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https://www.readbyqxmd.com/read/28638691/catastrophic-antiphospholipid-syndrome-treated-with-rituximab-a-case-report
#1
Atalay Doğru, Yunus Ugan, Mehmet Şahin, Nermin Karahan, Şevket Ercan Tunç
Catastrophic antiphospholipid syndrome (CAPS) is a rare and fatal condition that is characterized by diffuse venous and/or arterial thromboembolism within a short period of time and histopathological confirmation of small-vessel occlusion in at least one organ or tissue in the presence of positive antiphospholipid antibodies. Here we report the case of a 19-year-old woman with CAPS. During the first week of her hospitalization, she was diagnosed with CAPS on the basis of skin necrosis, pulmonary artery thrombosis, cerebral venous sinus thrombosis, and positive lupus anticoagulant...
June 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28503285/oral-contraceptive-related-transverse-sinus-thrombosis-as-an-initial-manifestation-of-antiphospholipid-syndrome-in-the-absence-of-systemic-lupus-erythematosus
#2
Payam Saadat, Reza Mohseni-Ahangar
BACKGROUND: Cerebral venous sinus thrombosis is a rare and potentially life-threatening neurologic manifestation of antiphospholipid syndrome. Oral contraceptive pills (OCP) may increase the risk of vascular events, even in people without family history of venous thrombosis. CASE PRESENTATION: A 31-year-old woman with four weeks of constant headache and history of taking OCP for one year has been selected for this study. The results of magnetic resonance imaging (MRI) of brain and venography confirmed a diagnosis of cerebral venous sinus thrombosis...
2017: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/28491814/varicella-zoster-virus-vzv-multifocal-vasculopathy-in-a-patient-with-systemic-lupus-erythematosus-a-diagnostic-and-treatment-dilemma
#3
Stephen Osiro, Nadim Salomon
Cerebral vasculopathy due to varicella-zoster virus (VZV) infection is well-documented. We report a fatal case of VZV multifocal vasculopathy in a patient with systemic lupus erythematosus (SLE) who presented with subacute changes in mental status and had multiple areas of hemorrhagic infarcts on brain imaging. However, the correct diagnosis was delayed by several confounding factors including the absence of zoster rash, normal cerebral angiography, persistently low cerebrospinal fluid (CSF) glucose and negative initial polymerase chain reaction (PCR) for VZV DNA in the CSF...
2017: IDCases
https://www.readbyqxmd.com/read/28479183/numerous-fusiform-and-saccular-cerebral-aneurysms-in-central-nervous-system-lupus-presenting-with-ischemic-stroke
#4
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/28467290/factors-associated-with-increased-white-matter-hyperintense-lesion-wmhi-load-in-patients-with-systemic-lupus-erythematosus-sle
#5
S S Shaharir, S S Osman, S A Md Rani, R Sakthiswary, M S M Said
Introduction White matter hyperintense (WMHI) lesions are the most common finding in magnetic resonance imaging (MRI) of the brain in patients with systemic lupus erythematosus (SLE). Objective The objective of this article is to determine the clinical factors associated with an increase in WMHI lesion load among SLE patients. Method A total of 83 SLE patients with MRI of the brain from National University of Malaysia Medical Centre were included. The WMHI lesion load was determined using the Scheltens score and Fazekas scale, and their distribution was divided into the deep white matter (DWMHI) and periventricular (PVH) regions...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28457083/intravenous-immunoglobulin-for-pediatric-neuropsychiatric-lupus-triggered-by-epstein-barr-virus-cerebral-infection
#6
Claudia Brogna, Raffaele Manna, Ilaria Contaldo, Domenico M Romeo, Maria Chiara Stefanini, Antonio Chiaretti, Eugenio Mercuri, Paolo Mariotti
No abstract text is available yet for this article.
December 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28428121/the-blood-brain-barrier-and-neuropsychiatric-lupus-new-perspectives-in-light-of-advances-in-understanding-the-neuroimmune-interface
#7
REVIEW
Ariel D Stock, Sivan Gelb, Ofer Pasternak, Ayal Ben-Zvi, Chaim Putterman
Experts have previously postulated a linkage between lupus associated vascular pathology and abnormal brain barriers in the immunopathogenesis of neuropsychiatric lupus. Nevertheless, there are some discrepancies between the experimental evidence, or its interpretation, and the working hypotheses prevalent in this field; specifically, that a primary contributor to neuropsychiatric disease in lupus is permeabilization of the blood brain barrier. In this commonly held view, any contribution of the other known brain barriers, including the blood-cerebrospinal fluid and meningeal barriers, is mostly excluded from the discussion...
