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JOURNAL ARTICLE
Kimberly K Coca, M Boyd Gillespie, Nicholas A Beckmann, Rongshun Zhu, Tamara M Nelson, Robert L Witt
OBJECTIVES/HYPOTHESIS: This study is a systematic review of the literature which seeks to estimate the expected treatment outcomes of a patient with Sjogren's syndrome (SS) undergoing therapeutic sialendoscopy. STUDY DESIGN: Systematic Review. METHODS: PubMed, Scopus, and Cochrane library databases were used to search for studies published as of August 2020 regarding the treatment outcomes of SS with sialendoscopy. The key search terms included "Sjogren's syndrome" and "sialendoscopy...
July 2021: Laryngoscope
#2
REVIEW
Elena K Shematorova, George V Shpakovski
The neuronal ceroid lipofuscinoses (NCLs) collectively constitute one of the most common forms of inherited childhood-onset neurodegenerative disorders. They form a heterogeneous group of incurable lysosomal storage diseases that lead to blindness, motor deterioration, epilepsy, and dementia. Traditionally the NCL diseases were classified according to the age of disease onset (infantile, late-infantile, juvenile, and adult forms), with at least 13 different NCL varieties having been described at present. The current review focuses on classic juvenile NCL (JNCL) or the so-called Batten (Batten-Spielmeyer-Vogt; Spielmeyer-Sjogren) disease, which represents the most common and the most studied form of NCL, and is caused by mutations in the CLN3 gene located on human chromosome 16...
October 29, 2020: International Journal of Molecular Sciences
#3
REVIEW
Takuya Akiyama, Hiroshi Oiwa, Masahiro Hayashi, Takamichi Sugimoto, Eiichi Nomura, Takemori Yamawaki
Sjögren syndrome (SS) is one of several collagen vascular diseases that occasionally involve the central nervous system. We report two cases of SS involving young patients who initially presented with aseptic meningitis. A male with recurrent AM was found to have anti-Ro/SSA and La/SSB antibodies in a screening test for autoimmune process. A minor salivary gland biopsy revealed lymphocytic infiltrations compatible with SS, although the patient did not exhibit sicca symptoms. A female presenting with AM and polyarthritis also reported xerophthalmia...
August 2020: Nagoya Journal of Medical Science
#4
REVIEW
Harini Bagavant, Aleksandra Michrowska, Umesh S Deshmukh
Sjögren's syndrome (SS) is a chronic rheumatic autoimmune disorder affecting multiple organ systems. The clinical findings in SS patients show considerable heterogeneity and overlap with other autoimmune diseases. In addition, the autoimmune response in SS initiates several years before the appearance of clinical symptoms. Thus, understanding the pathogenic mechanisms involved in the disease process have been a challenge. Several animal model systems of SS-like disease have been developed to overcome these issues...
December 2020: Autoimmunity Reviews
#5
JOURNAL ARTICLE
Marta Ziaja-Sołtys, Magdalena Kołodziejczyk, Beata Rymgayłło-Jankowska, Dominika Wróbel-Dudzińska, Ewa Suchodoła-Ratajewicz, Dominika Szlonzak, Tomasz Żarnowski, Anna Bogucka-Kocka
PURPOSE: Demodex mites infestation, typically asymptomatic, is a problem for patients with weakened immune systems because it often takes the form of symptomatic, massive infection. The Demodex mites play an important role in the occurrence of a range of eye surface diseases such as Demodex blepharitis, Meibomian gland dysfunctions, conjunctivitis and corneal changes. The ocular infection is closely related to the systemic invasion. Our goal was to minimize infestation and alleviate the symptoms of massive demodicosis so as to prevent further damage to the cornea...
