Case Description or Program Description: Myelitis is a rare complication of Systemic Lupus Erythematosus (SLE). Literature on treatment and functional outcomes are limited, especially in pediatrics. We describe the treatment and functional outcomes with the Functional Independence Measure for Children (WeeFIM) of two adolescents with lupus myelitis.

Setting: Quaternary Care Pediatric Hospital

Assessment/Results: Patient 1 is a 12-year-old female who presented with weakness in right arm and both legs, pain, fatigue, and urinary retention. Presentation consistent with incomplete C3 tetraplegia corroborated by longitudinally extensive T2 hyperintensity on MRI spine. Patient 2 is a 15-year-old female who presented with fever, headache, neck stiffness, and rash. Presentation consistent incomplete T10 paraplegia supported by MRI scattered T2 spinal cord hyperintensities from T1 to conus.

Both patients’ cerebrospinal fluid studies revealed pleocytosis. Rheumatologic work-up demonstrated: +ANA, dsDNA, anti-Smith, and RNP antibodies; elevated ESR; low C3/C4; and pancytopenia, meeting criteria for SLE with lupus myelitis. Treatment included pulse dose steroids, rituximab, cyclophosphamide, and plasmapheresis. Both patients were admitted to inpatient rehabilitation (IPR) at a non-ambulatory level requiring total assistance for bowel and bladder management. WeeFIM scores in mobility and self-care improved dramatically. By discharge, patient 1 was modified independent with wheeled walker and ankle-foot orthoses and patient 2 was ambulating independently. Both required intermittent catheterization and daily suppository for bladder and bowel management.

Discussion (relevance): Two previously healthy adolescent females developed incomplete non-traumatic spinal cord injuries with associated neurogenic bowel and bladder, longitudinally extensive MRI lesions, pleocytosis, and rheumatologic markers consistent with new-onset SLE with lupus myelitis. Aggressive immunomodulatory treatment and IPR resulted in functional improvements.

Conclusions: Though lupus myelitis has been described, pediatric treatment and outcomes are rarely reported. SLE with lupus myelitis should be considered in pediatric patients presenting with new-onset neurologic deficits particularly when associated with signs, symptoms, or laboratory findings consistent with autoimmune disease.