June 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28420047/organ-damage-accrual-and-distribution-in-systemic-lupus-erythematosus-patients-followed-up-for-more-than-10-years
#8
M Taraborelli, I Cavazzana, N Martinazzi, M Grazia Lazzaroni, M Fredi, L Andreoli, F Franceschini, A Tincani
Objective The aim of this study was to determine the prevalence, predictors and progression of organ damage in a monocentric cohort of systemic lupus erythematosus patients with a long follow-up. Organ damage was assessed by the Systemic Lupus International Collaborating Clinics Damage Index one year after diagnosis and every five years. Disease activity was measured by the systemic lupus erythematosus disease activity index (SLEDAI)-2K at the beginning of the follow-up. Univariate and multivariable analyses were used to detect items associated with damage...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28394228/electrophysiological-dysfunction-induced-by-anti-ribosomal-p-protein-antibodies-injection-into-the-lateral-ventricle-of-the-rat-brain
#9
N Gaburo, J Freire de Carvalho, C Timo-Iaria, C Bueno, M Reichlin, V S T Viana, E Bonfá
Objective Anti-ribosomal P antibodies (anti-P) are strongly associated with neuropsychiatric lupus. This study was designed to determine whether these antibodies are capable of causing electro-oscillogram (EOSG) and behavior alterations in rats. Methods IgG fraction anti-P positive and affinity-purified anti-P antibodies were injected intraventricularly in rats. Sequential cortical and subcortical EOSGs were analyzed during 30 days. IgG anti-Ro/SS-A and normal IgG were used as controls. Results All 13 animals injected with IgG anti-P demonstrated a high prevalence of polyspikes, diffusely distributed in hippocampal fields and cerebral cortex...
April 2017: Lupus
https://www.readbyqxmd.com/read/28362518/role-of-angiography-in-systemic-lupus-erythematosus-induced-choroiditis
#10
Kenneth Rohan Lee, Lai Yin Peng, Tajunisah Begum Iqbal, Visvaraja Subrayan
PURPOSE: To report a case of systemic lupus erythematosus-induced choroidal vasculitis. METHODS: A 34-year-old woman with a long-standing history of systemic lupus erythematosus had a sudden painless loss of vision in the right eye over 12 hours. Ocular examination revealed a visual acuity of counting fingers of 1 foot on the right eye and 20/20 on the left. There was a relative afferent pupillary defect on the right side with a pink, distinct optic disk margin...
March 31, 2017: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/28325644/dnase-active-trex1-frame-shift-mutants-induce-serologic-autoimmunity-in-mice
#11
Tomomi Sakai, Takuya Miyazaki, Dong-Mi Shin, Yong-Soo Kim, Chen-Feng Qi, Robert Fariss, Jeeva Munasinghe, Hongsheng Wang, Alexander L Kovalchuk, Parul H Kothari, Charles S Fermaintt, John P Atkinson, Fred W Perrino, Nan Yan, Herbert C Morse
TREX1/DNASE III, the most abundant 3'-5' DNA exonuclease in mammalian cells, is tail-anchored on the endoplasmic reticulum (ER). Mutations at the N-terminus affecting TREX1 DNase activity are associated with autoimmune and inflammatory conditions such as Aicardi-Goutières syndrome (AGS). Mutations in the C-terminus of TREX1 cause loss of localization to the ER and dysregulation of oligosaccharyltransferase (OST) activity, and are associated with retinal vasculopathy with cerebral leukodystrophy (RVCL) and in some cases with systemic lupus erythematosus (SLE)...
July 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28289923/tocilizumab-reverses-cerebral-vasculopathy-in-a-patient-with-homozygous-samhd1-mutation
#12
REVIEW
Michael Henrickson, Heng Wang
An auto-inflammatory syndrome consequent to SAMHD1 mutations involves cerebral vasculopathy characterized by multifocal stenosis and aneurysms within large arteries, moyamoya, chronic ischemia, and early-onset strokes (SAMS). While this condition involves the innate immune system, additional clinical features mimic systemic lupus erythematosus. Mutations in this gene can also cause a subset of the rare genetic condition Aicardi-Goutières syndrome. To date, no established therapy successfully prevents disease progression...
June 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28266034/elevated-serum-mfg-e8-level-is-possibly-associated-with-the-presence-of-high-intensity-cerebral-lesions-on-magnetic-resonance-imaging-in-patients-with-systemic-lupus-erythematosus
#13
Chikako Kishi, Sei-Ichiro Motegi, Osamu Ishikawa
Human milk fat globule-EGF factor 8 (MFG-E8), also known as lactadherin, is a secreted glycoprotein that plays essential roles in the clearance of apoptotic cells and angiogenesis. It has been reported that serum MFG-E8 levels are higher in systemic lupus erythematosus (SLE) patients compared with in healthy controls; however, a previous study reported no correlation between serum MFG-E8 levels and SLE disease activity. The objective of this study was to assess serum MFG-E8 levels and their clinical associations in patients with SLE...
March 7, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28258054/thrombophilia-risk-is-not-increased-in-children-after-perinatal-stroke
#14
Colleen Curtis, Aleksandra Mineyko, Patricia Massicotte, Michael Leaker, Xiu Yan Jiang, Amalia Floer, Adam Kirton
Perinatal stroke causes cerebral palsy and lifelong disability. Specific diseases are definable but mechanisms are poorly understood. Evidence suggests possible associations between arterial perinatal stroke and prothrombotic disorders but population-based, controlled, disease-specific studies are limited. Understanding thrombophilia in perinatal stroke informs pathogenesis models and clinical management. We conducted a population-based, prospective, case-control study to determine the association of specific perinatal stroke diseases with known thrombophilias...
