From the Guidelines
Transverse myelitis occurs in less than 1% of patients with Systemic Lupus Erythematosus (SLE). This rare but serious neurological complication typically presents as acute or subacute inflammation of the spinal cord, leading to motor, sensory, and autonomic dysfunction. The incidence may be higher in patients with active SLE, particularly those with other neuropsychiatric manifestations or antiphospholipid antibodies. According to the study by 1, the cumulative incidence of neuropsychiatric disorders in SLE patients categorizes them into common, relatively uncommon, and rare disorders, with transverse myelitis being considered a rare disorder.
Key Points
- The pathophysiology involves immune-mediated damage to the spinal cord, potentially through autoantibody production, immune complex deposition, or microvasculopathy.
- Early recognition is crucial as prompt treatment with high-dose corticosteroids, followed by cyclophosphamide or other immunosuppressants, may improve outcomes and prevent permanent neurological damage, as suggested by 1.
- Despite treatment, approximately one-third of SLE patients with transverse myelitis experience significant residual disability.
- The study by 1 emphasizes the need for further research to guide diagnostic, primary prevention, therapeutic, and monitoring decisions in NPSLE, highlighting the complexity of managing these patients.
Management and Treatment
- Prompt treatment with high-dose corticosteroids (typically methylprednisolone 1000mg IV daily for 3-5 days), followed by cyclophosphamide or other immunosuppressants, is recommended for transverse myelitis in SLE patients, as indicated by 1.
- Anticoagulation therapy may be considered in antiphospholipid-positive patients not responding to immunosuppressive therapy, as mentioned in 1.
- Maintenance immunosuppressive therapy is necessary to prevent relapses, which are common in SLE patients with transverse myelitis, as noted by 1.
From the Research
Incidence of Transverse Myelitis in SLE
- The incidence of transverse myelitis in Systemic Lupus Erythematosus (SLE) is estimated to be between 0.5% and 1% of all SLE patients 2.
- Transverse myelitis may be the presenting feature in 30%-60% of these patients 2.
Epidemiology and Prognosis
- A study found that the pooled 1-, 3- and 5-year relapse rates of transverse myelitis in SLE were 22%, 34%, and 36%, respectively 3.
- The presence of hypoglycorrhachia and a grade of A, B, or C on the American Spinal Injury Association Impairment Scale (AIS) at the onset of transverse myelitis were associated with a worse prognosis 3.
- Another study found that an initial severe neurological impairment and no cyclophosphamide use were associated with unfavorable neurological outcomes at six months and at the end of follow-up, respectively 4.
Treatment and Outcomes
- Treatment with intravenous methylprednisolone followed by cyclophosphamide seemed to be most effective in patients with transverse myelitis and SLE 5.
- A study found that 50% of patients with transverse myelitis and SLE showed complete recovery, 29% showed partial recovery, and 21% showed no improvement or deterioration 5.
- Early intensive therapy, including cyclophosphamide, may be initiated as soon as possible in patients with severe myelitis 3.