NIH During the last decades, there has been an increased interest in the discovery and validation of biomarkers that reliably reflect specific aspects of lupus. DOI: 10.2174/138161208799316401. Neuropsychiatric (NP) events vary in frequency, complexity, time of onset, rates of resolution and recurrence. After exclusion of other causes such as concomitant illnesses, infection, or drug side effects, these neuropsychiatric manifestations are attrib-uted to involvement of the nervous system in SLE, which is referred to as neuropsychiatric SLE (NPSLE) (1). Systemic lupus erythematosus (SLE) is an autoimmune multisysytem disorder with myriad presentation. Patients with SLE are affected with the following condi - tions: skin rash, arthritis, serositis, such as peri - carditis and pleurisy, cytopenia, nephritis and … Google Scholar | Medline. doi: 10.7759/cureus.5424. These problems usually occur at times of lupus flares in other body organs as well (such as arthritis, skin rash, or kidney disease). eCollection 2019. Symptoms of neuropsychiatric systemic lupus erythematosus may range from mild diffuse ones, to acute life threatening events. Neuropsychiatric systemic lupus erythematosus in elderly people: a case series. The American College of Rheumatology (ACR) developed standardized nomenclature and case definitions for neuropsychiatric involvement in SLE (NPSLE) in 1999. Peripheral complement proteins in schizophrenia: A systematic review and meta-analysis of serological studies. Low prevalence of neuropsychiatric systemic lupus erythematosus (NPSLE) was found among patients with psychosis and positive antinuclear antibodies (ANA), suggesting there may be a low diagnostic yield for using ANA as a screening test for NPSLE in patients with psychosis, according to study results published in Arthritis Care & Research.. Disclosures, Dionysis Nikolopoulos holds a degree in Medicine from the University of Athens, Greece. Women of childbearing age and certain racial groups are typically predisposed to developing the condition. This review focuses on the pathophysiology, treatment, and new potential therapies for neuropsychiatric manifestations of systemic lupus erythematosus. This is recommended in focal manifestations, such as strokes and transient ischemic attacks. These syndromes can precede other symptoms of SLE or may occur at any point during the course of the disease, thus, the clinical presentation is extremely variable 2-7. Muslimov IA, Iacoangeli A, Eom T, Ruiz A, Lee M, Stephenson S, Ginzler EM, Tiedge H. J Neurosci. Rheum. Neuropsychiatric Involvement in Systemic Lupus Erythematosus:Current Therapeutic Approach Author(s): Giovanni Sanna , Maria Laura Bertolaccini , Munther A. Khamashta Department of Rheumatology,Homerton University Hospital, London E9 6SR, UK. Objective. Distinction between neuropsychiatric manifestations related to SLE (primary NPSLE) and those with other causes (non SLE-related) is of the utmost importance and represents a primary aim in clinical practice. NPSLE is one of the most difficult problems for people with lupus as it is often serious and also not well understood. Lupus. This hypothesis is supported by a study conducted in lupus-prone mice, which showed that treatment with a BTKi attenuates the neuropsychiatric disease. National Center for Biotechnology Information, Unable to load your collection due to an error, Unable to load your delegates due to an error. Introduction (2) (SLE). Mongan D, Sabherwal S, Susai SR, Föcking M, Cannon M, Cotter DR. Schizophr Res. 2012 Oct;42(2):179-85. doi: 10.1016/j.semarthrit.2012.03.011. High-quality clinical trials to guide therapeutic decisions are lacking, owing to the rarity and heterogeneity of NPSLE; which ranges from mild to devastating manifestations. Systemic lupus erythematosus (SLE) is a chronic disease that can affect multiple organ systems, often resulting in central nervous system (CNS), kidney, skin, and hematologic involvement 1. As mentioned above, an inflammatory process is usually involved in diffuse manifestations, such as refractory seizures, inflammatory optic neuritis, peripheral neuropathies, severe psychosis, aseptic meningitis, myelitis, and acute confusional state. 