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https://www.readbyqxmd.com/read/29342508/features-treatment-and-outcomes-of-macrophage-activation-syndrome-in-childhood-onset-systemic-lupus-erythematosus
#1
R Ezequiel Borgia, Maya Gerstein, Deborah M Levy, Earl D Silverman, Linda T Hiraki
OBJECTIVE: To describe the features and treatment of macrophage activation syndrome (MAS) in a childhood-onset systemic lupus erythematosus (cSLE) single centre cohort, and compare the cSLE manifestations and outcomes between those with and without MAS. METHODS: We included all cSLE patients followed at SickKids Hospital from 2002-12, and identified those also diagnosed with MAS. Demographic, clinical and laboratory features of MAS and SLE, medication use, hospital and PICU admissions as well as damage indices and mortality were extracted from the Lupus database...
January 17, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29328521/the-central-vein-sign-differentiates-ms-from-cns-inflammatory-vasculopathies
#2
Pietro Maggi, Martina Absinta, Matteo Grammatico, Luisa Vuolo, Giacomo Emmi, Giovanna Carlucci, Gregorio Spagni, Alessandro Barilaro, Anna Maria Repice, Lorenzo Emmi, Domenico Prisco, Vittorio Martinelli, Roberta Scotti, Niloufar Sadeghi, Gaetano Perrotta, Pascal Sati, Bernard Dachy, Daniel S Reich, Massimo Filippi, Luca Massacesi
OBJECTIVES: In multiple sclerosis (MS), MRI is a sensitive tool for detecting white matter lesions, but its diagnostic specificity is still suboptimal; ambiguous cases are frequent in clinical practice. Detection of perivenular lesions in the brain (the "central vein sign") improves the pathological specificity of MS diagnosis, but comprehensive evaluation of this MRI biomarker in MS-mimicking inflammatory and/or autoimmune diseases, such as CNS inflammatory vasculopathies, is missing...
January 12, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29178933/the-chronically-inflamed-central-nervous-system-provides-niches-for-long-lived-plasma-cells
#3
Karolin Pollok, Ronja Mothes, Carolin Ulbricht, Alina Liebheit, Jan David Gerken, Sylvia Uhlmann, Friedemann Paul, Raluca Niesner, Helena Radbruch, Anja Erika Hauser
Although oligoclonal bands in the cerebrospinal fluid have been a hallmark of multiple sclerosis diagnosis for over three decades, the role of antibody-secreting cells in multiple sclerosis remains unclear. T and B cells are critical for multiple sclerosis pathogenesis, but increasing evidence suggests that plasma cells also contribute, through secretion of autoantibodies. Long-lived plasma cells are known to drive various chronic inflammatory conditions as e.g. systemic lupus erythematosus, however, to what extent they are present in autoimmune central nervous system inflammation has not yet been investigated...
November 25, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29067848/distinct-clinical-correlates-of-immune-thrombocytopenic-purpura-at-diagnosis-of-childhood-onset-and-adult-sle
#4
Gladys Cherres Xavier Esteves, Natali Weniger Spelling Gormezano, Oriany L Pereira, David Kern, Clovis Almeida Silva, Rosa Maria Rodrigues Pereira, Katia Tomie Kozu, Eloisa Bonfá, Nadia Emi Aikawa
OBJECTIVES: To compare clinical and laboratorial features between childhood-onset systemic lupus erythematosus (cSLE) and adult SLE (aSLE) at concomitant diagnosis of immune thrombocytopenic purpura (ITP). METHODS: This study evaluated 56 cSLE and 73 aSLE patients regularly followed at Pediatric and Rheumatology Divisions of the same University hospital with ITP (platelets count <100,000/mm3 in the absence of other causes) at lupus onset. RESULTS: Median current age was 11...
October 25, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29045736/clinical-trial-parameters-that-influence-outcomes-in-lupus-trials-that-use-the-systemic-lupus-erythematosus-responder-index
#5
Kenneth C Kalunian, Murray B Urowitz, David Isenberg, Joan T Merrill, Michelle Petri, Richard A Furie, Mary-Ann Morgan-Cox, Rebecca Taha, Steven Watts, Maria Silk, Matthew D Linnik
Objective: The SLE Responder Index (SRI) is a composite endpoint used in SLE trials. This investigation examined the clinical trial elements that drive response measured by the SRI. Methods: Analyses are based on data from two phase 3 trials (n = 2262) that evaluated the impact of an anti-B-cell activating factor antibody on disease activity using SRI-5 as the primary endpoint (ClinicalTrials.gov NCT01196091 and NCT01205438). Results: The SRI-5 response rate at week 52 for all patients was 32...
