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Sjogren' Syndrome

Huma Siddiqui, Tsute Chen, Ardita Aliko, Piotr M Mydel, Roland Jonsson, Ingar Olsen
BACKGROUND: Reduced salivation is considered a major clinical feature of most but not all cases of primary Sjögren's syndrome (pSS). Reduced saliva flow may lead to changes in the salivary microbiota. These changes have mainly been studied with culture that typically recovers only 65% of the bacteria present. OBJECTIVE: This study was to use high throughput sequencing, covering both cultivated and not-yet-cultivated bacteria, to assess the bacterial microbiota of whole saliva in pSS patients with normal salivation...
2016: Journal of Oral Microbiology
Siddharth Jain, Animesh Ray, Manish Soneja, S Leve Joseph Devarajan, Shyam Madabhushi, Anitha Swamy, T Naveen, S K Sharma
A known case of primary Sjogren's syndrome with chronic kidney disease presented with respiratory symptoms and subsequent altered sensorium. Chest imaging suggested cavitating lung lesions in both the lungs. Serum c-ANCA was positive without any upper respiratory tract involvement or active urinary sediments. Treatment with appropriate anti-microbials produced no improvement in the respiratory or neurological parameters. MRI brain revealed tell-tale signs of CNS vascultis. A diagnosis of GPA was made as per European Medical Agency algorithm and the patient was started on immune-suppressants to which there was dramatic response...
October 2016: Journal of the Association of Physicians of India
Minan Y Al-Ezzi, Neha Pathak, Anwar R Tappuni, Khalid S Khan
OBJECTIVES: The aim of this study is to assess the impact of dryness caused by primary Sjögren's Syndrome (pSS) on smell, taste and sexual function in female patients, and its influence on quality of life. METHODS: Electronic databases including MEDLINE via Ovid, Web of Science, SCOUPUS, EMBASE and COCHRANE LIBRARY were searched until April 2016. Studies that assessed the function of smell, taste and sexuality in pSS patients, defined by the American European Consensus Group (AECG) criteria...
October 20, 2016: Modern Rheumatology
Geraint P Williams, Peter Nightingale, Sue Southworth, Alastair K O Denniston, Paul J Tomlins, Stephen Turner, John Hamburger, Simon J Bowman, S John Curnow, Saaeha Rauz
Purpose: Ocular mucous membrane pemphigoid (OcMMP) is a rare autoimmune disorder resulting in progressive conjunctival fibrosis and ocular surface failure leading to sight loss in up to 50%. This study was designed to optimize an ocular surface sampling technique for identification of novel biomarkers associated with disease activity and/or progressive fibrosis. Methods: Fifty-seven patients with OcMMP underwent detailed examination of conjunctival inflammation and fibrosis using fornix depth measurement...
October 1, 2016: Investigative Ophthalmology & Visual Science
Sohei Kobayashi, Tyuji Hoshino, Takaki Hiwasa, Mamoru Satoh, Bahityar Rahmutulla, Sachio Tsuchida, Yuji Komukai, Tomoaki Tanaka, Hisahiro Matsubara, Hideaki Shimada, Fumio Nomura, Kazuyuki Matsushita
Anti-PUF60, poly(U)-binding-splicing factor, autoantibodies are reported to be detected in the sera of dermatomyositis and Sjogren's syndrome that occasionally associated with malignancies. PUF60 is identical with far-upstream element-binding protein-interacting repressor (FIR) that is a transcriptional repressor of c-myc gene. In colorectal cancers, a splicing variant of FIR that lacks exon2 (FIRΔexon2) is overexpressed as a dominant negative form of FIR. In this study, to reveal the presence and the significance of anti-FIRs (FIR/FIRΔexon2) antibodies in cancers were explored in the sera of colorectal and other cancer patients...