1-3. In another study in mice, angiotensin-converting enzyme inhibitors prevented microglia activation and preserved cognitive status and neuronal function. Nature Reviews Neurology, Vol. Epub 2020 May 24. Neuropsychiatric systemic lupus erythematosus (NPSLE) describes a wide range of brain-involved symptoms — symptoms like not thinking clearly, forgetting things or even seizures, stroke and psychosis—experienced by up to 95% of lupus patients. Central nervous system manifestations of systemic lupus erythematosus (CNS lupus) describe a wide variety of neuropsychiatric manifestations that are secondary to systemic lupus erythematosus (SLE) in the central nervous system (CNS). Neuropsychiatric systemic lupus erythematosus (NPSLE), comprised of numerous, complex central and peripheral nervous system symptoms, poses significant challenges at the bedside and even more in the laboratory. Neuropsychiatric systemic lupus erythematosus: pathophysiology and the future of treatment Systemic lupus erythematosus Systemic lupus erythematosus (SLE) is charac-terized by the presence of several autoantibodies, including anti-dsDNA antibodies [1,2]. This often results in a delay in diagnosis and appropriate treatment of the condition. Nevertheless, there is still a long way to go. Jia J, Xie J, Li H, Wei H, Li X, Hu J, et al. Epub 2019 Aug 31. Fragoso-Loyo H, et al. Author(s): Giovanni Sanna, Maria Laura Bertolaccini, Munther A. Khamashta. CrossRef; Google Scholar; Mani, Arash Shenavandeh, Saeedeh Sepehrtaj, Sayid Sadat and Javadpour, Ali 2015. | -, Crow M. Interferon-alpha: a therapeutic target in systemic lupus erythematosus. Neuropsychiatric systemic lupus erythematosus (NPSLE), comprised of numerous, complex central and peripheral nervous system symptoms, poses significant challenges at the bedside and even more in the laboratory. Different treatment regimens include nonsteroidal anti-inflammatory drugs, anticoagulation, and immunosuppressives such as cyclophosphamide, azathioprine, mycophenolate mofetil, and methotrexate. 1. seizure disorders 1.1. seizures and epilepsy are the most com… ... Hanly, JG, Su, L, Urowitz, MB Mood disorders in systemic lupus erythematosus: results from an international inception cohort study. When lupus affects the brain, spinal cord, or nerves, we call this neuropsychiatric SLE (NPSLE). Objectives Using a reversible multistate model, we prospectively examined neuropsychiatric (NP) events for attribution, outcome and association with health-related quality of life (HRQoL), in an international, inception cohort of systemic lupus erythematosus (SLE) patients. Muangchan C, van Vollenhoven RF, Bernatsky SR, Smith CD, Hudson M, Inanç M, Rothfield NF, Nash PT, Furie RA, Senécal JL, Chandran V, Burgos-Vargas R, Ramsey-Goldman R, Pope JE. Neuropsychiatric symptoms affect nearly half of the patients with systemic lupus erythematosus; however, the effect on disease severity, quality of life, and prognosis is tremendous. She had a 19-year history of atypical epileptic seizures and cognitive decline. Magro-Checa C, Beaart-van de Voorde LJ, Middelkoop HA, Dane ML, van der Wee NJ, van Buchem MA, Huizinga TW, Steup-Beekman GM. Borowoy AM, Pope JE, Silverman E, Fortin PR, Pineau C, Smith CD, Arbillaga H, Gladman D, Urowitz M, Zummer M, Hudson M, Tucker L, Peschken C. Semin Arthritis Rheum. Memory and learning functions in patients with systemic lupus erythematosus: A neuropsychological case-control study. To this end, all available diagnostic information, including lumbar puncture, neuroimaging, and presence of risk factors, should be taken into account to support the clinical evaluation. -. Patients with SLE and … Background: Neuropsychiatric systemic lupus erythematosus (NPSLE) occurs up to 75% of adult SLE patients, and is one of the major causes of death in SLE patients. Part of the Springer Nature Group. Objectives Using a reversible multistate model, we prospectively examined neuropsychiatric (NP) events for attribution, outcome and association with health-related quality of life (HRQoL), in an international, inception cohort of systemic lupus erythematosus (SLE) patients. Recent findings. Journal Name: Current Pharmaceutical Design. 1. SLE is a disease in which the immune system attacks the body's own cells and tissues. USA.gov. There are limited reports on the neuropsychiatric findings as the first manifestation of systemic lupus erythematosus in male patients. Expression of APOBEC family members as regulators of endogenous retroelements and malignant transformation in systemic autoimmunity. 