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Sjogren' Syndrome

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https://www.readbyqxmd.com/read/29907136/wilson-s-disease-combined-with-systemic-lupus-erythematosus-a-case-report-and-literature-review
#1
Yun Zhang, Dongmei Wang, Wei Wei, Xuejun Zeng
BACKGROUND: Wilson's disease (WD) is an inherited disorder in which defective biliary excretion of copper leads to its accumulation, particularly in the liver and brain. Systemic lupus erythematosus (SLE) is a multi-system disorder that can manifest in any system. Cases with concomitant WD and SLE, unrelated to treatment with penicillamine, have been rarely reported. CASE PRESENTATION: We report a case of a young woman who had typical neuropsychiatric symptoms and laboratory tests results of WD...
June 15, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29904425/analysis-of-clinical-risk-factors-in-relapsed-patients-with-class-iv-lupus-nephritis
#2
Jing Wang, Yuan-Yuan Qi, Xue-Ping Chen, Li Ma, Li-Li Zhang, Yu Zhao, Mei Wang
The present study aimed to investigate risk factors for renal recurrence in patients with type IV lupus nephritis (LN). Univariate and multivariate analyses were conducted to using the Cox proportional hazard model and the Kaplan-Meier method. A total 244 patients were diagnosed with type IV LN; 100 (28.49%) relapsed and 144 (41.03%) recovered successfully. Kaplan-Meier method analysis indicated that patients with type IV LN affecting the digestive tract had high renal recurrence rates. Patients with hyperglobulinemia, positive anti-ribonucleoprotein and anti-Sjögren's syndrome type B (anti-SSB) antibodies, thrombus in the loop or non-inflammatory necrotizing vasculopathy also had a high recurrence rate...
June 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29902805/evidence-for-the-detection-of-subclinical-retinal-involvement-in-systemic-lupus-erythematosus-and-sj%C3%A3-gren-syndrome-a-potential-association-with-therapies
#3
Paola Conigliaro, Paola Triggianese, Gianluca Draghessi, Claudia Canofari, Gianluca Aloe, Maria Sole Chimenti, Claudia Valeri, Carlo Nucci, Roberto Perricone, Massimo Cesareo
BACKGROUND: Retinal involvement in systemic lupus erythematosus (SLE) and Sjögren syndrome (SS) may be subclinical and thus underdiagnosed. OBJECTIVES: We aimed at evaluating morphological and functional visual abnormalities in a cohort of SLE and SS patients in the absence of an overt clinical visual impairment. We also investigated potential associations between retinal disorders and disease activity, organ involvement, and treatment with steroid and/or hydroxychloroquine...
June 14, 2018: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/29901591/prevalence-risk-factors-and-prognosis-of-interstitial-lung-disease-in-a-large-cohort-of-chinese-primary-sj%C3%A3-gren-syndrome-patients-a-case-control-study
#4
Hui Gao, Xue-Wu Zhang, Jing He, Jing Zhang, Yuan An, Ye Sun, Ru-Lin Jia, Sheng-Guang Li, Li-Jing Zhang, Zhan-Guo Li
To determine the prevalence of pulmonary complications in primary Sjögren syndrome (pSS), and to identify the risk factors and the prognosis associated with pulmonary involvement in pSS patients.A total of 1341 hospitalized patients (853 with pSS and 488 with secondary Sjögren syndrome [sSS]) were retrospectively reviewed. Of these, 165 hospitalized patients with pSS-associated interstitial lung disease (ILD) were analyzed and recruited as a study group. Eighty-four pSS patients without organ damage were included as a control group...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29901009/a-case-of-primary-sj%C3%A3-gren-s-syndrome-presenting-as-mass-like-encephalitis-with-progression-to-neuromyelitis-optica-spectrum-disorder
#5
Hyun-Jung Lee, Sung Hae Chang, Eun Ha Kang, Yun Jong Lee, Yeong Wook Song, You-Jung Ha
Neuromyelitis optica is an idiopathic inflammatory demyelinating disease of the central nervous system (CNS) that predominantly affects the optic nerves and spinal cord. With the discovery of the pathogenic anti-aquaporin-4 (AQP4) antibody, the disease was recognized as part of a spectrum of autoimmune diseases that target AQP4, collectively referred to as neuromyelitis optica spectrum disorder (NMOSD). NMOSD consists of conditions that affect various parts of the CNS with the AQP4 antibody. In this article, we report a 43-year-old female patient who was initially diagnosed with primary Sjögren's syndrome (pSS) with CNS involvement, but was later diagnosed with overlapping pSS and NMOSD, which required more intensive treatment...
