Review
Pharmacology & Pharmacy
Daniel Mrak, Michael Bonelli, Helga Radner
Summary: Systemic Lupus Erythematosus (SLE) is an autoimmune disease with complex and diverse manifestations. Involvement of the nervous system, known as neuropsychiatric lupus (NPSLE), presents with a wide range of symptoms. However, diagnosing and treating NPSLE remains challenging due to the lack of specific biomarkers or imaging techniques, and limited therapeutic options.
CURRENT PHARMACEUTICAL DESIGN
(2022)
Article
Immunology
Mathilde Labouret, Stefania Costi, Vincent Bondet, Vincent Trebossen, Enora Le Roux, Alexandra Ntorkou, Sophie Bartoli, Stephane Auvin, Brigitte Bader-Meunier, Veronique Baudouin, Olivier Corseri, Glory Dingulu, Camille Ducrocq, Cecile Dumaine, Monique Elmaleh, Nicole Fabien, Albert Faye, Isabelle Hau, Veronique Hentgen, Theresa Kwon, Ulrich Meinzer, Naim Ouldali, Cyrielle Parmentier, Marie Pouletty, Florence Renaldo, Isabelle Savioz, Flore Rozenberg, Marie-Louise Fremond, Alice Lepelley, Gillian I. Rice, Luis Seabra, Jean-Francois Benoist, Darragh Duffy, Yanick J. Crow, Pierre Ellul, Isabelle Melki
Summary: This study aimed to identify central nervous system (CNS) disease biomarkers of juvenile neuropsychiatric SLE (j-NPSLE). The results showed that cerebrospinal fluid (CSF) levels of neopterin and interferon-alpha (IFN-alpha) protein were significantly higher in active j-NPSLE cases with CNS involvement compared to j-SLE alone. These biomarkers could potentially be useful in the diagnosis and monitoring of j-NPSLE activity.
JOURNAL OF CLINICAL IMMUNOLOGY
(2022)
Article
Immunology
Jianing Wang, Jingyi Xu, Pingting Yang
Summary: Patients with systemic lupus erythematosus often experience nervous system complications called neuropsychiatric lupus erythematosus. Vascular injuries and neuroinflammation are the main pathological changes in neuropsychiatric lupus erythematosus. The specific autoimmune-mediated central nervous system damages differentiate neuropsychiatric lupus erythematosus from other CNS disorders.
JOURNAL OF AUTOIMMUNITY
(2022)
Article
Immunology
Cristina Cocco, Elias Manca, Giulia Corda, Maria Maddalena Angioni, Barbara Noli, Mattia Congia, Francesco Loy, Michela Isola, Elisabetta Chessa, Alberto Floris, Lorena Lorefice, Luca Saba, Alessandro Mathieu, Gian Luca Ferri, Alberto Cauli, Matteo Piga
Summary: Brain-reactive autoantibodies are found in patients with SLE and NPSLE, with higher frequency and titers observed in NPSLE patients. The target antigens of these autoantibodies are still undetermined, but they likely include β2GPI.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Medicine, General & Internal
Minhui Wang, Ziqian Wang, Shangzhu Zhang, Yang Wu, Zhang Li, Jiuliang Zhao, Qian Wang, Xinping Tian, Mengtao Li, Xiaofeng Zeng
Summary: Neuropsychiatric systemic lupus erythematosus (NPSLE) is a disease with diverse subtypes and severities, involving ischemic and inflammatory mechanisms as well as brain-intrinsic factors. Understanding the underlying mechanisms of NPSLE may lead to new treatment options, as shown in mouse models. However, current conventional therapies are only supported by observational studies, highlighting the need for controlled clinical trials.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Clinical Neurology
Jonathan S. Emerson, Simon M. Gruenewald, Lavier Gomes, Ming-Wei Lin, Sanjay Swaminathan
Summary: Recognizing neuropsychiatric involvement by SLE is essential but currently available diagnostic algorithms have many barriers that may delay diagnosis and treatment. The non-specific symptoms, markers, and neuroimaging findings of NPSLE highlight research gaps. Neuropsychological assessments and novel markers show promise in improving recognition. A composite panel of investigations may be needed to address the recognition gaps.
FRONTIERS IN NEUROLOGY
(2023)
Article
Rheumatology
Teresa Giani, Eve Md Smith, Eslam Al-Abadi, Kate Armon, Kathryn Bailey, Coziana Ciurtin, Joyce Davidson, Janet Gardner-Medwin, Kirsty Haslam, Dan P. Hawley, Alice Leahy, Valentina Leone, Flora McErlane, Devesh Mewar, Gita Modgil, Robert Moots, Clarissa Pilkington, Francesca Pregnolato, Athimalaipet Ramanan, Satyapal Rangaraj, Phil Riley, Arani Sridhar, Nick Wilkinson, Rolando Cimaz, Michael W. Beresford, Christian M. Hedrich
Summary: A significant proportion of JSLE patients experience NP involvement (25%), with common neurological symptoms including headaches, mood disorders, and cognitive impairment. NP-JSLE patients exhibit higher disease activity and damage compared to those without NP involvement.
