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https://www.readbyqxmd.com/read/28057714/elevated-il-37-il-18-and-il-18bp-serum-concentrations-in-patients-with-primary-sj%C3%A3-gren-s-syndrome
#1
Wang Liuqing, Xia Liping, Shen Hui, Lu Jing
The objectives of this study were to examine the serum levels of interleukin (IL)-37 and its clinical association in patients with primary Sjögren's syndrome (pSS) and to investigate whether or not IL-37 participates in the regulation of the pathogenesis of pSS. ELISA was used to analyse the serum levels of IL-37, total IL-18 and IL-18 binding protein (IL-18BP). The level of free IL-18 was calculated based on the mass action law. The correlations between the IL-37 serum levels with the laboratory values and the total IL-18 and IL-18BP serum levels were analyzed by a Spearman's correlation test...
January 5, 2017: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
https://www.readbyqxmd.com/read/28051234/neonatal-lupus-erythematosus-with-congenital-heart-block-in-twins
#2
Lamia Gargouri, Faiza Safi, Bayen Maalej, Souad Mallek, Fatma Turki, Imen Majdoub, Malek Akrout, Dorra Abid, Samir Kamoun, Abdelmajid Mahfoudh
Background - Neonatal lupus erythematosus is an uncommon acquired autoimmune disease caused by transplacental passage of maternal antibodies SSA/Ro, SSB/La or U1 ribonucleoproteins. The most common clinical manifestations are skin rash, cardiac lesions, thrombocytopenia, anemia and hepatosplenomegaly. Complete congenital heart block is usually irreversible needing a pacemaker implantation in two-thirds of cases. Cases report - We report neonatal lupus erythematosus with complete congenital heart block in twins...
July 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/28049394/coexistence-of-amyloidosis-and-primary-sj%C3%A3-gren-s-syndrome-an-overview-amyloidosis-and-sj%C3%A3-gren-s-syndrome
#3
Gabriela Hernandez-Molina, David Faz-Muñoz, Miguel Astudillo-Angel, Alejandra Iturralde-Chávez, Edgardo Reyes
BACKGROUND: The association of primary Sjögren´s syndrome (SS) and amyloidosis has been recognized but scarcely assessed. OBJECTIVE: Herein we analyzed the literature regarding this association in order to describe a SS phenotype prone to both conditions. METHODS: PubMed and CINAHL databases were searched from inception until April 2016 for the key words: amyloidosis, amyloid, Sjögren's syndrome and their combinations. RESULTS: Most of the cases corresponded to localized amyloidosis mainly at skin and lung, whereas only three cases were systemic...
January 3, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/27988434/anti-ro-ssa-and-anti-la-ssb-antibodies-association-with-mild-lupus-manifestations-in-645-childhood-onset-systemic-lupus-erythematosus
#4
REVIEW
Glaucia V Novak, Mariana Marques, Verena Balbi, Natali W S Gormezano, Kátia Kozu, Ana P Sakamoto, Rosa M R Pereira, Maria T Terreri, Claudia S Magalhães, Andressa Guariento, Adriana M E Sallum, Roberto Marini, Virginia Paes Leme Ferriani, Cássia Maria Barbosa, Tânia Caroline Monteiro de Castro, Valéria C Ramos, Eloisa Bonfá, Clovis A Silva
BACKGROUND: To our knowledge there are no studies assessing anti-Ro/SSA and anti-La/SSB autoantibodies in a large population of childhood-systemic lupus erythematosus(cSLE) patients. METHODS: This was a retrospective multicenter cohort study performed in 10 Pediatric Rheumatology services, São Paulo state, Brazil. Anti-Ro/SSA and anti-La/SSB antibodies were measured by enzyme linked immunosorbent assay(ELISA) in 645 cSLE patients. RESULTS: Anti-Ro/SSA and anti-La/SSB antibodies were evidenced in 209/645(32%) and 102/645(16%) of cSLE patients, respectively...
December 14, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27988051/the-clinical-characteristics-of-aqp4-antibody-positive-nmo-sd-in-a-large-cohort-of-chinese-han-patients
#5
Jingzi ZhangBao, Lei Zhou, Xiaoyang Li, Tongjia Cai, Jiahong Lu, Chuanzhen Lu, Chongbo Zhao, Chao Quan
We aim to summarize the clinical features of AQP4-ab-positive NMO/SD in a large Chinese Han cohort. The clinical data of 145 AQP4-ab-seropositive patients was retrospectively reviewed. 55.9% (81/145) of the patients were defined as NMO while 39.3% (57/145) were defined as NMOSD according to the criteria established in 2006 and 2007. The mean onset age was 34.4years and the female to male ratio was 8.7:1. The median disease duration was 57months. The median of "time to second attack" and "time to develop NMO" was 7 and 13months respectively...