October 31, 2020: Acta Parasitologica
#6
JOURNAL ARTICLE
Anne B Tjensvoll, Maria B Lauvsnes, Henrik Zetterberg, Jan T Kvaløy, Ingeborg Kvivik, Stian S Maroni, Ole J Greve, Mona K Beyer, Shunsei Hirohata, Chaim Putterman, Guido Alves, Erna Harboe, Kaj Blennow, Lasse G Gøransson, Roald Omdal
BACKGROUND: To test the hypothesis that neurofilament light (NfL) in CSF is a biomarker of CNS involvement in patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS), we measured NfL in CSF from 52 patients with lupus and 54 with pSS and explored associations with clinical, structural, immunological and biochemical abnormalities. METHODS: In CSF, we measured NfL, anti-P antibodies, protein S100B and TWEAK by ELISA and anti-NR2 antibodies by electrochemiluminescence...
April 2021: Journal of Neurology
#7
JOURNAL ARTICLE
Junho Lee, Jehan Alam, Eunji Choi, Yeon Kyeong Ko, Ahreum Lee, Youngnim Choi
Mice lacking IκB-ζ, a protein encoded by the Nfkbiz gene, spontaneously develop a Sjögren's syndrome-like disease involving the lachrymal glands, but no salivary gland symptoms have been reported. We found that Nfkbiz-/- female mice presented a significantly reduced salivary flow rate, focal lymphocytic sialadenitis (FLS), and a dysbiotic oral microbiota at week 24. To dissect the contributions of genetic and environmental factors to the salivary gland phenotype, Nfkbiz+/+ and Nfkbiz-/- mice were cohoused after weaning and evaluated at week 20...
October 30, 2020: NPJ Biofilms and Microbiomes
#8
JOURNAL ARTICLE
Deng-Ho Yang, Yu-Hsun Wang, Lung-Fa Pan, James Cheng-Chung Wei
Sjögren's syndrome (SS) is a chronic systemic inflammation disease with clinical presentation of dry eye, dry mouth, and polyarthralgia. Active inflammation is associated with an increased risk of associated arterial stiffness or subclinical atherosclerosis-related cardiovascular events. We used the longitudinal health insurance database of Taiwan, which includes one million participants, to evaluate the relationship between the clinical medication of hydroxychloroquine (HCQ) and the development of coronary artery disease (CAD)...
October 28, 2020: Journal of Clinical Medicine
#9
JOURNAL ARTICLE
Mahmoud Mona, Stefania Mondello, Joon Young Hyon, Wafaa Saleh, Kyudong Han, Hyo-Jung Lee, You-Jung Ha, Eun Ha Kang, Yun Jong Lee, Seunghee Cha
OBJECTIVES: To elucidate the clinical values of anti-M3R in Sjögren's syndrome (SS) in the largest cohort for an anti-M3R study. METHODS: The plasma of 361 subjects (156 primary SS [pSS], 62 non-SS-sicca [SICCA], 40 systemic lupus erythematosus [SLE], 50 rheumatoid arthritis [RA], and 53 healthy controls [HC]) was screened using our modified On-Cell-Western assay. Saliva from pSS (n=37) compared to SICCA (n=26) was also analysed. The sensitivity and specificity of anti-M3R and its association with comprehensive clinical and laboratory features were determined...
July 2021: Clinical and Experimental Rheumatology
#10
JOURNAL ARTICLE
Mahima Gupta, Christopher Dass, Heena Garg, Anjolie Chhabra
No abstract text is available yet for this article.
February 2021: Paediatric Anaesthesia
#11
JOURNAL ARTICLE
Yanhong Chen, Yongqiang Chen, Beibei Zu, Jia Liu, Li Sun, Chen Ding, Duping Wang, Xing Cheng, DeLiang Yang, Guoping Niu
OBJECTIVE: To evaluate the plasma levels of lnc-DC in primary Sjögren's syndrome (pSS) patients and investigate the potential associations between lnc-DC and disease activity. METHODS: In this study, we recruited 358 enrollments, including 127 pSS patients without immune thrombocytopenia (ITP), 22 pSS patients with ITP, 50 systemic lupus erythematosus (SLE) patients, and 50 patients with rheumatoid arthritis (RA) and 109 healthy individuals, from Xuzhou Central Hospital...
