Myelitis in the course of systemic lupus erythematosus. Review

Abstract

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.