Central Nervous System Lupus Vasculitis

Central nervous system vasculitis that is associated with SYSTEMIC LUPUS ERYTHEMATOSUS. Clinical manifestations may include DEMENTIA; SEIZURES; CRANIAL NERVE DISEASES; HEMIPARESIS; BLINDNESS; DYSPHASIA; and other neurological disorders.

Also Known As:

Lupus Vasculitis, Central Nervous System; Central Nervous System Lupus; Central Nervous System Systemic Lupus Erythematosis; Lupus Meningoencephalitis; Systemic Lupus Erythematosis, Central Nervous System; Lupus Meningoencephalitides; Meningoencephalitides, Lupus; Meningoencephalitis, Lupus; Neuropsychiatric Systemic Lupus Erythematosus

Networked: 234 relevant articles (6 outcomes, 21 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Psychotic Disorders (Schizoaffective Disorder)
2.	Systemic Lupus Erythematosus (Libman-Sacks Disease)
3.	Mania (Manias)
4.	Lupus Nephritis
5.	Cognitive Dysfunction

Experts

1.	Hirohata, Shunsei: 9 articles (01/2021 - 01/2006)
2.	Arinuma, Yoshiyuki: 7 articles (01/2020 - 04/2008)
3.	Steup-Beekman, Gerda M: 5 articles (01/2018 - 01/2006)
4.	Ichinose, Kunihiro: 4 articles (01/2022 - 04/2015)
5.	Kawakami, Atsushi: 4 articles (01/2022 - 04/2015)
6.	van Buchem, Mark A: 4 articles (01/2018 - 01/2006)
7.	Yoshio, Taku: 4 articles (11/2011 - 01/2005)
8.	Li, Xue: 3 articles (11/2022 - 01/2019)
9.	Sun, Xiaolin: 3 articles (11/2022 - 01/2019)
10.	Wang, Qingwen: 3 articles (11/2022 - 01/2019)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Central Nervous System Lupus Vasculitis:

