Myelopathy manifested clinically as acute longitudinal or
transverse myelitis constitutes one of the most severe and rare neuropsychiatric manifestations of
systemic lupus erythematosus (SLE) (1-3% of patients).
Myelitis has been observed less commonly in other
connective tissue diseases, mostly in
antiphospholipid syndrome, and rarely in Sjögren's syndrome, Behçet's disease and
mixed connective tissue disease.
Acute transverse myelitis (ATM) may also be present in diseases of various etiology, including
multiple sclerosis,
sarcoidosis,
infectious diseases and
malignancies.
Myelitis in SLE is manifested as a dramatic
spinal cord injury leading to
paralysis or
muscular paresis, sensory deficits, and smooth muscle dysfunction usually in the form of sphincter dysfunction. The imaging technique of choice in case of suspected ATM is magnetic resonance imaging with intravenous
contrast agent (
gadolinium diethylenetriamine-pentaacid). Cerebrospinal fluid (CSF) examination in patients with ATM in the course of SLE indicate usually
pleocytosis with prevalence of granulocytes, increased
protein levels, low
glucose levels, significantly hindering differential diagnosis in the early stage of the disease. Observations made by the authors (2 female patients with SLE) show that
antibodies specific to SLE can be found in the CSF collected in the acute phase of
myelitis. These observations have not yet been confirmed by other researchers. Early introduction of intravenous immunosuppression with large doses of
cyclophosphamide and glucocorticosteroids improves the long-term prognosis. Other therapeutic approaches have been also used in more severe cases. Even with appropriate
therapy, prognosis in this disease is uncertain.