2010;36(1):173–186. The underlying pathogenesis, severity, and diversity of manifestations make management of primary NPSLE particularly challenging. To facilitate the process of attribution, a number of “attribution models” have been proposed, most recently by the Italian Study Group on NPSLE. Association of cerebrospinal fluid anti-NR2 glutamate receptor antibodies with diffuse neuropsychiatric systemic lupus erythematosus. Central nervous system (CNS) infection is a consequence of intensive immunosuppressive therapy that patients with SLE might undergo. Brain imaging, especially magnetic resonance imaging, is frequently used to diagnose or exclude overt cerebral pathologies such as edema, hemorrhage, and central thrombosis. Neuropsychiatric systemic lupus erythematosus or NPSLE refers to the neurological and psychiatric manifestations of systemic lupus erythematosus. The SLICC criteria introduced an essentially complete list of these manifestations, which, with chronic cutaneous LE, included hypertrophic (verrucous) lupus, lupus panniculitis (lupus profundus), mucosal lupus, lupus erythematosus tumidus, chilblains lupus and discoid lupus/lichen planus overlap, in addition to localized or generalized classic discoid rash . Abstract. In the case of a presumed thrombotic mechanism associated with aPL (antiphospholipid syndrome [APS]), anticoagulation therapy is recommended for secondary prevention. Autoantibody profiling in systemic lupus erythematosus Song-Chou Hsieh,1 Chia-Li Yu1,2 1Department of Internal Medicine, 2Institute of Molecular Medicine, National Taiwan University College of Medicine, Taipei, Taiwan Abstract: Systemic lupus erythematosus (SLE) is an archetype of systemic autoimmune disease characterized by the production of a broad spectrum of autoantibodies. Neuropsychiatric systemic lupus erythematosus: pathogenesis and biomarkers. HHS 13-08-2019 | Systemic lupus erythematosus | Editorial | Article. Keeping you informed. When a non-thrombotic manifestation is accompanied by the persistent presence of aPL antibodies at moderate-to-high titers, antiplatelet therapy only is sufficient. Brain imaging, especially magnetic resonance imaging, is frequently used to diagnose or exclude overt cerebral pathologies such as edema, hemorrhage, and central thrombosis. Get the latest public health information from CDC: https://www.coronavirus.gov, Get the latest research information from NIH: https://www.nih.gov/coronavirus, Find NCBI SARS-CoV-2 literature, sequence, and clinical content: https://www.ncbi.nlm.nih.gov/sars-cov-2/. systemic lupus erythematosus (SLE) develop neurologic or psychiatric symptoms. Magro-Checa C, Zirkzee EJ, Huizinga TW, Steup-Beekman GM. aPL persistently positive at moderate-to-high titers. Lupus of the nervous system may involve symptoms. For refractory NPSLE, intravenous immunoglobulin (IVIG), plasmapheresis, and rituximab have been used. COVID-19 is an emerging, rapidly evolving situation. In the last decades, the rate of successful pregnancies for these women has greatly increased, thanks to the general improvement in SLE management, but also to the availability of drugs compatible with pregnancy and lactation ( Lazzaroni et al., 2016 ). past or synchronous major neuropsychiatric event; and. Clipboard, Search History, and several other advanced features are temporarily unavailable. In moderate cases, glucocorticoids with or without immunosuppressive therapy (cyclophosphamide/ azathioprine/ mycophenolate mofetil) should be administered. 2006;15(5):308–318. Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives. Background: Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disorder characterized by multiple affected systems. Cerebral blood flow abnormalities in neuropsychiatric systemic lupus erythematosus. Neuropsychiatric systemic lupus erythematosus (NPSLE) 2 manifestations are more frequent among individuals with disease onset during childhood (cSLE) compared with SLE diagnosed during … 2016 Mar;76(4):459-83. doi: 10.1007/s40265-015-0534-3. A consideration of pathologic mechanisms stratified by anatomic location allows enhanced understanding of diverse symptomatology, kinetics of disease, symptom … The pathogenesis of NPSLE is multifactorial and involves various inflammatory cytokines, autoantibodies, and immune complexes resulting in vasculopathic, cytotoxic and autoantibody-mediated neuronal injury. Arthritis Rheumatol 2015; 67: 1837 – 1847. Journal Home. The pathogenesis of NPSLE is multifactorial and involves various inflammatory cytokines, autoantibodies, and immune complexes resulting in vasculopathic, cytotoxic and autoantibody-mediated neuronal injury. Neuropsychiatric Systemic Lupus Erythematosus, Anti-Ribosomal P Antibodies, Anti-NR2 Glutamate Receptor Binding Antibodies, Interleukin-6, Granulocyte-Colony Stimulating Factor, The Blood-Brain Barrier . ↵ Mok CC, Lanchbury JS, Wai Chan D, Sing Lau