2020: Journal of Immunology Research
#12
Madiha Huq, Neha Sanan, Phuong Daniels, Robert Hostoffer
INTRODUCTION: Posner-Schlossman syndrome (PSS) is a rare glaucomatocyclitic crisis with clinical features including recurrent episodes of unilateral elevated intraocular pressure. Autoimmune and infectious causes have been proposed as potential etiologies of PSS. We report the first case of PSS in the setting of common variable immunodeficiency (CVID). Case Report . A sixty-two-year-old Caucasian female with a medical history of CVID and ulcerative colitis presented to the emergency room with complaints of acute right-sided vision changes...
2020: Case Reports in Ophthalmological Medicine
#13
MULTICENTER STUDY
Shijia Liu, Yunke Guo, Lu Lu, Jiawei Lu, Mengying Ke, Tingting Xu, Yan Lu, Wenjun Chen, Jue Wang, Deshun Kong, Qiuxiang Shen, Youjuan Zhu, WenFeng Tan, Wei Ji, Wei Zhou
Rheumatoid arthritis (RA), afflicting over 1% of the population, is an inflammatory joint disease leading to cartilage damage and ultimately impaired joint function. Disease-modifying anti-rheumatic drugs are considered as the first-line treatment to inhibit the progression of RA, and the treatment depends on the disease status assessment. The disease activity score 28 as clinical gold standard is extensively used for RA assessment, but it has the limitations of delayed assessment and the need for specialized expertise...
2020: Frontiers in Immunology
#14
JOURNAL ARTICLE
Anders Bergström, Laurent Frantz, Ryan Schmidt, Erik Ersmark, Ophelie Lebrasseur, Linus Girdland-Flink, Audrey T Lin, Jan Storå, Karl-Göran Sjögren, David Anthony, Ekaterina Antipina, Sarieh Amiri, Guy Bar-Oz, Vladimir I Bazaliiskii, Jelena Bulatović, Dorcas Brown, Alberto Carmagnini, Tom Davy, Sergey Fedorov, Ivana Fiore, Deirdre Fulton, Mietje Germonpré, James Haile, Evan K Irving-Pease, Alexandra Jamieson, Luc Janssens, Irina Kirillova, Liora Kolska Horwitz, Julka Kuzmanovic-Cvetković, Yaroslav Kuzmin, Robert J Losey, Daria Ložnjak Dizdar, Marjan Mashkour, Mario Novak, Vedat Onar, David Orton, Maja Pasarić, Miljana Radivojević, Dragana Rajković, Benjamin Roberts, Hannah Ryan, Mikhail Sablin, Fedor Shidlovskiy, Ivana Stojanović, Antonio Tagliacozzo, Katerina Trantalidou, Inga Ullén, Aritza Villaluenga, Paula Wapnish, Keith Dobney, Anders Götherström, Anna Linderholm, Love Dalén, Ron Pinhasi, Greger Larson, Pontus Skoglund
Dogs were the first domestic animal, but little is known about their population history and to what extent it was linked to humans. We sequenced 27 ancient dog genomes and found that all dogs share a common ancestry distinct from present-day wolves, with limited gene flow from wolves since domestication but substantial dog-to-wolf gene flow. By 11,000 years ago, at least five major ancestry lineages had diversified, demonstrating a deep genetic history of dogs during the Paleolithic. Coanalysis with human genomes reveals aspects of dog population history that mirror humans, including Levant-related ancestry in Africa and early agricultural Europe...
October 30, 2020: Science
#15
JOURNAL ARTICLE
Jiangyun Luo, Yongpeng Ge
RATIONALE: Tracheobronchial amyloidosis (TBA) associated with Sjögren syndrome is very rare. Here, we describe a case with this phenomenon, in order to better understand the condition. PATIENT CONCERNS: A 52-year-old woman presented after 6 months of coughing, sputum, and dyspnea. Chest computed tomography revealed thickened bronchial walls, which were irregular on the left side the trachea. She had a history of dry eye and dry mouth of at least 3 years' duration...
