Background/Purpose: Systemic Lupus Erythematosus (SLE) with acute myelitis or optic neuritis is rare and causes significant morbidity. Data on these conditions remain limited. This study aimed to evaluate the clinical profile and outcomes of patients presenting with demyelinating syndromes (transverse myelitis or optic neuritis) in SLE.

Methods: A retrospective chart review was conducted, identifying patients diagnosed with SLE and myelitis or optic neuritis at our center between 2000 and 2023. SLE was diagnosed using ACR-EULAR 2019 criteria. The Expanded Disability Status Scale (EDSS) was used to assess disability levels. Data were analyzed using descriptive statistics.

Results: A total of 25 female patients were identified with a mean age of 27.04 ± 9.69 years. The interval between SLE diagnosis and onset of demyelination varied widely, with 24% of patients presenting with demyelination as an initial manifestation. The mean SLEDAI was 17.72± 6.88. Sixteen patients (64%) reached the nadir of clinical presentation within 24 hours. Severe impairments included motor deficit (88%), sensory level involvement (88%), urinary retention (72%), and optic neuritis (16%). MRI was abnormal in all cases, with thoracic cord involvement in 88%, longitudinally extensive myelitis in 53%, and area postrema involvement in 8.3%. ANA was positive in all patients, 76% had low complements, and 12% tested positive for aquaporin-4 antibodies. Pulse methylprednisolone was administered to 92%; cyclophosphamide (68%) was the most used second-line therapy. At baseline, 72% had severe disability (EDSS > 5), which improved after six months, with only 12% retaining severe disability. Three patients relapsed after treatment and one patient succumbed to sepsis. No patients with optic neuritis developed optic atrophy within one year.

Conclusion: Demyelinating syndromes in SLE primarily affect young females and correlate with high disease activity. Early immunosuppressive therapy leads to improved outcomes , though some patients retain residual disability, underscoring the need for prompt intervention.

Table 1: Patient Demographics and Clinical Characteristics

Table 2: Treatment and Outcomes

Figure 1: EDSS (Expanded Disability Status Scale) Score at baseline and 6 month and EDSS Criteria

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/demyelinating-syndromes-in-systemic-lupus-erythematosus-a-23-year-retrospective-analysis-of-clinical-features-and-treatment-outcomes-from-a-tertiary-care-center/