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https://www.readbyqxmd.com/read/28937580/diagnosis-of-systemic-inflammatory-diseases-among-patients-admitted-for-acute-pericarditis-with-pericardial-effusion
#1
Maureen Assayag, Rachid Abbas, Noémie Chanson, Anne Perozziello, Gregory Ducrocq, Jean-François Alexandra, Antoine Dossier, Thomas Papo, Karim Sacre
AIMS: Acute pericarditis may be the heralding manifestation of various systemic inflammatory diseases (SIDs). The aim of this study was to identify clinical indicators for SIDs in patients admitted for acute pericarditis with pericardial effusion. METHODS: All consecutive adult patients hospitalized in a Department of Internal Medicine over a 10-year period for acute pericarditis with pericardial effusion were retrospectively reviewed. Patients with cancer and tuberculosis were excluded...
September 20, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28937480/tubulointerstitial-nephritis-in-sj%C3%A3-gren-syndrome-treated-with-mycophenolate-mofetil
#2
Abhilash Koratala, Westley H Reeves, Mark S Segal
No abstract text is available yet for this article.
October 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28937084/primary-sj%C3%A3-gren-s-syndrome-complicated-by-anti-neutrophil-cytoplasmic-antibody-mediated-crescentic-glomerulonephritis
#3
Imed Ben Ghorbel, Nabil Belfeki, Thouraya Ben Salem, Mohamed Habib Houman
Ocular and oral dryness are the hallmark of Sjögren's syndrome (SS). However, SS can be associated with a variety of complications, affecting organs such as the liver, kidneys, lungs, muscle, and nervous system. Renal involvement has been usually in the form of tubulointerstitial nephritis. However, glomerulonephritis is rare in primary SS. We report three clinical cases of SS with anti-neutrophil cytoplasmic antibody-mediated crescentic glomerulo-nephritis treated with prednisone and cyclophosphamide, with favorable outcome...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28933131/the-clinical-spectrum-of-primary-sj%C3%A3-gren-s-syndrome-beyond-exocrine-glands
#4
M C Leone, A Alunno, G Cafaro, V Valentini, E Marcucci, E Bartoloni, R Gerli
Although primary Sjögren's syndrome (pSS) is a mild indolent chronic disease mainly characterized by mucosal dryness in the majority of cases, a consistent subgroup of patients display extra-glandular manifestations. Virtually any organs and systems can be affected, leading to a more serious disease prognosis. Therefore, the prompt identification of patients at higher risk of extra-glandular manifestations is necessary to start a thorough follow up and an aggressive treatment. The aim of this review article is to provide an overview of epidemiological, clinical and serological features of extra-glandular manifestations in pSS as well as current knowledge about putative biomarkers useful in clinical practice...
September 21, 2017: Reumatismo
https://www.readbyqxmd.com/read/28931060/isolated-positive-anti-ss-b-autoantibodies-are-not-related-to-clinical-features-of-systemic-autoimmune-diseases-results-from-a-routine-population-survey
#5
Sabine Jardel, Nicole Fabien, Arnaud Hot, Sandra Vukusic, Jacques Tebib, Vincent Cottin, Pascal Sève, Maurice Laville, Alexandre Belot, Isabelle Durieu, Lorna Garnier, Frédéric Coutant, Quitterie Reynaud, Jean Christophe Lega
OBJECTIVE: To assess in clinical practice the frequency and diagnosis associated with the SS-B-positive/SS-A negative autoantibody profile. METHODS: We analyzed a one-year consecutive population of 624 patients referred by clinicians to the immunology laboratory to investigate anti-SS-A and/or anti-SS-B autoantibodies, who were detected using luminex technology. Data were analyzed for patients with isolated anti-SS-B autoantibodies. The clinical characteristics and diagnosis of connective tissue diseases (CTD) were retrieved according to the international criteria...
2017: PloS One
https://www.readbyqxmd.com/read/28928382/functional-effects-of-proinflammatory-factors-present-in-sj%C3%A3-gren-s-syndrome-salivary-microenvironment-in-an-in-vitro-model-of-human-salivary-gland
#6
Mayte Arce-Franco, María Dominguez-Luis, Martina K Pec, Carlos Martínez-Gimeno, Pablo Miranda, Diego Alvarez de la Rosa, Teresa Giraldez, José María García-Verdugo, José David Machado, Federico Díaz-González
Primary Sjögren's syndrome (pSS) is an autoimmune exocrinopathy in which the role that the immune response plays in reducing exocrine gland function, including the glandular microenvironment of cytokines, has not been fully understood. Epithelial cells from biopsies of human parotid gland (HPG) were used to establish a model of human salivary gland in vitro. In this model, the functional consequences of several proinflammatory soluble factors present in the pSS glandular microenvironment were assessed. Stimulation with isoproterenol and calcium produced a significant increase in the basal activity of amylase in the HPG cell supernatants...