C (1998) Interleukin-10 promoter polymorphisms in southern Chinese patients with systemic lupus erythematosus. In 1999, the American College of Rheumatology developed 19 discrete neuropsychiatric syndromes that comprised NPSLE. Systemic lupus erythematosus (SLE) is an autoimmune disease that affects the skin and musculoskeletal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, … doi:10.1002/art.23399 OpenUrl CrossRef PubMed Web of Science Diversity of neuropsychiatric manifestations in systemic lupus erythematosus. Epub 2019 Aug 12. Herein, we report a case of a male patient who presented with a five … Clinical approaches have shown that neuropsychiatric (NP) symptoms such as de- pression, psychosis, seizures, headache, cogni- tive disorder and stroke occur in … However, results of a systematic review suggest a target INR of 3–4 for secondary APS, without an increase in major bleeding risk. Systemic lupus erythematosus (SLE) is a disorder which can affect the central nervous system and result in a broad range of psychiatric syndromes such as psychosis, mood disorders, acute confusion and cognitive dysfunction. Neuropsychiatric lupus: the prevalence and autoantibody associations depend on the definition: results from the 1000 faces of lupus cohort. Danchenko N, Satia J, Anthony M. Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. NPSLE is associated with increased morbidity and mortality. 2013 Apr;61(4):262-7. Huang MW, Stock AD, Mike EV, Herlitz L, Kolbeck R, Putterman C. Lupus. Distinction between neuropsychiatric manifestations related to SLE (primary NPSLE) and those with other causes (non SLE-related) is of the utmost importance and represents a primary aim in clinical practice. Neuropsychiatric lupus (NPSLE) is a severe and potentially life-threatening condition, reported to occur in 25%–70% of patients with systemic lupus erythematosus (SLE). A breakthrough study demonstrated that type I interferon (IFN) activates microglia, which then engulf synaptic material, leading to cognitive impairment. A 58-year-old woman presented to neuropsychiatric services with increased frequency of confusional episodes and intermittent psychotic symptoms. It is estimated that over half of people with SLE have neuropsychiatric involvement. Key words:Systemic lupus erythematosus, Multistate modeling, Neuropsychiatric 7 Nervous system disease in SLE consists of neurologic and psychiatric events, predominantly affecting the central nervous system(1). This suggests that systemic lupus erythematosus (SLE) is more common in elderly people than was originally thought and is a potentially treatable cause of organic brain disorder. The initial step in the management of NPSLE is to treat any secondary cause, if present, as well as to symptomatically treat neuropsychiatric manifestations with anticonvulsants, antidepressants, or antipsychotics. Disclosures, 07-05-2019 | Systemic lupus erythematosus | Podcast | Article, 09-04-2019 | Systemic lupus erythematosus | News, 20-06-2019 | Systemic lupus erythematosus | Highlight | Article. Epub 2020 May 27. Neuropsychiatric systemic lupus erythematosus (NPSLE) is a manifestation of systemic lupus erythematosus (SLE). 10. As systemic lupus erythematosus (SLE) is a notoriously demanding disease, involvement of the nervous system, collectively termed neuropsychiatric SLE (NPSLE), represents the foremost diagnostic and therapeutic challenge for the treating physician. Med. Central nervous system damage, a major organ manifestation of systemic lupus erythematosus (SLE), causes significant morbidity and mortality. In this study, mice treated with the type I IFN receptor-blocking antibody anifrolumab exhibited attenuation of CNS disease. Disclosures, Antonis Fanouriakis is consultant rheumatologist at Asklepieion General Hospital and Attikon University General Hospital, Athens, Greece. Neuropsychiatric systemic lupus erythematosus (NPSLE) is a manifestation of systemic lupus erythematosus (SLE). North Am. 961203319861677. . First, other causes (non-SLE factors) such as infection, malignancy, metabolic disorders, and drug-related adverse events should be excluded through clinical examination, lab tests, and neuroimaging (magnetic resonance imaging); the initial work-up for a particular manifestation should be similar to that carried out in a patient without lupus. Putting research into context. Neuropsychiatric systemic lupus erythematosus (NPSLE) is the least understood, yet perhaps the most prevalent manifestation of lupus. 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