1.	AntibodiesIBA 03/01/2009 - "Antineuronal antibodies and electroneurophysiological studies in pediatric patients with neuropsychiatric systemic lupus erythematosus." 01/01/2023 - "Mouse models, antibodies, and neuroimaging: Current knowledge and future perspectives in neuropsychiatric systemic lupus erythematosus (NPSLE)." 11/01/2020 - "Association of anti-NR2 and U1RNP antibodies with neurotoxic inflammatory mediators in cerebrospinal fluid from patients with neuropsychiatric systemic lupus erythematosus." 01/01/2018 - "Neuropsychiatric systemic lupus erythematosus (NPSLE) is often difficult to diagnose and distinguish from other diseases, because no NPSLE-specific antibodies have been identified. " 01/01/2018 - "Novel anti-suprabasin antibodies may contribute to the pathogenesis of neuropsychiatric systemic lupus erythematosus."
2.	CytokinesIBA 04/01/2015 - "Distinguishing the cerebrospinal fluid cytokine profile in neuropsychiatric systemic lupus erythematosus from other autoimmune neurological diseases." 01/01/2003 - "The heterogeneity of neuropsychiatric systemic lupus erythematosus is reflected in lack of association with cerebrospinal fluid cytokine profiles." 01/01/1998 - "Cerebrospinal fluid cytokine concentrations in a patient with lupus meningoencephalitis: differences from cytokine profiles in central nervous system infections." 08/03/2018 - "Based on all the articles reviewed, the antibodies and cytokines showed the most effective evidence in causing the different manifestations of neuropsychiatric systemic lupus erythematosus (NPSLE), but studies regarding the drugs being effective against all the symptoms are inconclusive as there are very few studies. " 01/01/2003 - "The objective was to study the occurrence of autoantibodies and cytokines in serum and cerebrospinal fluid (CSF) in neuropsychiatric systemic lupus erythematosus (NPSLE). "
3.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 05/01/2016 - "Cyclophosphamide (CYC) is used in severe neuropsychiatric systemic lupus erythematosus (NPSLE), but long-term data regarding its efficacy and safety are lacking. " 05/01/1996 - "Pulse cyclophosphamide in the treatment of neuropsychiatric systemic lupus erythematosus." 01/01/1995 - "Role of intravenous cyclophosphamide in the treatment of severe neuropsychiatric systemic lupus erythematosus." 09/01/2022 - "We describe a rare case of PML, which was misdiagnosed as neuropsychiatric systemic lupus erythematosus (SLE) with a catastrophic outcome due to delay in diagnosis with superadded cyclophosphamide therapy. " 01/01/2004 - "Cyclophosphamide remains the 'gold standard' treatment for severe organ threatening systemic lupus erythematosus (SLE), especially renal and central nervous system lupus. "
4.	SteroidsIBA 08/01/2005 - "High-dose steroid with or without anticoagulation is the mainstay of treatment in neuropsychiatric systemic lupus erythematosus (NPSLE). " 01/01/2012 - "It can be due to drugs (steroids) used for SLE or secondary to SLE because of its brain involvement, which is termed as neuropsychiatric systemic lupus erythematosus (NPSLE). " 09/01/2004 - "A case of central nervous system lupus in succession to lupus peritonitis: a difficulty in the differential diagnosis between lupus psychosis and steroid-induced psychosis." 04/01/1988 - "A patient with systemic lupus erythematosus presenting both central nervous system lupus and steroid induced psychosis." 12/01/2021 - "Malignant Catatonia After Antipsychotic and High-Dose Steroid Use in a Teenager With Neuropsychiatric Systemic Lupus Erythematosus."
5.	Adrenal Cortex Hormones (Corticosteroids)IBA 06/01/1976 - "This suggests that massive doses of corticosteroids may be needed for effective treatment of central nervous system lupus." 02/01/2016 - "Corticosteroids in Lupus Nephritis and Central Nervous System Lupus." 02/01/2006 - "[Appearance of central nervous system lupus during corticosteroid therapy and warfarinization in a patient with pure red cell aplasia and antiphospholipid syndrome]." 04/01/2000 - "We report three female patients with systemic lupus erythematosus complicated by massive intestinal hemorrhage during the recovery from lupus nephritis (case 1, 2) or central nervous system lupus (case 3) on high dose corticosteroid therapy. " 03/01/1999 - "No significant association with calcification was noted for the following variables: age at disease onset, duration of the disease, sex, the maximum value of the serum calcium, organic phosphate, and uric acid, Raynaud's phenomenon, lupus nephritis, femoral avascular necrosis, central nervous system lupus, proteinuria, or the use of drugs such as corticosteroids, synthetic vitamin D, and nonsteroidal antiinflammatory drugs. "
6.	Mycophenolic Acid (Cellcept)FDA LinkGeneric 01/01/2015 - "We report a case of neuropsychiatric systemic lupus erythematosus successfully treated with mycophenolate mofetil (MMF). " 08/01/2005 - "Mycophenolate mofetil in neuropsychiatric systemic lupus erythematosus." 08/01/2005 - "We report a successfully treated case of neuropsychiatric systemic lupus erythematosus, presenting as psychosis, whose long-term remission was maintained on treatment with mycophenolate mofetil." 01/07/2022 - "Primary central nervous system lymphoma in a patient with neuropsychiatric systemic lupus erythematosus receiving mycophenolate mofetil: A case report and literature review." 10/15/2022 - "Neuropsychiatric Systemic Lupus Erythematosus with Cerebral Vasculitis and Lupus Nephritis Successfully Treated with High-dose Glucocorticoids and Mycophenolate Mofetil."
7.	Intravenous Immunoglobulins (IVIG)FDA Link 01/01/2022 - "Neuropsychiatric Systemic Lupus Erythematosus Presenting as Mania With Psychosis Treated Successfully With Intravenous Immunoglobulin: A Diagnosis of Associations and Exclusions." 02/01/2003 - "We describe a patient with central nervous system lupus who developed a loss of B cells with associated hypogammaglobulinemia and sinopulmonary infections requiring intravenous immunoglobulin. "
8.	Staphylococcal Protein A (Protein A)IBA 11/01/2020 - "We described protein A immunoadsorption combination with immunosuppressive treatment improved rapidly a patient with Neuropsychiatric systemic lupus erythematosus." 11/01/2020 - "Protein A immunoadsorption combination with immunosuppressive therapy improves neuropsychiatric systemic lupus erythematosus: A case report."
9.	Biomarkers (Surrogate Marker)IBA 03/01/2019 - "To explore the amplitude of low frequency fluctuation (ALFF) and functional connectivity (FC) disorders in non-neuropsychiatric systemic lupus erythematosus (non-NPSLE) patients by resting-state functional magnetic resonance imaging (rs-fMRI) and to study whether there are some clinical biomarkers that can be used to monitor the brain dysfunction. " 04/01/2017 - "Objectives The objective of this study was to determine whether advanced MRI could provide biomarkers for diagnosis and prognosis in neuropsychiatric systemic lupus erythematosus (NPSLE). " 10/01/2015 - "Due to the lack of reliable biomarkers in diagnosing and monitoring neuropsychiatric systemic lupus erythematosus (NPSLE), the aim of this study was to examine the utility of measurements obtained through spectral domain optical coherence tomography (SD-OCT) as a biomarker for NP involvement in SLE. " 01/01/2023 - "Novel CSF biomarkers for diagnosis and integrated analysis of neuropsychiatric systemic lupus erythematosus: based on antibody profiling." 01/30/2022 - "Biomarkers in Neuropsychiatric Systemic Lupus Erythematosus: A Systematic Literature Review of the Last Decade."
10.	AutoantibodiesIBA 11/01/2018 - "The present study was carried out to elucidate the roles of serum autoantibodies in the development of blood-brain barrier (BBB) damages in neuropsychiatric systemic lupus erythematosus (NPSLE). " 04/01/2004 - "We undertook this study to examine the presence of autoantibodies to MAP-2 in neuropsychiatric systemic lupus erythematosus (NPSLE). " 01/01/2023 - "The pathogenesis of neuropsychiatric systemic lupus erythematosus (NPSLE) is widely unknown, and the role of autoantibodies is still undetermined. " 01/01/2023 - "Brain-reactive autoantibodies in neuropsychiatric systemic lupus erythematosus." 12/01/2020 - "Absence of Neuronal Autoantibodies in Neuropsychiatric Systemic Lupus Erythematosus."

