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https://www.readbyqxmd.com/read/28100575/atrophic-pityriasis-versicolor-occurring-in-a-patient-with-sj%C3%A3-gren-s-syndrome
#1
Elena Marinello, Stefano Piaserico, Mauro Alaibac
Pityriasis versicolor is one of the most frequent epidermal mycotic infections in the world, but its atrophic variant is rarely described. The aetiology of the atrophy is still unknown, and two main hypotheses have been formulated, one suggesting a correlation with long-term use of topical steroids and the other a delayed type hypersensitivity to epicutaneous antigens derived from components of the fungus. Atrophic pityriasis versicolor is a benign disease, but needs to be distinguished from other more severe skin diseases manifesting with cutaneous atrophy...
January 18, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28099084/mechanisms-and-new-strategies-for-primary-sj%C3%A3-gren-s-syndrome
#2
Clio P Mavragani
Primary Sjögren's syndrome (SS) is a common chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, mainly salivary and lacrimal, resulting in oral and ocular dryness, although virtually any organ system can be affected. SS-related systemic manifestations are classified as either related to the presence of periepithelial infiltrates in exocrine and parenchymal organs or resulting from immunocomplex deposition due to B cell hyperactivity with increased risk for B cell lymphoma development...
January 14, 2017: Annual Review of Medicine
https://www.readbyqxmd.com/read/28092973/elevated-ambulatory-systolic-diastolic-pressure-regression-index-is-genetically-determined-in-hypertensive-patients-with-coronary-heart-disease
#3
Marcin Wirtwein, Olle Melander, Marketa Sjőgren, Michal Hoffmann, Krzysztof Narkiewicz, Marcin Gruchala, Wojciech Sobiczewski
OBJECTIVES: Ambulatory systolic-diastolic pressure regression index (ASDPRI) as a composite marker of cardiovascular (CV) properties is related to CV complications. However, genetic determinants of ASDPRI are not known. The aim of this study is to report the relationship between certain single nucleotide polymorphisms (SNP) and ASDPRI in hypertensive patients with CAD confirmed by coronary angiography. METHODS: A total of 1345 hypertensive subjects with CAD were included...
January 16, 2017: Blood Pressure
https://www.readbyqxmd.com/read/28089983/detailed-analysis-of-the-articular-domain-in-patients-with-primary-sj%C3%A3-gren-syndrome
#4
Rada V Moerman, Suzanne Arends, Petra M Meiners, Arjan Vissink, Frederik K L Spijkervet, Frans G M Kroese, Elisabeth Brouwer, Hendrika Bootsma
OBJECTIVE: We used the 28-joint Disease Activity Score (DAS28) and the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (ESSDAI) articular domain to assess the effect of rituximab (RTX) and abatacept (ABA) on articular involvement in primary Sjögren syndrome (pSS). METHODS: Patients with pSS treated with RTX (n = 18) or ABA (n = 13) and having a DAS28 erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) level ≥ 3.2 at baseline were selected...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28089075/conjunctival-impression-cytology-evaluation-of-patients-with-dry-eye-disease-using-scleral-contact-lenses
#5
Sarah La Porta Weber, Rossen Mihaylov Hazarbassanov, Alex Nasaré, José Álvaro Pereira Gomes, Ana Luisa Hofling-Lima
PURPOSE: To evaluate conjunctival impression cytology and HLADR expression changes after wearing scleral contact lenses (ScCLs) for moderate to severe dry eye disease (DED). DESIGN: Prospective interventional case series. METHODS: Forty-one eyes from 25 patients with moderate to severe DED were evaluated for Esclera ScCL treatment. Best-corrected visual acuity (BCVA) and slit-lamp findings were assessed. Impression cytology specimens were obtained from DED patients at the baseline and after wearing ScCLs for 12 months...
January 6, 2017: Contact Lens & Anterior Eye: the Journal of the British Contact Lens Association
https://www.readbyqxmd.com/read/28077839/atypical-trigeminal-neuralgia-a-rare-neurological-manifestation-of-systemic-lupus-erythematosus
#6
Viki Kumar, Jaspinder Kaur, Pallavi Pothuri, Sahiba Bandagi
BACKGROUND Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder of unknown etiology which can present at any age with symptoms of mucocutaneous, musculoskeletal, renal, central nervous system, and nonspecific clinical pictures making the disease a "master of mimicry". CASE REPORT A 53-year-old female, who was recently diagnosed with SLE, presented with right-sided sharp and electric shock-like facial pain starting at the side of her right nostril and traveling down the naso-labial fold and then back to the angle of the jaw, mostly in the region of V2-V3 distribution with no radiation beyond trigeminal distribution...
