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Neuropsychiatric Systemic Lupus Erythematosus - Pathogenesis, Clinical Aspects and Treatment (Hardcover, 1st ed. 2018)
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Neuropsychiatric Systemic Lupus Erythematosus - Pathogenesis, Clinical Aspects and Treatment (Hardcover, 1st ed. 2018)
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Neuropsychiatric manifestation in systemic lupus erythematosus
(NPSLE) is one of the most recalcitrant complications of the
disease. According to the 1999 ACR nomenclature and case
definitions, diffuse psychiatric/neuropsychological syndromes in
NPSLE (anxiety disorder, acute confusional state, cognitive
dysfunction, mood disorder, psychosis) (diffuse NPSLE) present
psychiatric manifestations unlike neurologic syndromes (focal
NPSLE) originating from focal CNS lesions, such as cerebrovascular
disease, demyelinating syndrome, headache, aseptic meningitis,
chorea, seizures and myelopathy. A number of studies have reported
that diffuse NPSLE is usually associated with the presence of
autoantibodies against neuronal cells in serum as well as in
cerebrospinal fluid (CSF). Moreover, IL-6 has been shown to be
elevated in CSF of patients with diffuse NPSLE. Recently, it has
been demonstrated that the severity of blood-brain barrier damages
plays a crucial role in the development of acute confusional state,
the severest form of diffuse NPSLE through the accelerated entry of
larger amounts of autoantibodies to NMDA receptor subunit NR2 into
the CNS. Since the importance of autoantibodies in the NPSLE has
been now evident, such an aggressive treatment, especially B cell
depleting therapy, would make sense in that it would reduce the
levels of pathogenic autoantibodies, leading to a better prognosis
of NPSLE. As far as we know, no single book specifically dedicated
to NPSLE alone has been published as yet. As mentioned above, NPSLE
constitutes a vastly expanding field of research with increasing
numbers of papers published annually. Therefore, we believe that an
effort to collect and critically review these publications is
invaluable. Such an effort will provide an important contribution
to basic researchers as well as clinicians working in the field of
neurology, rheumatology, psychiatry and internal medicine fields.
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