Therapies and Procedures

1.	Therapeutics 11/01/2012 - "Prospective study of clinical phenotypes in neuropsychiatric systemic lupus erythematosus; multidisciplinary approach to diagnosis and therapy." 05/01/2010 - "The dynamic changes of 21 patients in the neuropsychiatric systemic lupus erythematosus group before and after induction therapy were further analyzed. " 06/15/2003 - "[Drug alternatives in therapy of neuropsychiatric systemic lupus erythematosus (NPSLE)]." 01/01/2003 - "Central nervous system lupus: a clinical approach to therapy." 05/19/1991 - "A review of the diagnostic methods and therapy for central nervous system lupus is presented. "
2.	Plasmapheresis 04/01/2003 - "The role of plasmapheresis in the treatment of severe central nervous system neuropsychiatric systemic lupus erythematosus." 10/01/2001 - "We report our experience with use of plasmapheresis (PP) as an adjunct therapy in severe progressive neuropsychiatric systemic lupus erythematosus (NPSLE). " 12/01/2018 - "The cases in this clinical report will highlight a subtype of neuropsychiatric systemic lupus erythematosus demonstrating imaging findings of striatal inflammation responsive to plasmapheresis similar to those in the subset of N-methyl-D-aspartate receptor autoimmune encephalitis that involves the striatum. "
3.	Spinal Injections 04/29/2023 - "Correction: The influence of intrathecal injection of methotrexate and dexamethasone on neuropsychiatric systemic lupus erythematosus (NPSLE): a retrospective cohort study of 386 patients with NPSLE." 01/01/2023 - "The influence of intrathecal injection of methotrexate and dexamethasone on neuropsychiatric systemic lupus erythematosus (NPSLE): a retrospective cohort study of 386 patients with NPSLE." 11/01/2014 - "The objective is to explore the clinical curative effects of methylprednisolone combined with MTX and DXM intrathecal injection in treating neuropsychiatric systemic lupus erythematosus (NPSLE) and its effects on autoantibody level and anti-N-methyl-D-aspartate receptor subtype NR2a/2b antibody (anti-NR2 antibody) level. "
4.	Immunomodulation 07/01/2023 - "Bortezomib is efficacious in the treatment of severe childhood-onset neuropsychiatric systemic lupus erythematosus with psychosis: a case series and mini-review of B-cell immunomodulation in antibody-mediated diseases."
5.	Decompressive Craniectomy 01/01/2021 - "Neuropsychiatric systemic lupus erythematosus with cerebellar vasculitis and obstructive hydrocephalus requiring decompressive craniectomy."