October 17, 2017: Rheumatology
https://www.readbyqxmd.com/read/28955334/the-role-of-brain-reactive-autoantibodies-in-brain-pathology-and-cognitive-impairment
#6
REVIEW
Simone Mader, Lior Brimberg, Betty Diamond
Antibodies to different brain proteins have been recently found to be associated with an increasing number of different autoimmune diseases. They need to penetrate the blood-brain barrier (BBB) in order to bind antigens within the central nervous system (CNS). They can target either neuronal or non-neuronal antigen and result in damage either by themselves or in synergy with other inflammatory mediators. Antibodies can lead to acute brain pathology, which may be reversible; alternatively, they may trigger irreversible damage that persists even though the antibodies are no longer present...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28913566/adar-rna-editing-in-human-disease-more-to-it-than-meets-the-i
#7
REVIEW
Angela Gallo, Dragana Vukic, David Michalík, Mary A O'Connell, Liam P Keegan
We review the structures and functions of ADARs and their involvements in human diseases. ADAR1 is widely expressed, particularly in the myeloid component of the blood system, and plays a prominent role in promiscuous editing of long dsRNA. Missense mutations that change ADAR1 residues and reduce RNA editing activity cause Aicardi-Goutières Syndrome, a childhood encephalitis and interferonopathy that mimics viral infection and resembles an extreme form of Systemic Lupus Erythmatosus (SLE). In Adar1 mouse mutant models aberrant interferon expression is prevented by eliminating interferon activation signaling from cytoplasmic dsRNA sensors, indicating that unedited cytoplasmic dsRNA drives the immune induction...
September 2017: Human Genetics
https://www.readbyqxmd.com/read/28882191/effects-of-sustained-i-c-v-infusion-of-lupus-csf-and-autoantibodies-on-behavioral-phenotype-and-neuronal-calcium-signaling
#8
Minesh Kapadia, Dunja Bijelić, Hui Zhao, Donglai Ma, Ljudmila Stojanovich, Milena Milošević, Pavle Andjus, Boris Šakić
Systemic lupus erythematosus (SLE) is a potentially fatal autoimmune disease that is often accompanied by brain atrophy and diverse neuropsychiatric manifestations of unknown origin. More recently, it was observed that cerebrospinal fluid (CSF) from patients and lupus-prone mice can be neurotoxic and that acute administration of specific brain-reactive autoantibodies (BRAs) can induce deficits in isolated behavioral tasks. Given the chronic and complex nature of CNS SLE, the current study examines broad behavioral performance and neuronal Ca(2+) signaling in mice receiving a sustained infusion of cerebrospinal fluid (CSF) from CNS SLE patients and putative BRAs (anti-NR2A, anti-ribosomal P, and anti-α-tubulin)...
September 7, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28854831/autoimmune-diseases-induced-by-biological-agents-a-review-of-12-731-cases-biogeas-registry
#9
REVIEW
Marta Pérez-De-Lis, Soledad Retamozo, Alejandra Flores-Chávez, Belchin Kostov, Roberto Perez-Alvarez, Pilar Brito-Zerón, Manuel Ramos-Casals
Biological drugs are therapies designed to target a specific molecule of the immune system that have been linked with the development of autoimmune diseases. Areas covered: The BIOGEAS Registry currently collects information about nearly 13,000 reported cases of autoimmune diseases developed in patients exposed to biologics, including more than 50 different systemic and organ-specific autoimmune disorders, of which psoriasis (n=6375), inflammatory bowel disease (n=845), demyelinating CNS disease (n=803), interstitial lung disease (n=519) and lupus (n=369) were the most frequently reported...
November 2017: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/28798699/schizophrenia-or-atypical-lupus-erythematosus-with-predominant-psychiatric-manifestations-over-25%C3%A2-years-case-analysis-and-review
#10
Axel Mack, Christiane Pfeiffer, E Marion Schneider, Karl Bechter
We observed a case over 25 years of relapsing-remitting schizophrenic spectrum disorder, varying regarding the main symptomatology between more depressive or more schizoaffective or rather typical schizophrenic syndrome. Diseased phases were repeatedly accompanied by minor skin lesions, which were initially classified as mixed tissue disorder. Psychotic phases were waxing-waning over years. During one later relapse, skin involvement was severe, classified to likely represent an allergic reaction to psychopharmaca; this generalized exanthema remitted rapidly with cortisone treatment and azathioprine...