October 15, 2016: Oncotarget
Arsenio Spinillo, Fausta Beneventi, Elena Locatelli, Vèronique Ramoni, Roberto Caporali, Claudia Alpini, Giulia Albonico, Chiara Cavagnoli, Carlomaurizio Montecucco
BACKGROUND: The burden of pregnancy complications associated with well defined, already established systemic rheumatic diseases preexisting pregnancy such as rheumatoid arthritis, systemic lupus erythematosus or scleroderma is well known. Systemic rheumatic diseases are characterized by a long natural history with few symptoms, an undifferentiated picture or a remitting course making difficult a timely diagnosis. It has been suggested that screening measures for these diseases could be useful but the impact of unrecognized systemic rheumatic disorders on pregnancy outcome is unknown...
October 18, 2016: BMC Pregnancy and Childbirth
Marcella Nebbioso, Valeria Fameli, Magda Gharbiya, Marta Sacchetti, Anna Maria Zicari, Alessandro Lambiase
The dry eye disease (DED) is a chronic multifactorial disorder of the tears that also involves the ocular surface, the lacrimal glands, and meibomian dysfunction. Furthermore, DED is often associated with Sjögren's syndrome (SS) and other autoimmune disorders. Sometimes, this chronic or subclinical condition is difficult to diagnose and treat, due to its heterogeneity. Areas covered: A literature search of relevant publications about treatment of DED was performed. All relevant articles published between 2011 and 2016 were identified through a computerized search for review and clinical trials using Pub Med database...
October 18, 2016: Expert Opinion on Investigational Drugs
Nicole C Droz, Stephanie D Mathew
After presenting with nonspecific symptoms, a 52-year-old female was found to have a 3.4 × 2.6 cm retroperitoneal mass resulting in hydronephrosis on abdominal computed tomography scan. After extensive workup including surgical debulking, excisional biopsy, and autoimmune panel, she was found to have idiopathic retroperitoneal fibrosis (IRF) with negative staining for immunoglogulin G4 (IgG4). Two years after presentation, the patient developed dyspnea, cough, and xerostomia. Repeat anti-Sjögren Syndrome A and anti-Sjögren Syndrome B titers and labial gland biopsy confirmed the diagnosis of concomitant Sjögren's syndrome (SS)...
October 2016: Military Medicine
Chih-Ching Yeh, Wen-Chang Wang, Chien-Sheng Wu, Fung-Chang Sung, Chien-Tien Su, Ying-Hua Shieh, Shih-Ni Chang, Fu-Hsiung Su
[This corrects the article DOI: 10.1371/journal.pone.0161958.].
2016: PloS One
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...
October 5, 2016: Cornea
Julie A Birt, YingMeei Tan, Neelufar Mozaffarian
OBJECTIVES: To better understand the real-world characteristics and costs of Sjögren's syndrome (SS). METHODS: Analysing the MarketScan Commercial Claims database from Jan. 1, 2006 to Dec. 31, 2011, we identified 10,414 patients ≥18 years old newly diagnosed with SS. Patient characteristics, drugs (commonly used for SS), resource utilisation, and medical costs were evaluated for 12 months pre- and post-diagnosis. RESULTS: Mean age was 55 years; 90% were female...
October 7, 2016: Clinical and Experimental Rheumatology
Gabriela M Balarini, Eliana Zandonade, Leandro Tanure, Gilda A Ferreira, Wildner M Sardenberg, Érica V Serrano, Cleia C Dias, Túlio P Navarro, Hilde H Nordal, Piotr M Mydel, Johan G Brun, Karl A Brokstad, Eva Gerdts, Roland Jonsson, Valéria Valim
OBJECTIVES: We aimed to identify the association of carotid atherosclerosis with the traditional risk factors, disease features, cytokine profile, and calprotectin in patients with primary Sjögren's syndrome (pSS). METHODS: 63 primary pSS patients and 63 age- and sex-matched healthy controls underwent carotid ultrasound, clinical and laboratory examination. The presence of carotid plaques was taken as carotid atherosclerosis. The covariates of carotid atherosclerosis were identified in univariate and multivariate regressions...
