Neuropsychiatric Systemic Lupus Erythematosus

Popescu, Alexandra; Kao, Amy H.

doi:10.2174/157015911796557984

Cited by 164 publications

(166 citation statements)

References 110 publications

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“…The observed lesions were sparing peripherally, and T1-weighted images revealed typically multifocal small hypointense foci in periventricular areas, the pons of the cerebellum and the middle cerebellar peduncles. Differential diagnoses based on MR findings should include thromboembolic stroke, multiple sclerosis, encephalitis, lupus erythematosus, Ménière's disease, migraine, and schizophrenia [6,8,9].…”

Section: Discussionmentioning

confidence: 99%

Diagnostic implications and effective treatment of Susac syndrome with thrombophilia

Grygiel-Górniak¹,

Puszczewicz²,

Czaplicka³

2016

Reumatologia

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Susac syndrome is an uncommon autoimmune microangiopathy characterized by a combination of encephalopathy, branch retinal artery occlusions (BRAO), and hearing loss. In this report, we present a detailed history of a 31-year-old man with Susac syndrome with thrombophilia. The diagnosis was based on clinical signs and neurological, otolaryngological and ocular examination. Fluorescence retinal angiography showed right BRAO. A slight (30 dB) sensorineural lowering of the hearing level was observed in the audiogram. Numerous ovoid lesions in the corpus callosum were detected in magnetic resonance (MR). The applied treatment included glucocorticosteroids, disease-modifying antirheumatic drugs, anticoagulant treatment and immunoglobulin infusions. After treatment, ophthalmologic symptoms receded, the recanalization of BRAO was detected in angiography, and the lesions in the corpus callosum were reduced in control MR. To date, this is the first report showing the effective treatment of Susac syndrome, which was confirmed simultaneously in MR and fluorescein angiography in a case of Susac syndrome with thrombophilia.

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Section: Discussionmentioning

confidence: 99%

Diagnostic implications and effective treatment of Susac syndrome with thrombophilia

Grygiel-Górniak¹,

Puszczewicz²,

Czaplicka³

2016

Reumatologia

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“…Of note, immunomodulating agents that have been established as effective treatments for MS may severely exacerbate both NMO [46,47] and SLE [48]. Therefore, corticosteroids alone or in combination with immunosuppressants (azathioprine, mycophenolate mofetil, cyclophosphamide) should prove effective in reducing incidence of relapse in both NMO [49][50][51] and NPSLE [4,52]. As described above, treatment of NMO is very similar to that of NPSLE, and this has been demonstrated in two patients with NMO and NPSLE who were successfully treated with high-dose corticosteroid therapy following plasmapheresis with azathioprine or cyclophosphamide [53].…”

Section: Treatmentmentioning

confidence: 99%

Systemic Lupus Erythematosus with Neuromyelitis Optica

Takahashi¹

2014

J Clin Cell Immunol

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Systemic lupus erythematosus (SLE) is an autoimmune disease that can have detrimental effects on many different systems in the body, including the central nervous system. Neuropsychiatric SLE (NPSLE) refers to several different neurological and/or behavioral clinical syndromes, and has been reported as having a prevalence rate of approximately 30−40%, while manifestation of myelitis or optic neuritis of NPSLE is rare (~1%). Myelitis and optic neuritis are easily identifiable since myelitis is frequently transverse, and manifests as severe disturbances in both motor and sensory pathways, while optic neuritis is often both bilateral and severe. At least 85% of patients experience relapses in the form of optic neuritis, transverse myelitis, or both. Furthermore, some cases of NPSLE with optic neuritis are often complicated by myelitis. Interestingly, the characteristics of myelitis or optic neuritis in NPSLE are quite similar to neuromyelitis optica (NMO), a disease characterized by bilateral optic neuropathy and transverse myelopathy. In fact, magnetic resonance imaging (MRI) of patients with NPSLE has demonstrated longitudinal spinal involvement showing cord swelling and hyperintense lesions in central regions. These findings are also typically observed in MRIs of patients with NMO. Additionally, anti-aquaporin 4 (AQP4) antibodies have been discovered in patients with NMO and with NPSLE with myelitis and/or optic neuritis. Therefore, complications that are often encountered with NMO should be considered when treating cases of NPSLE with myelitis and/or optic neuritis. Moreover, since the treatment of NMO closely resembles the therapeutic approaches taken for NPSLE, corticosteroids alone or in combination with immunosuppressants could prove effective in reducing incidents of relapse. Some patients, however, may be refractory to steroid therapy; in such cases, plasma exchange may have priority over other second-line therapeutic strategies, such as intravenous immunoglobulin and rituximab, because of treatment approaches typically employed in NMO. In this review, I will discuss pathological similarities between NPSLE with myelitis and/or optic neuritis and NMO with the aim of demonstrating that our knowledge of NMO should be considered when treating NPSLE with myelitis and/or optic neuritis.

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“…Systemic lupus erythematosus (SLE) is an autoimmune disease prevalently affecting any part of the peripheral nervous system or CNS, defined as neuropsychiatric SLE (NPSLE) [110]. The pathogenesis of NPSLE is multifactorial and involves various inflammatory cytokines, autoantibodies, and immune complexes resulting in vasculopathic, cytotoxic and autoantibody-mediated neuronal injury [111].…”

Section: Roles Of Immune and Inflammation In Drementioning

confidence: 99%

Modulation of Immunity and the Inflammatory Response: A New Target for Treating Drug-resistant Epilepsy

Líu²,

Qin³

2013

Current Neuropharmacology

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Until recently, epilepsy medical therapy is usually limited to anti-epileptic drugs (AEDs). However, approximately 1/3 of epilepsy patients, described as drug-resistant epilepsy (DRE) patients, still suffer from continuous frequent seizures despite receiving adequate AEDs treatment of sufficient duration. More recently, with the remarkable progress of immunology, immunity and inflammation are considered to be key elements of the pathobiology of epilepsy. Activation of inflammatory processes in brain tissue has been observed in both experimental seizure animal models and epilepsy patients. Anti-inflammatory and immunotherapies also showed significant anticonvulsant properties both in clinical and in experimental settings. The above emerging evidence indicates that modulation of immunity and inflammatory processes could serve as novel specific targets to achieve potential anticonvulsant effects for the patients with epilepsy, especially DRE. Herein we review the recent evidence supporting the role of inflammation in the development and perpetuation of seizures, and also discuss the recent achievements in modulation of inflammation and immunotherapy applied to the treatment of epilepsy. Apart from medical therapy, we also discuss the influences of surgery, ketogenic diet, and electroconvulsive therapy on immunity and inflammation in DRE patients. Taken together, a promising perspective is suggested for future immunomodulatory therapies in the treatment of patients with DRE.

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Neuropsychiatric Systemic Lupus Erythematosus

Cited by 164 publications

References 110 publications

Diagnostic implications and effective treatment of Susac syndrome with thrombophilia

Diagnostic implications and effective treatment of Susac syndrome with thrombophilia

Systemic Lupus Erythematosus with Neuromyelitis Optica

Modulation of Immunity and the Inflammatory Response: A New Target for Treating Drug-resistant Epilepsy

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