2017: Frontiers in Psychiatry
https://www.readbyqxmd.com/read/28774170/an-autopsy-case-of-epstein-barr-virus%C3%A2-associated-diffuse-large-b-cell-lymphoma-of-the-central-nervous-system-in-an-immunocompromised-host
#11
Sun-Young Park, Seong Ik Kim, Hannah Kim, Yoojin Lee, Sung-Hye Park
Lymphomas arising in the central nervous system (CNS) of immunocompromised hosts represent non-Hodgkin's lymphomas exclusively associated with Epstein-Barr virus (EBV). Here we report an autopsy case of EBV-associated CNS diffuse large B-cell lymphoma (DLBCL) in a host suffering from systemic lupus erythematosus who underwent immunosuppressive therapy. After autopsy, EBV-associated CNS DLBCL as well as pulmonary mixed aspergillosis and Pneumocystis jirovecii pneumonia were added to the clinical impressions of complicated pneumonia and cerebral hemorrhage in this immunocompromised patient...
August 4, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28764615/cerebral-tuberculosis-in-a-patient-with-systemic-lupus-erythematosus-following-cyclophosphamide-treatment-a-case-report
#12
S Cooray, H Zhang, R Breen, G Carr-White, R Howard, M Cuadrado, D D'Cruz, G Sanna
Central nervous system (CNS) tuberculosis (TB) is a rare but catastrophic event in patients with systemic lupus erythematosus (SLE). Here we report a case of cerebral TB in a patient with lupus myocarditis and nephritis, following cyclophosphamide immunosuppression. To our knowledge this is the first reported case of cerebral TB in SLE in a non-endemic country. A 31-year-old female with SLE and a history of regular travel to Kenya presented to our centre with clinical features of acute heart failure. She was diagnosed with severe lupus myocarditis, and a renal biopsy also confirmed lupus nephritis...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28712818/systemic-lupus-erythematosus-and-thyroid-disease-experience-in-a-single-medical-center-in-taiwan
#13
Yu-Chuan Liu, Wen-Ya Lin, Ming-Chin Tsai, Lin-Shien Fu
BACKGROUND: To investigate the association of systemic lupus erythematosus (SLE) with thyroid diseases in a medical center in central Taiwan. METHODS: This is a retrospective cohort of 2796 SLE patients in a tertiary referral medical center from 2000 to 2013. We screened SLE by catastrophic illness registration from national insurance bureau; and thyroid diseases by ICD 9 codes, then confirmed by thyroid function test, auto-antibody, medical and/or surgical intervention...
June 28, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/28656478/autoantibodies-against-myelin-sheath-and-s100%C3%AE-are-associated-with-cognitive-dysfunction-in-patients-with-rheumatoid-arthritis
#14
Talita Siara Almeida Baptista, Laura Esteves Petersen, Julia K Molina, Tatiana de Nardi, Andréa Wieck, Aline do Prado, Deise Marcela Piovesan, Mauro Keisermann, Rodrigo Grassi-Oliveira, Moisés Evandro Bauer
Rheumatoid arthritis (RA) has been associated with cognitive impairment and peripheral production of autoantibodies. Autoantibodies against central nervous system (CNS) proteins and S100 calcium-binding β (S100β) were found increased in diseases characterized by cognitive impairment like Alzheimer disease and Neuropsychiatric Systemic Lupus Erythematosus (NPSLE). The aim of this study was to investigate the plasma levels of autoantibodies against myelin basic protein (anti-MBP), myelin oligodendrocyte glycoprotein (anti-MOG) and S100β, and their relationships with cognitive performance in RA patients...
June 27, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28614301/microglia-dependent-synapse-loss-in-type-i-interferon-mediated-lupus
#15
Allison R Bialas, Jessy Presumey, Abhishek Das, Cees E van der Poel, Peter H Lapchak, Luka Mesin, Gabriel Victora, George C Tsokos, Christian Mawrin, Ronald Herbst, Michael C Carroll
Systemic lupus erythematosus (SLE) is an incurable autoimmune disease characterized by autoantibody deposition in tissues such as kidney, skin and lungs. Notably, up to 75% of patients with SLE experience neuropsychiatric symptoms that range from anxiety, depression and cognitive impairment to seizures and, in rare cases, psychosis-collectively this is referred to as central nervous system (CNS) lupus. In some cases, certain autoantibodies, such as anti-NMDAR or anti-phospholipid antibodies, promote CNS lupus...