September 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28927315/systematic-review-and-meta-analysis-of-the-epidemiology-of-polyautoimmunity-in-sj%C3%A3-gren-s-syndrome-secondary-sj%C3%A3-gren-s-syndrome-focusing-on-autoimmune-rheumatic-diseases
#7
H Alani, J R Henty, N L Thompson, E Jury, C Ciurtin
OBJECTIVE: The epidemiology of polyautoimmunity in Sjögren's syndrome (secondary Sjögren's syndrome - sSS) is not well defined and has not been investigated before using a systematic approach. We conducted a systematic review of the epidemiology of sSS associated with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), scleroderma, and myositis, assessing the prevalence rates (PRs) and clinical and serological features of sSS. METHOD: A systematic literature search of PubMed and Embase databases (updated to March 2016) was performed to identify all published data on PR, demographic profile, clinical manifestations, laboratory features, and causes of death associated with sSS...
September 20, 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/28926640/aire-deficient-mice-provide-a-model-of-corneal-and-lacrimal-gland-neuropathy-in-sj%C3%A3-gren-s-syndrome
#8
Feeling Y Chen, Albert Lee, Shaokui Ge, Sara Nathan, Sarah M Knox, Nancy A McNamara
Sjögren's syndrome (SS) is a chronic, autoimmune exocrinopathy that leads to severe dryness of the mouth and eyes. Exocrine function is highly regulated by neuronal mechanisms but little is known about the link between chronic inflammation, innervation and altered exocrine function in the diseased eyes and exocrine glands of SS patients. To gain a better understanding of neuronal regulation in the immunopathogenesis of autoimmune exocrinopathy, we profiled a mouse model of spontaneous, autoimmune exocrinopathy that possess key characteristics of peripheral neuropathy experienced by SS patients...
2017: PloS One
https://www.readbyqxmd.com/read/28920690/regional-intestinal-permeability-in-rats-a-comparison-of-methods
#9
Carl Roos, David Dahlgren, E Sjögren, Christer Tannergren, Bertil Abrahamsson, H Lennernas
Currently, the screening of new drug candidates for intestinal permeation is typically based on in vitro models which give no information regarding regional differences along the gut. When evaluation of intestinal permeability by region is undertaken, two preclinical rat models are commonly used, the Ussing chamber method and single-pass intestinal perfusion (SPIP). To investigate the robustness of in vivo predictions of human intestinal permeability, a set of four model compounds was systematically investigated in both these models, using tissue specimens and segments from the jejunum, ileum, and colon of rats from the same genetic strain...
September 18, 2017: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/28919520/antibodies-against-citrullinated-alpha-enolase-peptides-in-primary-sjogren-s-syndrome
#10
Adrianos Nezos, Ilir Cinoku, Clio P Mavragani, Haralampos M Moutsopoulos
Citrullinated alpha enolase (CEP-1) has been designated as a major antigenic target of antibodies against citrullinated proteins (ACPA) in patients with rheumatoid arthritis (RA). Our aim is to determine the prevalence of anti-CEP-1 in a cohort of ACPA positive (ACPA+) primary Sjogren's syndrome (pSS) patients. Anti-CEP1 titers were determined by ELISA in sera from 15 ACPA+ and 45 ACPA- age/sex matched pSS; 12 ACPA+ RA patients and 30 healthy controls (HC). Increased anti-CEP-1 antibody titers were detected in nine out of the 15 (60%) ACPA+ pSS patients and 5 out of 12 (41...
September 14, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28916716/deregulation-of-microrna-expression-in-purified-t-and-b-lymphocytes-from-patients-with-primary-sj%C3%A3-gren-s-syndrome
#11
Shu-Fang Wang-Renault, Saida Boudaoud, Gaétane Nocturne, Elodie Roche, Nelly Sigrist, Christian Daviaud, Andreas Bugge Tinggaard, Victor Renault, Jean-François Deleuze, Xavier Mariette, Jörg Tost
OBJECTIVE: MicroRNAs (miRNAs) play an important role in the pathogenesis of autoimmune diseases such as primary Sjögren's syndrome (pSS). This study is the first to investigate miRNA expression patterns in purified T and B lymphocytes from patients with pSS using a high-throughput quantitative PCR (qPCR) approach. METHODS: Two independent cohorts of both patients with pSS and controls, one for discovery and one for replication, were included in this study. CD4+ T cells and CD19+ B cells were isolated from peripheral blood mononuclear cells by magnetic microbeads and expression of miRNAs was profiled using the Exiqon Human miRNome panel I analysing 372 miRNAs...