January 12, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28076899/genome-wide-association-analysis-reveals-genetic-heterogeneity-of-sj%C3%A3-gren-s-syndrome-according-to-ancestry
#7
Kimberly E Taylor, Quenna Wong, David M Levine, Caitlin McHugh, Cathy Laurie, Kimberly Doheny, Mi Y Lam, Alan N Baer, Stephen Challacombe, Hector Lanfranchi, Morten Schiødt, M Srinivasan, Hisanori Umehara, Frederick B Vivino, Yan Zhao, Stephen Shiboski, Troy E Daniels, John S Greenspan, Caroline H Shiboski, Lindsey A Criswell
OBJECTIVE: Sjögren's Syndrome (SS) is a systemic autoimmune disease affecting primarily the lacrimal and salivary glands. The Sjögren's International Collaborative Clinical Alliance (SICCA) is an international multisite observational study whose participants have been genotyped on the Omni 2.5M platform and undergone deep phenotyping using common protocol-directed methods, providing a unique opportunity to examine the genetic etiology of SS across ancestry and disease subsets. METHODS: We perform GWAS analyses utilizing dbGaP controls on all subjects (1405 cases, 1622 SICCA controls, 3125 external controls), European (similarly 585, 966, 2580), and Asian (similarly 460, 224, 901) with ancestry adjustments via principal component analyses...
January 11, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28073684/are-the-women-with-sj%C3%A3-gren-s-syndrome-satisfied-with-their-sexual-activity
#8
Hatice Isik, Metin Isik, Oner Aynioglu, Deniz Karcaaltincaba, Ahmet Sahbaz, Tugba Beyazcicek, Mehmet Ibrahim Harma, Nejat Demircan
OBJECTIVE: Females with Sjögren's Syndrome (SS) often experience vaginal dryness and dyspareunia, along with glandular and extraglandular symptoms. We aimed to evaluate sexual function and life quality in women with SS. METHODS: Forty-six premenopausal women with SS and forty-seven age-matched controls were studied. Age, duration of the disease, medications, and comorbid diseases were noted. Participants completed 36-Item Short Form Health Survey (SF-36) and Female Sexual Function Index (FSFI)...
December 27, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28067896/pathogenic-role-of-endogenous-tnf-%C3%AE-in-the-development-of-sj%C3%A3-gren-s-like-sialadenitis-and-secretory-dysfunction-in-non-obese-diabetic-mice
#9
Jing Zhou, Toshihisa Kawai, Qing Yu
Patients with Sjögren's syndrome (SS), an autoimmune disease primarily affecting exocrine glands, exhibit enhanced TNF-α expression in the saliva and salivary glands. However, the precise in vivo role of TNF-α during the initiation and development of SS is not clearly defined. The present study is undertaken to determine the function of endogenously produced TNF-α in the pathogenesis of SS in non-obese diabetic (NOD) mice, a model of this human disease. Administration of a neutralizing anti-TNF-α antibody to female NOD mice during the stage prior to disease onset significantly improved salivary secretion, indicating a remission of clinical symptoms of SS...
January 9, 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/28060066/assessment-of-the-tear-meniscus-by-strip-meniscometry-and-keratograph-in-patients-with-dry-eye-disease-according-to-the-presence-of-meibomian-gland-dysfunction
#10
Kang Won Lee, Jung Yong Kim, Hee Seung Chin, Kyoung Yul Seo, Tae-Im Kim, Ji Won Jung
PURPOSE: We aimed to compare the tear meniscus measurements using strip meniscometry (SM) and Keratograph5M (K5M) between 3 subtypes of dry eye disease patient groups, classified according to the presence of meibomian gland dysfunction (MGD), and normal subjects. METHODS: We enrolled 145 eyes from 145 subjects and measured the tear meniscus using SM and K5M, tear film breakup time, ocular surface staining, and ocular surface disease index; the Schirmer test; and examined lid margins and meibomian glands...
February 2017: Cornea
https://www.readbyqxmd.com/read/28060064/automated-measurement-of-tear-film-dynamics-and-lipid-layer-thickness-for-assessment-of-non-sj%C3%A3-gren-dry-eye-syndrome-with-meibomian-gland-dysfunction
#11
Yong Woo Ji, Jeihoon Lee, Hun Lee, Kyoung Yul Seo, Eung Kweon Kim, Tae-Im Kim
PURPOSE: To investigate automated values from an advanced corneal topographer with a built-in real keratometer, color camera, and ocular surface interferometer for the evaluation of non-Sjögren dry eye syndrome (NSDES) with meibomian gland dysfunction (MGD). METHODS: Sixty-four patients (64 eyes) diagnosed with NSDES with MGD were enrolled. All eyes were evaluated using the Ocular Surface Disease Index (OSDI), fluorescence staining score, tear film breakup time (TBUT), Schirmer test, and MGD grade...