January 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/27987517/-cerebral-infarction-in-a-patient-with-primary-sjogren-s-syndrome-a-case-report-and-literature-review
#6
D Yang, L Qiao, L D Zhao
Sjogren's syndrome (SS) is a chronic autoimmune disorder characterized by lymphocytes infiltration in the exocrine glands. Central nervous system complications of primary SS are not rare, but ischemic stroke has been rarely reported. Here we report a 43-year-old female with a two-year history of primary SS, presenting with sudden cerebral infarction. Her primary SS was diagnosed on the basis of clinical features, high levels of serum anti-SSA and anti-SSB antibodies, salivary gland secretion evaluation and positive sublingual gland biopsy results...
December 18, 2016: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/27977016/heart-sounds-at-home-feasibility-of-an-ambulatory-fetal-heart-rhythm-surveillance-program-for-anti-ssa-positive-pregnancies
#7
B F Cuneo, A J Moon-Grady, S-E Sonesson, S Levasseur, L Hornberger, M T Donofrio, A Krishnan, A Szwast, L Howley, D W Benson, E Jaeggi
OBJECTIVE: Fetuses exposed to anti-SSA (Sjögren's) antibodies are at risk of developing irreversible complete atrioventricular block (CAVB), resulting in death or permanent cardiac pacing. Anti-inflammatory treatment during the transition period from normal heart rhythm (fetal heart rhythm (FHR)) to CAVB (emergent CAVB) can restore sinus rhythm, but detection of emergent CAVB is challenging, because it can develop in ⩽24 h. We tested the feasibility of a new technique that relies on home FHR monitoring by the mother, to surveil for emergent CAVB...
December 15, 2016: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/27940588/a-multicentre-study-of-95-biopsy-proven-cases-of-renal-disease-in-primary-sj%C3%A3-gren-s-syndrome
#8
Magali Jasiek, Alexandre Karras, Véronique Le Guern, Evguenia Krastinova, Rafik Mesbah, Stanislas Faguer, Noémie Jourde-Chiche, Anne-Laure Fauchais, Laurent Chiche, Emmanuelle Dernis, Guillaume Moulis, Jean-Baptiste Fraison, Estibaliz Lazaro, Perrine Jullien, Eric Hachulla, Alain Le Quellec, Philippe Rémy, Aurélie Hummel, Nathalie Costedoat-Chalumeau, Pierre Ronco, Philippe Vanhille, Vannary Meas-Yedid, Carole Cordonnier, Sophie Ferlicot, Laurent Daniel, Raphaele Seror, Xavier Mariette, Eric Thervet, Hélène François, Benjamin Terrier
OBJECTIVE: Renal involvement is a rare event during primary SS (pSS). We aimed to describe the clinico-biological and histopathological characteristics of pSS-related nephropathy and its response to treatment. METHODS: We conducted a French nationwide, retrospective, multicentre study including pSS patients fulfilling American-European Consensus Group criteria or enlarged American-European Consensus Group criteria, and with biopsy-proven renal involvement. RESULTS: A total of 95 patients were included (median age 49 years)...
December 10, 2016: Rheumatology
https://www.readbyqxmd.com/read/27939798/diplopia-and-sjogren-s-disease-a-rare-case-report
#9
Kaushalendra Tripathi, Richa Tripathi, Navid Seraji-Bozorgzad
Sjogren's syndrome is a chronic autoimmune disorder which affects the exocrine glands with lymphocytic infiltration, and occasionally involves central nervous system. It is usually rare and manifests as a lesion in the trigeminal nerve. Our case discusses the involvement of the oculomotor and abducens nerves along with the prevalence of such cases as seen on literature review. We describe a case of a middle aged woman who presented with ophthalmoplegic symptoms. The symptoms resolved in response to steroid therapy and serum analysis was positive for anti SSA antibodies...