September 8, 2016: Clinical and Experimental Rheumatology
Charles Nicaise, Laura Weischelbaum, Liliane Schandene, Valérie Gangji, Florence Dehavay, Joanna Bouchat, Benoît Balau, Thomas Vogl, Muhammad S Soyfoo
OBJECTIVES: To determine the role of S100A8/A9 in the pathogenesis of primary Sjögren's syndrome (pSS). METHODS: The serum levels of S100A8/A9 were determined in pSS patients and healthy controls by ELISA. The expression of S100A8/A9 in salivary glands was assessed by immunohistochemistry. The phenotype of S100A8+ and S100A9+ cells was identified using double immunofluorescence. The effects of S100A8/A9 on cytokine production by peripheral blood mononuclear cells (PBMCs) from pSS patients were determined in vitro by flow cytometry...
October 7, 2016: Clinical and Experimental Rheumatology
Fabiola Atzeni, Luigi Gianturco, Chiara Colombo, Cristian Ricci, Piercarlo Sarzi-Puttini, Maurizio Turiel
October 3, 2016: Clinical and Experimental Rheumatology
Julian Lawrence Ambrus, Lakshmanan Suresh, Ammon Peck
Sjogren's syndrome (SS) is a complex heterogeneous autoimmune disease resulting in loss of salivary gland and lacrimal gland function that may include multiple systemic manifestations including lymphoma. Multiple cell types participate in disease pathogenesis. This review discusses evidence for abnormal B cell subpopulations in patients with SS, critical roles of B cells in SS and the status of B cell-directed therapies in the management of patients with SS.
October 8, 2016: Journal of Clinical Medicine
Melis Palamar, Nazan Ozsan, Fahri Sahin
A 31-year-old female with Primary Sjögren Syndrome (pSS) presented with bilateral puffiness around the eye for 3 years. The lacrimal glands were hypertrophic and edematous bilaterally. Schirmer 1 score was 2 and 1 mm and tear-film break-up time was 3 and 4 seconds, in the right and the left eyes, respectively. An incisional biopsy from the left lacrimal gland revealed diffuse and intense CD20, CD5, and bcl-2 positivity with negative cyclin D1 and CD23 which supported lymphoma. Upon haematology consultation extranodal marginal zone lymphoma diagnosis was made...
2016: Case Reports in Ophthalmological Medicine
Mehmet Taşdemir, Chiar Hasan, Ayşe Ağbaş, Özgür Kasapçopur, Nur Canpolat, Lale Sever, Salim Çalışkan
Systemic lupus erythematosus and Sjögren's syndrome are chronic auto- inflammatory disorders which can lead to serious organ damage. Although systemic lupus erythematosus and Sjögren's syndrome were previously considered two forms of the same disease because of presence of clinical coexistence of these two conditions, the view that they are two different conditions with mutual characteristics has become prominent in recent years. In this paper, we reported a 16 year-old girl who was followed up with a diagnosis of Sjögren's syndrome for six years and then was observed to have overlap of systemic lupus erythematosus...
September 2016: Türk Pediatri Arşivi
K Mugundhan, M C Mayan Vasif, G Prakash, N Balamurugan, K G Sivakumar, B Bakthavatchalam
No abstract text is available yet for this article.
March 2016: Journal of the Association of Physicians of India
Phayvanh P Sjogren, Richard K Gurgel, Albert H Park
OBJECTIVES: To determine whether a canal wall down mastoidectomy can provide long-term benefit for children with aural stenosis. METHODS: Retrospective case series of children with congenital aural stenosis having undergone a canal wall down mastoidectomy over a twelve-year period at a tertiary children's hospital. RESULTS: Data from thirteen children who underwent a total of twenty canal wall down mastoidectomies for aural stenosis were reviewed...
November 2016: International Journal of Pediatric Otorhinolaryngology
Chang-Fu Kuo, Shue-Fen Luo, Kuang-Hui Yu, Lai-Chu See, Weiya Zhang, Michael Doherty
BACKGROUND: Systemic sclerosis (SSc) is a rare and devastating disease affecting skin and internal organs. Familial aggregation of SSc and co-aggregation with other autoimmune diseases is rarely reported. METHODS: We identified 23,658,577 beneficiaries registered with the National Health Insurance database in 2010, 1891 of whom had SSc. We identified 21,009,551 parent-child relationships and 17,168,340 full sibling pairs. The familial risks of SSc and other autoimmune diseases and familial transmission were estimated...
October 12, 2016: Arthritis Research & Therapy
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