February 2017: Cornea
https://www.readbyqxmd.com/read/28053878/microparticle-subpopulations-are-potential-markers-of-disease-progression-and-vascular-dysfunction-across-a-spectrum-of-connective-tissue-disease
#12
E M McCarthy, D Moreno-Martinez, F L Wilkinson, N J McHugh, I N Bruce, J D Pauling, M Y Alexander, B Parker
OBJECTIVE: Microparticles (MPs) are membrane-bound vesicles derived from vascular and intravascular cells such as endothelial cells (EMPs) and platelets (PMPs). We investigated EMP and PMP numbers across a spectrum of autoimmune rheumatic diseases (AIRDs) with the aim of comparing the levels of, and relationship between, EMPs and PMPs. METHODS: Patients with Systemic Lupus Erythematosus (SLE) (n = 24), Systemic Sclerosis (SSc) (n = 24), Primary Raynauds Phenomenon (RP) (n = 17) and "other CTD" (n = 15) (Primary Sjogrens Syndrome, UCTD or MCTD) as well as 15 healthy controls were recruited...
June 2017: BBA Clinical
https://www.readbyqxmd.com/read/28052128/gba2-mutations-cause-a-marinesco-sj%C3%A3-gren-like-syndrome-genetic-and-biochemical-studies
#13
Kristoffer Haugarvoll, Stefan Johansson, Carlos E Rodriguez, Helge Boman, Bjørn Ivar Haukanes, Ove Bruland, Francisco Roque, Inge Jonassen, Maria Blomqvist, Wenche Telstad, Jan-Eric Månsson, Per Morten Knappskog, Laurence A Bindoff
BACKGROUND: With the advent new sequencing technologies, we now have the tools to understand the phenotypic diversity and the common occurrence of phenocopies. We used these techniques to investigate two Norwegian families with an autosomal recessive cerebellar ataxia with cataracts and mental retardation. METHODS AND RESULTS: Single nucleotide polymorphism (SNP) chip analysis followed by Exome sequencing identified a 2 bp homozygous deletion in GBA2 in both families, c...
2017: PloS One
https://www.readbyqxmd.com/read/28050735/semi-quantitative-analysis-of-salivary-gland-scintigraphy-in-sj%C3%A3-gren-s-syndrome-diagnosis-a-first-line-tool
#14
Tiziana Angusti, Emanuela Pilati, Antonella Parente, Renato Carignola, Matteo Manfredi, Simona Cauda, Elena Pizzigati, Julien Dubreuil, Francesco Giammarile, Valerio Podio, Andrea Skanjeti
OBJECTIVE: The aim of this study was the assessment of semi-quantified salivary gland dynamic scintigraphy (SGdS) parameters independently and in an integrated way in order to predict primary Sjögren's syndrome (pSS). MATERIALS AND METHODS: Forty-six consecutive patients (41 females; age 61 ± 11 years) with sicca syndrome were studied by SGdS after injection of 200 MBq of pertechnetate. In sixteen patients, pSS was diagnosed, according to American-European Consensus Group criteria (AECGc)...
January 3, 2017: Clinical Oral Investigations
https://www.readbyqxmd.com/read/28043400/dental-issues-in-lacrimo-auriculo-dento-digital-syndrome-an-autosomal-dominant-condition-with-clinical-and-genetic-variability
#15
M J Hajianpour, Hannah Bombei, Scott M Lieberman, Rachael Revell, Rachana Krishna, Robert Gregorsok, Simon Kao, Jeff M Milunsky
BACKGROUND AND OVERVIEW: Lacrimo-auriculo-dento-digital (LADD) syndrome is an autosomal dominant disorder with variable lacrimal and salivary gland hypoplasia and aplasia, auricular anomalies and hearing loss, dental defects and caries, and digital anomalies. CASE DESCRIPTION: The authors present the cases of 2 unrelated children with enamel defects and history of dry mouth leading to recurrent dental caries. The referring diagnoses were Sjögren disease and hypohidrotic ectodermal dysplasia, respectively...
