Demyelinating disease in SLE: is it multiple sclerosis or lupus?
Among the 12 systemic lupus erythematosus (SLE)-related central nervous system (CNS) syndromes defined by the American College of Rheumatology (ACR), demyelinating syndrome and myelopathy are two of the less prevalent and more poorly understood ones. One important issue concerning demyelinating disease in SLE is that it can be easily misdiagnosed with other central nervous system demyelinating disorders such as multiple sclerosis (MS). A clinically isolated neurological syndrome can be the presenting feature before other concomitant symptoms of SLE appear or definite MS is diagnosed. Although challenging, some diagnostic tests used in clinical practice and research may help to differentiate between these entities. These tests have improved the understanding of the pathogenesis in these diseases, but some points, such as the role of antiphospholipid antibodies in SLE-associated transverse myelitis, remain unclear and are a matter of ongoing debate. This review discusses clinical, pathophysiological, radiological and therapeutic concepts of demyelinating disease of the CNS in SLE, focussing on its differentiation from MS and its relation with other CNS demyelinating processes, such as transverse myelitis, optic neuritis and neuromyelitis optica.
Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. Electronic address: C.Magro_Checa@lumc.nl.
César
C
Magro Checa
Danielle
D
Cohen
Eduard L E M
EL
Bollen
Mark A
MA
van Buchem
Tom W J
TW
Huizinga
Gerda M
GM
Steup-Beekman
D017152:Antibodies, Antiphospholipid / Q000097:blood; D020278:Demyelinating Autoimmune Diseases, CNS / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D009103:Multiple Sclerosis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy
D017152:Antibodies, Antiphospholipid
Demyelination; Multiple sclerosis; Neuromyelitis optica; Neuropsychiatric lupus; Optic neuritis; Transverse myelitis