January 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#10
C Bason, I Pagnini, A Brucato, S Maestroni, A Puccetti, C Lunardi, R Cimaz
Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27900999/evaluating-anti-smd1-amino-acid-83-119-peptide-reactivity-in-children-with-systemic-lupus-erythematosus-and-other-immunological-diseases
#11
Hai-Ou Yang, Xiao-Qing Zhang, Qi-Hua Fu
BACKGROUND: SmD1-amino-acid 83-119 peptide (SmD183-119) is the major epitope of Smith (Sm) antigen, which is specific for adult systemic lupus erythematosus (SLE). The anti-SmD183-119 antibody has exhibited higher sensitivity and specificity than anti-Sm antibody in diagnosing adult SLE. However, the utility of anti-SmD183-119antibodies remains unclear in children with SLE (cSLE). This study aimed to assess the characteristics of anti-SmD183-119antibody in the diagnosis of cSLE. METHODS: Samples from 242 children with different rheumatological and immunological disorders, including autoimmune diseases (SLE [n = 46] and ankylosing spondylitis [AS, n = 11]), nonautoimmune diseases (Henoch-Schonlein purpura [HSP, n = 60], idiopathic thrombocytopenia purpura [n = 27], hematuria [n = 59], and arthralgia [n = 39]) were collected from Shanghai Children's Medical Center from March 6, 2012 to February 27, 2014...
2016: Chinese Medical Journal
https://www.readbyqxmd.com/read/27893665/is-it-relevant-to-screen-young-women-hospitalized-in-psychiatric-department-for-neuropsychiatric-systemic-lupus-erythematosus-npsle-a-prospective-study-of-100-psychiatric-inpatients
#12
Alexandra Audemard-Verger, Elizabeth Comby, Clément Nathou, Audrey Sultan, Mathieu Frémont, Aurélie Baldolli, Louis Simon Trumier, Vincent Marzloff, Brigitte Le Mauff, Jennifer Manuzak, Sonia Dollfus, Boris Bienvenu
On the basis that diagnosis of neuropsychiatric systemic lupus erythematosus (NPSLE) is sometimes difficult and systemic lupus erythematosus (SLE) can present with isolated psychiatric symptoms, we initiated a survey in a psychiatric department to screen for NPSLE in young female inpatients.We prospectively studied consecutive young female patients referred to the department of psychiatry. Antinuclear antibodies (ANA), anti-deoxyribonucleic acid (DNA), and antiextractable soluble nuclear antigens (ENA) in the serum of patients were screened...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27864697/characteristics-of-primary-sj%C3%A3-gren-s-syndrome-patients-with-igg4-positive-plasma-cells-infiltration-in-the-labial-salivary-glands
#13
Chang Liu, Huayong Zhang, Genhong Yao, Yunxia Hu, Jingjing Qi, Yan Wang, Weiwei Chen, Xiaojun Tang, Wenchao Li, Liwei Lu, Luo Gu, Lingyun Sun
The purpose of this study was to investigate the characteristics of primary Sjögren's syndrome (pSS) patients with IgG4 positive (IgG4(+)) plasma cell infiltration in labial salivary glands (LSGs). Paraffin sections of LSGs from 336 pSS patients were stained with IgG4 and IgG monoclonal antibodies. According to the infiltration of IgG4(+) plasma cells, patients were divided and clinical and serological characteristics were analyzed and compared. Based on the infiltration of IgG4(+) plasma cells in the LSGs, patients were divided into three subgroups, low IgG4, moderate IgG4, and high IgG4 groups...
November 18, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27862026/unusual-presentation-of-sj%C3%A3-gren-associated-neuropathy-with-plasma-cell-rich-infiltrate
#14
Elie Naddaf, Sarah E Berini, P James B Dyck, Ruple S Laughlin
INTRODUCTION: Sjögren syndrome is thought to be a lymphocyte-driven process. Peripheral nervous system involvement occurs in about 20-25% of patients. A sensory predominant, large fiber peripheral neuropathy is most common, and it is usually associated with a subacute to chronic presentation. METHODS: We report a rare case of an acute Sjögren-associated, sensory predominant, length-dependent peripheral neuropathy mimicking Guillain-Barré syndrome. The patient presented with sensory ataxia preceded by fever and polyarthralgia...
