Background/Purpose: Transverse myelitis (TM) has an incidence of 1-2% in pediatric SLE and is often a feature of early disease. Most patients ( >60%) have fair neurological outcomes with some residual symptoms, however complete recovery only occurs in about 10-30% of patients. Most recovery occurs within the first three months, though a small fraction of patients will have no improvement resulting in significant disability. Previous studies suggest factors associated with poorer recovery include younger age, long spinal segment involvement, initial severe myelitis, presence of fever, hypocomplementemia, and higher CRP. We describe the presentation and outcome of all SLE-associated acute TM at a pediatric quaternary center.

Methods: We performed an IRB-approved query of our EMR (period of 3/30/12-6/1/24) and recorded clinical features, imaging, and laboratory data. We utilized SLEDAI score as a proxy measure of disease activity at the time of spinal cord inflammatory event. 

Results: During the defined time period, four out of 436 children with lupus (1%) developed TM. Patients’ median age at time of event was 14 years (range=10-17), and 75% presented within a year of SLE diagnosis (range=0-3 years). All patients were female, none were Hispanic, and 75% were Black. Median SLEDAI score at event was 12.5 (range=2-24). Two (50%) had concomitant renal involvement. All had long segment TM; median number of segments was 15.5 (range 3-22). One (25%) was febrile at the time of event. All presented with weakness, three (75%) with paresthesias, three (75%) with respiratory failure, two (50%) with urinary retention, and one (25%) with optic neuritis. MRI brain findings were notable for concern for demyelination, diffuse cortical damage, and abnormal T2 and flair hyperintensity. All had hypocomplementemia, two (50%) had elevated CRP and two (50%) had positive aPL. 

Treatment included IV methylprednisolone, PLEX, IVIG, rituximab, and CYC. Three (75%) received anticoagulation. A recent patient with a severe long-segment presentation and features of NMO also received IT dexamethasone and eculizumab. Two patients had cardiac arrest during their admission (presumed spinal shock or brainstem involvement); this was the cause of the only death. Other complications included quadriplegia (25%), and paraplegia (25%). One had close to full neurologic recovery with minor residual right-sided weakness and sensory symptoms. No patient had a flare of TM or any subsequent NPSLE feature after aggressive induction.

Conclusion: During a 12-year period, we reported an expected rate of spinal cord disease in a large pSLE cohort. Even with maximal therapy, neurologic outcomes were sub-optimal. Future directions may include better correlation of clinical phenotype with imaging findings (white vs. gray matter involvement) and enhanced identification of immune dysregulation targets for more directed treatments.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/acute-transverse-myelitis-in-pediatric-systemic-lupus-erythematosus/