December 2017: Archives of Rheumatology
https://www.readbyqxmd.com/read/29901004/chronic-non-productive-cough-in-patients-with-primary-sj%C3%A3-gren-s-syndrome
#6
Gonca Doğru, Ayşe Balkarli, Atalay Dogru
Objectives: This study aims to evaluate the frequency of chronic cough due to dryness in the trachea and the relationship between depression and chronic cough in patients with primary Sjögren's syndrome (pSS). Patients and methods: Eighty non-smoking patients (7 males, 73 females; mean age 47.6±9.2 years; range 18 to 70 years) with newly diagnosed pSS were included. All patients were evaluated clinically, radiologically, and physiologically. Patients with cough were assessed using the Leicester Cough Questionnaire and visual analog scale...
December 2017: Archives of Rheumatology
https://www.readbyqxmd.com/read/29900998/beta-2-microglobulin-in-whole-unstimulated-saliva-can-effectively-distinguish-between-sj%C3%A3-gren-s-syndrome-and-non-autoimmune-sicca-symptoms
#7
Janett Riega-Torres, Guillermo Delgado-García, Julio César Salas-Alanís, Cassandra Skinner-Taylor, Lorena Pérez-Barbosa, Mario Garza-Elizondo, Celia Nohemí Sánchez-Domínguez, Luis Ángel Ceceñas-Falcón, Karim Mohamed-Noriega, Jesús Mohamed-Hamsho, David Vega-Morales
Objectives: This study aims to describe salivary beta-2 microglobulin (sB2M) levels in our setting and to assess the performance of sB2M for the diagnosis of Sjögren's syndrome (SS). Patients and methods: This cross-sectional, comparative study included 192 SS patients (2 males, 190 females; mean age 53.1 years; range 23 to 84 years) and 64 healthy controls (1 male, 63 females; mean age 46.9 years; range 21 to 82 years). Patients were divided into three groups as those with primary SS, secondary SS, and sicca non-Sjögren's syndrome (snSS)...
December 2017: Archives of Rheumatology
https://www.readbyqxmd.com/read/29899769/neuro-ichthyotic-syndromes-a-case-series
#8
Faruk Incecık, Ozlem M Herguner, Mehmet N Ozbek, Serdal Gungor, Mustafa Yılmaz, Wiliam B Rizzo, Gülen G Mert
Background: The neuro-ichthyotic diseases are clinically and genetically heterogeneous. The purpose of this study was to evaluate the clinical and neuroradiological findings and to analyze mutation in 15 patients with neuro-ichthyotic diseases. Materials and Methods: We retrospectively analyzed the records of 15 patients with the diagnosis of neuro-ichthyotic diseases. Results: Eight female and seven male patients (age range 11 months-52 years) were investigated...
January 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29894742/association-of-high-5-hydroxymethylcytosine-levels-with-ten-eleven-translocation-2-overexpression-and-inflammation-in-sj%C3%A3-gren-s-syndrome-patients
#9
Carolina Lagos, Patricia Carvajal, Isabel Castro, Daniela Jara, Sergio González, Sergio Aguilera, María-José Barrera, Andrew F G Quest, Verónica Bahamondes, Claudio Molina, Ulises Urzúa, Marcela A Hermoso, Cecilia Leyton, María-Julieta González
Here, we determined the 5-hydroxymethylcytosine (5hmC), 5-methylcytosine (5mC), Ten Eleven Translocation (TETs), and DNA methyltransferases (DNMTs) levels in epithelial and inflammatory cells of labial salivary glands (LSG) from Sjögren's syndrome (SS)-patients and the effect of cytokines on HSG cells. LSG from SS-patients, controls and HSG cells incubated with cytokines were analysed. Levels of 5mC, 5hmC, DNMTs, TET2 and MeCP2 were assessed by immunofluorescence. In epithelial cells from SS-patients, an increase in TET2, 5hmC and a decrease in 5mC and MeCP2 were observed, additionally, high levels of 5mC and DNMTs and low levels of 5hmC were detected in inflammatory cells...