December 30, 2016: Journal of the American Dental Association
https://www.readbyqxmd.com/read/28042126/b-cell-depletion-therapy-normalizes-circulating-follicular-th-cells-in-primary-sj%C3%A3-gren-syndrome
#16
Gwenny M Verstappen, Frans G M Kroese, Petra M Meiners, Odilia B Corneth, Minke G Huitema, Erlin A Haacke, Bert van der Vegt, Suzanne Arends, Arjan Vissink, Hendrika Bootsma, Wayel H Abdulahad
OBJECTIVE: To assess the effect of B cell depletion therapy on effector CD4+ T cell homeostasis and its relation to objective measures of disease activity in patients with primary Sjögren syndrome (pSS). METHODS: Twenty-four patients with pSS treated with rituximab (RTX) and 24 healthy controls (HC) were included. Frequencies of circulating effector CD4+ T cell subsets were examined by flow cytometry at baseline and 16, 24, 36, and 48 weeks after the first RTX infusion...
January 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28033649/thrombospondin-derived-peptide-attenuates-sj%C3%A3-gren-s-syndrome-associated-ocular-surface-inflammation-in-mice
#17
Laura Contreras-Ruiz, Fayaz A Mir, Bruce Turpie, Sharmila Masli
Sjögren's syndrome is a second most common rheumatic disease in which autoimmune response targets exocrine glands (salivary and lacrimal glands) resulting in clinical symptoms of dry mouth and dry eye. Inflammation of the lacrimal gland induces tear abnormalities that contribute to the inflammation of the ocular surface, which includes ocular mucosa. Thrombospondin-1 (TSP-1) plays a critical regulatory role in the ocular mucosa and as such TSP-1-/- mice spontaneously develop chronic ocular surface inflammation associated with Sjögren's syndrome (SS)...
December 29, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28032845/physical-fatigue-characterises-patient-experience-of-primary-sj%C3%A3-gren-s-syndrome
#18
Suzanne Arends, Petra M Meiners, Rada V Moerman, Frans G M Kroese, Elisabeth Brouwer, Frederik K L Spijkervet, Arjan Vissink, Hendrika Bootsma
OBJECTIVES: Besides ocular and oral dryness, fatigue is a major symptom in patients with primary Sjögren's syndrome (pSS). Our aim was to investigate the importance of fatigue in relation to other symptoms experienced as well as to evaluate the effect of rituximab treatment on fatigue in pSS patients with active disease. METHODS: This analysis was based on data from our open-label rituximab study in 28 pSS patients. Symptoms of dryness, physical fatigue, pain, and mental fatigue were scored on 0-10 scales (according to ESSPRI)...
December 28, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28024826/predictors-of-discordance-between-symptoms-and-signs-in-dry-eye-disease
#19
Jelle Vehof, Nicole Sillevis Smitt-Kamminga, Simone A Nibourg, Christopher J Hammond
PURPOSE: To investigate predictors of discordance between symptoms and signs in dry eye disease (DED). DESIGN: Cross-sectional association study. PARTICIPANTS: A total of 648 patients with dry eye from the Groningen LOngitudinal Sicca StudY (GLOSSY), a tertiary dry eye clinic patient cohort from the Netherlands. METHODS: Patient symptoms were assessed using the Ocular Surface Disease Index (OSDI) questionnaire. Dry eye signs were assessed by tear osmolarity, Schirmer test, tear breakup time, corneal and conjunctival staining, and meibomian gland dysfunction, all in both eyes, and a composite dry eye signs severity score was calculated from these 6 tests for each patient...
December 23, 2016: Ophthalmology
https://www.readbyqxmd.com/read/28017209/high-dose-methotrexate-with-leucovorin-rescue-for-monumentally-severe-cns-inflammatory-syndromes
#20
Shin C Beh, Eric Kildebeck, Ram Narayan, Allen Desena, Doug Schell, Elizabeth S Rowe, Vernon Rowe, Dennis Burns, Louis Whitworth, Teresa C Frohman, Benjamin Greenberg, Elliot M Frohman
BACKGROUND: At sufficiently high doses, methotrexate (HDMTX) achieves substantial CNS penetration, whereas other tissues can be rescued from the effects of HDMTX by leucovorin rescue (LR), which does not penetrate the blood-brain barrier. OBJECTIVES: To report on the efficacy and safety of HDMTX with LR (HDMTX-LR), in the treatment of acute demyelinating inflammatory CNS syndromes refractory to conventional immunotherapy. METHODS: We performed a retrospective chart review of 12 patients treated (6 multiple sclerosis [MS], 4 neuromyelitis optica [NMO], and 2 Sjogren's syndrome myelopathy [SSM]) with HDMTX-LR after failing to improve, or exhibiting worsening following conventional immunotherapy...
January 15, 2017: Journal of the Neurological Sciences
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