June 22, 2017: Nature
https://www.readbyqxmd.com/read/28545366/role-of-positron-emission-tomography-for-central-nervous-system-involvement-in-systemic-autoimmune-diseases-status-and-perspectives
#16
Daniele Mauro, Gaetano Barbagallo, Salvatore D'Angelo, Sannino Pasqualina, Saverio Naty, Caterina Bruno, Ignazio Olivieri, Rosa Daniela Grembiale, Francesco Ursini
In the last years, an increasing interest in molecular imaging has been raised by the extending potential of positron emission tomography (PET). The role of PET imaging, originally confined to the oncology setting, is continuously extending thanks to the development of novel radiopharmaceutical and to the implementation of hybrid imaging techniques, where PET scans are combined with computed tomography (CT) or magnetic resonance imaging(MRI) in order to improve spatial resolution. Early preclinical studies suggested that 18F-FDG PET can detect neuroinflammation; new developing radiopharmaceuticals targeting more specifically inflammation-related molecules are moving in this direction...
May 23, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28529699/evolution-immunity-and-the-emergence-of-brain-superautoantigens
#17
Serge Nataf
While some autoimmune disorders remain extremely rare, others largely predominate the epidemiology of human autoimmunity. Notably, these include psoriasis, diabetes, vitiligo, thyroiditis, rheumatoid arthritis and multiple sclerosis. Thus, despite the quasi-infinite number of "self" antigens that could theoretically trigger autoimmune responses, only a limited set of antigens, referred here as superautoantigens, induce pathogenic adaptive responses. Several lines of evidence reviewed in this paper indicate that, irrespective of the targeted organ (e...
2017: F1000Research
https://www.readbyqxmd.com/read/28479183/numerous-fusiform-and-saccular-cerebral-aneurysms-in-central-nervous-system-lupus-presenting-with-ischemic-stroke
#18
Shahram Majidi, Christopher R Leon Guerrero, Shreya Gandhy, Kathleen M Burger, Dimitri Sigounas
Central nervous system (CNS) involvement occurs in up to 50% of patients with systemic lupus erythematosus (SLE). Cerebral aneurysm formation is a rare complication of CNS lupus. The majority of these patients present with subarachnoid hemorrhage. We report a patient with an active SLE flare who presented with a recurrent ischemic stroke and was found to have numerous unruptured fusiform and saccular aneurysms in multiple vascular territories. He was treated with high-dose steroid and rituximab along with aspirin and blood pressure control for stroke prevention...
May 4, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28428903/sle-and-non-hodgkin-s-lymphoma-a-case-series-and-review-of-the-literature
#19
Prajwal Boddu, Abdul S Mohammed, Chandrahasa Annem, Winston Sequeira
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder punctuated by varied multiorgan complications all along the course of its natural history. Lymphoma represents a relatively well-recognized malignant phenomenon associated with lupus. The cause and effect relationships of lymphoma in SLE have been subject to extensive scrutiny with several studies reporting on clinic-pathologic characteristics and risk factors predicting lymphoma development in SLE. However, the pathogenic role of immunosuppressives in SLE-related lymphoma still remains unclear, and indices to help guide diagnosis, prognostication, therapy, and posttreatment monitoring are yet to be established...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28420057/central-nervous-system-vasculitis-in-systemic-lupus-erythematosus-a-case-series-report-in-a-tertiary-referral-centre
#20
M Rodrigues, O Galego, C Costa, D Jesus, P Carvalho, M Santiago, A Malcata, L Inês
Central nervous system (CNS) vasculitis (CNS) in systemic erythematosus lupus (SLE) is a rare and challenging diagnosis. We report four cases of CNS vasculitis that occurred 5 to 16 years after the diagnosis of SLE. Magnetic resonance imaging (MRI) detected different features suggestive of CNS vasculitis: enhancement and thickening of the vascular wall, vascular stenosis, ischemic brain lesions and intracerebral haemorrhage unlikely to correspond to other mimic aetiologies. Three patients received combination therapy with glucocorticoids (GC) and cyclophosphamide (CYC)...
November 2017: Lupus
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