March 3, 2017: Blood
https://www.readbyqxmd.com/read/28212920/neonatal-lupus-follow-up-in-infants-with-anti-ssa-ro-antibodies-and-review-of-the-literature
#15
REVIEW
Antonio Alberto Zuppa, Riccardo Riccardi, Simonetta Frezza, Francesca Gallini, Rita Maria Paola Luciano, Giovanni Alighieri, Costantino Romagnoli, Sara De Carolis
Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur...
April 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28195100/a-54-year-old-male-with-rapidly-progressive-neurologic-syndrome-clinicopathologic-correlation-of-a-rare-diagnosis
#16
Deepti Mutreja, Nikhil Moorchung, Salil Gupta, Rajeev Saxena, Rohini S Doshetty, Bhaskar Nandi
Diagnosis of systemic lupus erythematosus (SLE) as primary presentation with central nervous system involvement as a rapidly progressive neurologic syndrome is extremely rare. We present a rare case of a 54-year-old hypertensive male patient, who presented with a fulminant neurologic syndrome. He presented with cerebellar and meningeal signs, aseptic meningitis and had a rapid downhill course following admission. A postmortem revealed feature of systemic connective tissue fulfilling diagnostic criteria of SLE with lupus cerebritis...
January 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28179389/homonymous-hemianopia-in-the-primary-antiphospholipid-syndrome
#17
Derek Kwun-Hong Ho, Rishi Ramessur, Mradul Gupta, John P Mathews
A woman aged 26 years was referred by her GP to the eye casualty department with sudden-onset left homonymous hemianopia and right-sided headache. Full ophthalmic examination was normal with the exception of a left homonymous hemianopia confirmed with automated perimetry. Urgent CT imaging revealed a non-haemorrhagic cerebral infarct in the right parieto-occipital region. Subsequent blood tests confirmed a diagnosis of antiphospholipid syndrome with positivity in IgG anticardiolipin antibody, IgG anti-β2-GP1 antibody and the Lupus anticoagulant screen...
February 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28133515/recognition-of-transmembrane-protein-39a-as-a-tumor-specific-marker-in-brain-tumor
#18
Jisoo Park, Hyunji Lee, Quangdon Tran, Kisun Mun, Dohoon Kim, Youngeun Hong, So Hee Kwon, Derek Brazil, Jongsun Park, Seon-Hwan Kim
Transmembrane protein 39A (TMEM39A) belongs to the TMEM39 family. TMEM39A gene is a susceptibility locus for multiple sclerosis. In addition, TMEM39A seems to be implicated in systemic lupus erythematosus. However, any possible involvement of TMEM39A in cancer remains largely unknown. In the present report, we provide evidence that TMEM39A may play a role in brain tumors. Western blotting using an anti-TMEM39A antibody indicated that TMEM39A was overexpressed in glioblastoma cell lines, including U87-MG and U251-MG...
January 2017: Toxicological Research
https://www.readbyqxmd.com/read/28068928/newly-recognized-cerebral-infarctions-on-postmortem-imaging-a-report-of-three-cases-with-systemic-infectious-disease
#19
Sakon Noriki, Kazuyuki Kinoshita, Kunihiro Inai, Toyohiko Sakai, Hirohiko Kimura, Takahiro Yamauchi, Masayuki Iwano, Hironobu Naiki
BACKGROUND: Postmortem imaging (PMI) refers to the imaging of cadavers by computed tomography (CT) and/or magnetic resonance imaging (MRI). Three cases of cerebral infarctions that were not found during life but were newly recognized on PMI and were associated with severe systemic infections are presented. CASE PRESENTATIONS: An 81-year-old woman with a pacemaker and slightly impaired liver function presented with fever. Imaging suggested interstitial pneumonia and an iliopsoas abscess, and blood tests showed liver dysfunction and disseminated intravascular coagulation (DIC)...
January 10, 2017: BMC Medical Imaging
https://www.readbyqxmd.com/read/28065614/cerebral-venous-sinus-thrombosis-presenting-feature-of-systemic-lupus-erythematosus
#20
Rajesh Kumar Singh, Sanjeev Kumar Bhoi, Jayantee Kalita, Usha Kant Misra
BACKGROUND: CVST (cerebral venous sinus thrombosis) may sometimes be associated with autoimmune disorders that require specific treatment. The clinical and magnetic resonance imaging (MRI) findings of systemic lupus erythematosus (SLE) patients with CVST are presented and contrasted with CVST without SLE. METHODS: Consecutive patients with CVST admitted in neurology service during 2012-2016 were included. The diagnosis of CVST was confirmed by MR venography or digital subtraction angiography...
March 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
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