Article
Neuroimaging
Marjolein Bulk, Thijs van Harten, Boyd Kenkhuis, Francesca Inglese, Ingrid Hegeman, Sjoerd van Duinen, Ece Ercan, Cesar Magro-Checa, Jelle Goeman, Christian Mawrin, Mark van Buchem, Gerda Steup-Beekman, Tom Huizinga, Louise van der Weerd, Itamar Ronen
Summary: Neuroinflammation in NPSLE does not necessarily accompany iron accumulation, suggesting that the inflammatory pathomechanism in SLE may differ from neurodegenerative diseases and multiple sclerosis.
NEUROIMAGE-CLINICAL
(2021)
Review
Immunology
Irene Carrion-Barbera, Tarek Carlos Salman-Monte, F. Vilchez-Oya, Jordi Monfort
Summary: The neuropsychiatric involvement in systemic lupus erythematosus poses challenges for clinicians at both diagnostic and therapeutic levels. The prevalence of NPSLE varies widely due to factors such as different definitions, study designs, populations, and follow-up procedures. Authors have attempted to standardize definitions and develop tools to aid in diagnosis, while EULAR has provided management recommendations.
AUTOIMMUNITY REVIEWS
(2021)
Review
Rheumatology
Takanori Ichikawa, Yasuhiro Shimojima, Dai Kishida, Tomoki Kaneko, Yoshiki Sekijima
Summary: This case report presents a rare occurrence of PCNSL in a patient with NPSLE, with histological evidence confirming B-cell lymphoma positive for Epstein-Barr virus-encoded RNA. Treatment involving surgery, radiation therapy, rituximab infusion, and belimumab administration resulted in the patient's recovery. Additionally, the study suggests that CNS damage and long-term exposure to immunosuppressants may contribute to the development of PCNSL by impacting oncogenic immune responses within the CNS.
RHEUMATOLOGY INTERNATIONAL
(2021)
Review
Immunology
Tao Ming Sim, Anselm Mak, Sen Hee Tay
Summary: Central nervous system involvement in systemic lupus erythematosus (SLE), known as neuropsychiatric SLE (NPSLE), is a severe manifestation of the disease. The immunopathogenesis of NPSLE is complex, with neutrophils playing a central role. Understanding the mechanisms behind NPSLE and targeting neutrophils could be crucial for translational research in NPSLE.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Valeria Rella, Cinzia Rotondo, Alberto Altomare, Francesco Paolo Cantatore, Addolorata Corrado
Summary: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations. Dysregulation of the immune system due to genetic, hormonal, and environmental factors can lead to various complications, including bone involvement such as osteoporosis.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Immunology
Martin Valdez-Lopez, Eduardo Aguirre-Aguilar, Sergio Ivan Valdes-Ferrer, Francisco M. Martinez-Carrillo, Antonio Arauz, Ana Barrera-Vargas, Javier Merayo-Chalico
Summary: Posterior Reversible Encephalopathy Syndrome (PRES) is an acute neurological syndrome characterized by seizures, altered mental status, headache, and visual disturbances. It is often associated with Systemic Lupus Erythematosus (SLE), with SLE causing alterations in pathways implicated in the development of PRES. This suggests that PRES in the setting of SLE may be considered a distinct neuropsychiatric syndrome.
AUTOIMMUNITY REVIEWS
(2021)
Article
Immunology
Yihan Wang, Yating Ren, Tao Hong, Dingqi Lu, Fan Zhang, Yi Cao, Xinchang Wang
Summary: This study used lipidomic analysis to explore the influence of the liver-brain axis on neuropsychiatric lupus (NPSLE) in mice. The results showed differential expression of lipid metabolites in the hippocampus and liver, with the glycerophospholipid metabolism pathway being most relevant to depressive behavior in mice.
JOURNAL OF INFLAMMATION RESEARCH
(2022)
Article
Immunology
Xiaoyue Qiao, Li Lu, Kangxing Zhou, Liping Tan, Xuan Liu, Jiali Ni, Yayi Hou, Jun Liang, Huan Dou
Summary: This study evaluated the correlation between PRG2 and NPSLE for the first time. The findings showed that PRG2 protein was overexpressed in NPSLE patients and correlated with the subset scores of psychosis. Additionally, the study revealed a correlation between hippocampal PRG2 level and learning and memory ability in mice.
CLINICAL IMMUNOLOGY
(2022)