June 9, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29892541/lymphocytic-interstitial-pneumonia-in-a-patient-with-mixed-connective-tissue-disease-a-case-report
#10
Chin-Wei Kuo, Kung-Chao Chang, Han-Yu Chang, Tang-Hsiu Huang
Lymphocytic interstitial pneumonia (LIP) is an uncommon interstitial lung disease that is characterized by an interstitial infiltrate of lymphoplasmacytic cells. While idiopathic LIP appears to be extremely rare, most reported cases of LIP have been associated with coexisting immune derangements, particularly autoimmune diseases such as Sjögren's syndrome. In this report, we describe the presentation of LIP in a patient with underlying mixed connective tissue disease.
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29891110/antinuclear-antibodies-and-cancer-a-literature-review
#11
REVIEW
Alexandru Vlagea, Sandra Falagan, Gerardo Gutiérrez-Gutiérrez, Juan Moreno-Rubio, María Merino, Francisco Zambrana, Enrique Casado, María Sereno
Antinuclear antibodies (ANAs) are a spectrum of autoantibodies targeted to various nuclear and cytoplasmic components of the cells. They are very useful as serological markers for different autoimmune disease, like systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), scleroderma, polymyositis, or mixed connective tissue disease. In these years, an increasing attention has been focussed in the relationship between tumours and autoimmunity. Different authors have demonstrated that ANAs are presented, not only in autoimmune diseases, also in serum of patients with different types of cancers...
July 2018: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/29890347/endoplasmic-reticulum-stress-in-autoimmune-diseases-can-altered-protein-quality-control-and-or-unfolded-protein-response-contribute-to-autoimmunity-a-critical-review-on-sj%C3%A3-gren-s-syndrome
#12
REVIEW
María-José Barrera, Sergio Aguilera, Isabel Castro, Sergio González, Patricia Carvajal, Claudio Molina, Marcela A Hermoso, María-Julieta González
For many years, researchers in the field of autoimmunity have focused on the role of the immune components in the etiopathogenesis of autoimmune diseases. However, some studies have demonstrated the importance of target tissues in their pathogenesis and the breach of immune tolerance. The immune system as well as target tissue cells (plasmatic, β-pancreatic, fibroblast-like synoviocytes, thyroid follicular and epithelial cells of the lachrymal glands, salivary glands, intestine, bronchioles and renal tubules) share the characteristic of secretory cells with an extended endoplasmic reticulum (ER)...
June 8, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29889694/an-update-on-the-role-of-type-i-interferons-in-systemic-lupus-erythematosus-and-sj%C3%A3-gren-s-syndrome
#13
Gudny Ella Thorlacius, Marie Wahren-Herlenius, Lars Rönnblom
PURPOSE OF REVIEW: Systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS) share several clinical and laboratory features, including an overexpression of type I interferon (IFN) regulated genes. The genetic background to this IFN signature and the role of the type I IFN system in the disease process have been partly clarified. Here, we summarize the latest information concerning the type I IFN system in both diseases. RECENT FINDINGS: A number of gene variants in the type I IFN signalling pathways associate with an increased risk for both SLE and pSS in several ethnicities...
June 9, 2018: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/29885537/impact-of-obesity-on-autoimmune-arthritis-and-its-cardiovascular-complications
#14
REVIEW
Katrien Van Raemdonck, Sadiq Umar, Zoltán Szekanecz, Ryan K Zomorrodi, Shiva Shahrara
Obesity can instigate and sustain a systemic low-grade inflammatory environment that can amplify autoimmune disorders and their associated comorbidities. Metabolic changes and inflammatory factors produced by the adipose tissue have been reported to aggravate autoimmunity and predispose the patient to cardiovascular disease (CVD) and metabolic comorbidities. Rheumatoid arthritis (RA) and psoriatic arthritis (PsA) are autoimmune arthritic diseases, often linked with altered body mass index (BMI). Severe joint inflammation and bone destruction have a debilitating impact on the patient's life; there is also a staggering risk of cardiovascular morbidity and mortality...
June 6, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29882349/t-follicular-like-helper-cells-in-the-peripheral-blood-of-patients-with-primary-sj%C3%A3-gren-s-syndrome
#15
Karl A Brokstad, Marita Fredriksen, Fan Zhou, Brith Bergum, Johan G Brun, Rebecca J Cox, Kathrine Skarstein
Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by exocrine gland dysfunction, mainly causing sicca symptoms. B cells have a prominent role in SS, and the T follicular helper (TFH ) cells provide B cells with survival- and specialization signals in germinal centers. Here we investigate peripheral TFH cells in pSS. Sixteen pSS patients and healthy controls were enrolled in the study, with thirteen women and three men in each group. Whole blood was collected and separated into PBMC and plasma, followed by cryopreservation...