October 23, 2020: Medicine (Baltimore)
#16
RANDOMIZED CONTROLLED TRIAL
Q Shao, S Wang, H Jiang, L Liu
Objective: To evaluate the clinical efficacy and safety of iguratimod for the treatment of primary Sjögren's syndrome (pSS) and explore its possible mechanism of action. Method: We conducted a randomized, placebo-controlled clinical trial in 66 pSS patients. Patients were randomized in a 2:1 ratio to receive oral iguratimod for 24 weeks or matching placebo. The primary endpoint was the EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI). Secondary endpoints included mental discomfort visual analogue scale (VAS) score, patient global assessment (PGA), EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI), Schirmer's test values, unstimulated whole salivary flow, erythrocyte sedimentation rate (ESR), and immunoglobulin G (IgG)...
March 2021: Scandinavian Journal of Rheumatology
#17
JOURNAL ARTICLE
Pragnya R Donthineni, Anthony V Das, Swapna S Shanbhag, Sayan Basu
Purpose: To describe the visual outcomes and complications following cataract surgery in dry eye disease (DED). Methods: This retrospective study included 668 eyes of 399 patients with DED, who underwent cataract surgery between 2011 and 2019 at our multi-tier ophthalmology hospital network. Based on etiology, they were divided into three groups: cicatrizing conjunctivitis (CC), meibomian gland dysfunction (MGD), and Sjogren's syndrome (SS). The data on demographics, visual impairment, surgical technique, visual outcomes, and complications were collected using an electronic medical record system...
2020: Frontiers in Medicine
#18
JOURNAL ARTICLE
Maria Herrala, Jopi J W Mikkonen, Paula Pesonen, Reijo Lappalainen, Leo Tjäderhane, Raija K Niemelä, Hubertus Seitsalo, Tuula Salo, Sami Myllymaa, Arja M Kullaa
PURPOSE: To investigate inter- and intra-individual variation in the levels and outputs (concentration multiplied by salivary flow rate) of salivary metabolites in patients with primary Sjögren's syndrome (pSS). METHODS: A total of 56 samples of stimulated saliva were collected from 14 female pSS patients during four laboratory visits within 20 weeks and analyzed using proton nuclear magnetic resonance spectroscopy. Single saliva samples from each of 15 controls were also analyzed...
October 29, 2020: Journal of Oral Science
#19
JOURNAL ARTICLE
Hauke Thomsen, Xinjun Li, Kristina Sundquist, Jan Sundquist, Asta Försti, Kari Hemminki
DESIGN: Addison's disease (AD) is a rare autoimmune disease (AID) of the adrenal cortex, present as an isolated AD or part of autoimmune polyendocrine syndromes (APSs) 1 and 2. Although AD patients present with a number of AID co-morbidities, population-based family studies are scarce, and we aimed to carry out an unbiased study on AD and related AIDs. METHODS: We collected data on patients diagnosed with AIDs in Swedish hospitals and calculated standardized incidence ratios (SIRs) in families for concordant AD and for other AIDs, the latter as discordant relative risks...
November 2020: Endocrine Connections
#20
Arjit Agarwal, Shruti Chandak
Bilateral lacrimal gland enlargement is uncommon; however, its presence induces brainstorming process and intensive discussion between a clinician and an imaging specialist, leading to exploration of multiple systemic disease patterns such as lymphoproliferative disorders, sarcoidosis, Sjögren's syndrome, and tuberculosis. Thoughtful analysis and diagnostic work-up are required to confirm the diagnosis. Sarcoidosis is a rare systemic disease, with ocular involvement being still rarer. Here, we report a case of a young male presenting with nodular swelling over lateral aspects of both the eyes...
July 2020: Taiwan Journal of Ophthalmology
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