November 16, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27833976/histopathological-and-clinical-traps-in-lichen-sclerosus-a-case-report
#15
Daciana Elena Brănişteanu, Daniel Constantin Brănişteanu, Gabriela Stoleriu, Dan Ferariu, Cătălina Maria Voicu, Loredana Elena Stoica, Constantin Căruntu, Daniel Boda, Florina Mihaela Filip-Ciubotaru, Andreea Dimitriu, Cezar Doru Radu
Lichen sclerosus et atrophicus and limited systemic scleroderma (acrosclerosis) are inflammatory skin diseases that ultimately evolve into two distinct modes of atrophic scar formation, but which can easily be confused clinically. They are very rarely associated. The literature has reported cases in which lichen sclerosus was associated with various forms of scleroderma, but often with localized morphea. The characteristic histopathological picture of lichen sclerosus includes a thin epidermis, with orthohyperkeratosis and vascular degeneration in the basal layer, loss of elastic fibers, and band-like inflammatory infiltrate in the papillary dermis, while systemic sclerosis is characterized by excessive deposition of collagen in the dermis, accompanied by reduction in adnexal structures and their entrapment in collagen, and the presence of perivascular lymphocytic inflammatory infiltrate...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/27803420/acquired-gitelman-syndrome-in-an-anti-ssa-antibody-positive-patient-with-a-slc12a3-heterozygous-mutation
#16
Takeshi Kusuda, Tadashi Hosoya, Takayasu Mori, Katsuhito Ihara, Hidenori Nishida, Motoko Chiga, Eisei Sohara, Tatemitsu Rai, Ryuji Koike, Shinichi Uchida, Hitoshi Kohsaka
A 36-year-old woman developed hypokalemic metabolic alkalosis after anti SS-A antibody was found to be positive. Diuretic loading test results were compatible with Gitelman syndrome (GS). The patient had a heterozygous mutation in SLC12A3, which encodes for thiazide-sensitive NaCl cotransporter (NCCT). While the mutation may be responsible for a latent hypofunction of NCCTs, the underlying anti-SSA antibody-associated autoimmunity induced the manifestation of its hypofunction. To the best of our knowledge, this is the first report to demonstrate that anti SS-A antibody-associated autoimmunity may induce GS in a patient with a SLC12A3 heterozygous mutation...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27789466/2016-american-college-of-rheumatology-european-league-against-rheumatism-classification-criteria-for-primary-sj%C3%A3-gren-s-syndrome-a-consensus-and-data-driven-methodology-involving-three-international-patient-cohorts
#17
REVIEW
Caroline H Shiboski, Stephen C Shiboski, Raphaèle Seror, Lindsey A Criswell, Marc Labetoulle, Thomas M Lietman, Astrid Rasmussen, Hal Scofield, Claudio Vitali, Simon J Bowman, Xavier Mariette
OBJECTIVES: To develop and validate an international set of classification criteria for primary Sjögren's syndrome (SS) using guidelines from the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). These criteria were developed for use in individuals with signs and/or symptoms suggestive of SS. METHODS: We assigned preliminary importance weights to a consensus list of candidate criteria items, using multi-criteria decision analysis...
January 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27785888/2016-american-college-of-rheumatology-european-league-against-rheumatism-classification-criteria-for-primary-sj%C3%A3-gren-s-syndrome-a-consensus-and-data-driven-methodology-involving-three-international-patient-cohorts
#18
Caroline H Shiboski, Stephen C Shiboski, Raphaèle Seror, Lindsey A Criswell, Marc Labetoulle, Thomas M Lietman, Astrid Rasmussen, Hal Scofield, Claudio Vitali, Simon J Bowman, Xavier Mariette
OBJECTIVE: To develop and validate an international set of classification criteria for primary Sjögren's syndrome (SS) using guidelines from the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). These criteria were developed for use in individuals with signs and/or symptoms suggestive of SS. METHODS: We assigned preliminary importance weights to a consensus list of candidate criteria items, using multi-criteria decision analysis...
January 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/27693175/esophageal-abnormalities-in-juvenile-localized-scleroderma-is-it-associated-with-other-extracutaneous-manifestations
#19
Clarissa C M Valões, Glaucia V Novak, Juliana B Brunelli, Katia T Kozu, Ricardo K Toma, Clovis A Silva
OBJECTIVE: To assess esophageal involvement (EI) in juvenile localized scleroderma (JLS) population and the possible association between this gastrointestinal manifestation and demographic data, clinical features, laboratory exams, treatments and outcomes. METHODS: For a period of 31 years, 5,881 patients with rheumatic diseases were followed in our Pediatric Rheumatology Division. EI was defined by the presence of symptoms (solid/liquid dysphagia, heartburn, esophageal regurgitation, nausea/vomiting and epigastralgia) and confirmed by at least one EI exam abnormality: barium contrast radiography, upper gastrointestinal endoscopy and 24-hour esophageal pH-monitoring...
September 17, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27692137/clinical-features-and-natural-history-of-interstitial-pneumonia-with-autoimmune-features-a-single-center-experience
#20
Sandra Chartrand, Jeffrey J Swigris, Lina Stanchev, Joyce S Lee, Kevin K Brown, Aryeh Fischer
OBJECTIVE: To describe the clinical phenotype and natural history of a cohort of patients with interstitial pneumonia with autoimmune features (IPAF). METHODS: A retrospective, single center study of 56 patients with IPAF evaluated between February 2008 and August 2014. All clinical data were extracted from the electronic medical record and longitudinal changes in forced vital capacity (FVC) were analyzed with mixed-effects, piecewise linear regression models that considered time as a continuous factor...
October 2016: Respiratory Medicine
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