June 8, 2018: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29881381/current-insights-in-microbiome-shifts-in-sjogren-s-syndrome-and-possible-therapeutic-interventions
#16
REVIEW
Christina Tsigalou, Elisavet Stavropoulou, Eugenia Bezirtzoglou
Sjogren's syndrome (SS) is an autoimmune disease, among the most common ones, that targets mainly the exocrine glands as well as extra-glandular epithelial tissues. Their lymphocytic infiltration leads to manifestations from other organs (e.g., kidneys, lungs, liver, or thyroid), apart from sicca symptoms (xerostomia and keratoconjunctivitis). SS is more prevalent in women than in men (9:1). Moreover, p.SS patients are in increased risk to develop lymphoma. Certain autoantibodies (e.g., antibodies against ribonucleoprotein autoantigens Ro-SSA and La-SSB) are ultimate hallmarks for the disease...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29880622/cerebellar-degeneration-in-primary-sj%C3%B3-gren-syndrome
#17
Mohammad Heidary, Samira Alesaeidi, Khashayar Afshari
Neurological manifestations are reported as a consequence of primary Sjӧgren syndrome (PSS). Any part of the brain and peripheral nervous system can be involved in PSS. However, cerebellar degeneration and atrophy associated with PSS have been rarely reported. Our report describes a 22-year-old woman who presented with cerebellar ataxia, arthritis and arthralgia. Evaluation of her symptoms, autoantibodies and salivary gland pathology was in favour of the diagnosis of Sjögren syndrome. Also, her brain MRI revealed cerebellar degeneration...
June 6, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29879064/primary-sjogren-syndrome-diagnosed-simultaneously-with-localized-amyloidosis-of-the-lacrimal-gland-a-case-report
#18
Seong-Min Kweon, Jung Hee Koh, Han-Na Lee, Eunsung Kim, Min Wook So, Ho-Jin Shin, Hee Young Choi, Seung-Geun Lee
INTRODUCTION: Amyloidosis accompanied by Sjögren's syndrome (SS) has been reported to occur primarily in the skin, lungs, tongue, and mammary gland. However, SS in association with secondary amyloidosis is rarely reported, and knowledge of its relevance is inadequate. Here we report a case of primary SS diagnosed simultaneously with localized amyloidosis of the lacrimal gland. CASE PRESENTATION: A 45-year-old woman complaining of a left eyelid mass was referred to the hospital and was diagnosed with localized amyloidosis after excisional biopsy...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29876386/perturbation-of-transcriptome-in-non-neoplastic-salivary-gland-epithelial-cell-lines-derived-from-patients-with-primary-sj%C3%A3-gren-s-syndrome
#19
Aigli G Vakrakou, Alexandros Polyzos, Efstathia K Kapsogeorgou, Dimitris Thanos, Menelaos N Manoussakis
The data presented here are related to the research article titled "Impaired anti-inflammatory activity of PPARγ in the salivary epithelia of Sjögren's syndrome patients imposed by intrinsic NF-κB activation " (Vakrakou et al., Journal of Autoimmunity, in press, 2017). In the cited manuscript, using comparative analyses of salivary gland biopsy specimens and ductal salivary gland epithelial cell (SGEC) lines from SS patients and disease controls, we have demonstrated that the ductal epithelia of SS patients display constitutively reduced PPARγ expression, transcriptional activity and anti-inflammatory function that were associated with cell-autonomously activated NF-κB and IL-1β pathways in these cells...
April 2018: Data in Brief
https://www.readbyqxmd.com/read/29876352/anti-microbial-antibodies-host-immunity-and-autoimmune-disease
#20
REVIEW
Peilin Zhang, Lawrence M Minardi, J Todd Kuenstner, Steven M Zekan, Rusty Kruzelock
Autoimmune diseases are a spectrum of clinical inflammatory syndromes with circulating autoantibodies. Autoimmune diseases affect millions of patients worldwide with enormous costs to patients and society. The diagnosis of autoimmune diseases relies on the presence of autoantibodies and the treatment strategy is to suppress the immune system using specific or non-specific immunosuppressive agents. The discovery of anti-microbial antibodies in the blood of patients with Crohn's disease and Sjogren's syndrome and cross-reactivity of anti-microbial antibodies to human tissue suggests a new molecular mechanism of pathogenesis, raising the possibility of designing a new therapeutic strategy for these patients...
2018: Frontiers in Medicine
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