September 15, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28916403/a-case-of-severe-acquired-hypertriglyceridemia-in-a-7-year-old-girl
#12
Jessica S Lilley, MacRae F Linton, Jennifer C Kelley, T Brent Graham, Sergio Fazio, Hagai Tavori
We report a case of severe type I hyperlipoproteinemia caused by autoimmunity against lipoprotein lipase (LPL) in the context of presymptomatic Sjögren's syndrome. A 7-year-old mixed race (Caucasian/African American) girl was admitted to the intensive care unit at Vanderbilt Children's Hospital with acute pancreatitis and shock. She was previously healthy aside from asthma and history of Hashimoto's thyroiditis. Admission triglycerides (TGs) were 2191 mg/dL but returned to normal during the hospital stay and in the absence of food intake...
August 12, 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28916184/role-of-the-il-12-il-35-balance-in-sj%C3%A3-gren-s-syndrome
#13
Olivier Fogel, Elodie Rivière, Raphaèle Seror, Gaetane Nocturne, Saida Boudaoud, Bineta Ly, Jacques-Eric Gottenberg, Véronique Le Guern, Jean-Jacques Dubost, Joanne Nititham, Kimberly E Taylor, Philippe Chanson, Philippe Dieudé, Lindsey A Criswell, Bernd Jagla, Alice Thai, Michael Mingueneau, Xavier Mariette, Corinne Miceli-Richard
BACKGROUND: An interferon (IFN) signature is involved in the pathogenesis of primary Sjögren's syndrome (pSS), but whether the signature is type 1 or 2 remains controversial. Mouse models and genetic studies suggested the involvement of T helper 1 and type 2 IFN pathways. Likewise, polymorphisms of interleukin 12A gene (IL-12A), which encodes for IL-12p35, have been associated with pSS. IL-12p35 subunit is shared by 2 heterodimers, IL-12 and IL-35. OBJECTIVE: To confirm the genetic association of IL-12A polymorphism and pSS and elucidate the involvement of the IL-12/IL-35 balance in pSS by functional studies...
September 12, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28916029/oral-mucosa-biology-and-salivary-biomarkers
#14
Rosie Qin, Andrea Steel, Nasim Fazel
Although the surfaces of both the skin and oral mucosa are protected by squamous epithelial cells and fall within the scope of dermatologic practice, the oral cavity contains highly specialized structures and functions distinct from other skin biology and pathologic conditions and are also the purview of clinicians who care for patients with skin and mucosal diseases. We describe the distinct features of the tongue, mucosa, and salivary glands. In particular, we examine the composition and function of the saliva, with special focus on salivary biomarkers...
September 2017: Clinics in Dermatology
https://www.readbyqxmd.com/read/28915122/understanding-fetal-factors-that-contribute-to-preterm-birth-sj%C3%A3-gren-larsson-syndrome-as-a-model
#15
Pippa Staps, Marije Hogeveen, Joris Fuijkschot, Joris van Drongelen, Michèl A A P Willemsen
AIM: Preterm birth is the world's leading cause of neonatal death. Unfortunately, the pathophysiology of preterm birth remains poorly understood. Sjögren-Larsson syndrome is a rare, neurometabolic disorder caused by a fatty aldehyde dehydrogenase deficiency. A majority of patients with Sjögren-Larsson syndrome is born preterm. METHODS: Data of all known Dutch patients with Sjögren-Larsson syndrome and all cases reported in literature were analyzed to learn from preterm birth in context of this rare disease...
September 15, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28914372/the-role-of-gut-microbiota-in-the-pathogenesis-of-rheumatic-diseases
#16
Danli Zhong, Chanyuan Wu, Xiaofeng Zeng, Qian Wang
Rheumatic diseases refer to many diseases with a loss of immune self-tolerance, leading to a chronic inflammation, degeneration, or metabolic derangement in multiple organs or tissues. The cause of rheumatic diseases remains to be elucidated, though both environmental and genetic factors are required for the development of rheumatic diseases. Over the past decades, emerging studies suggested that alteration of intestinal microbiota, known as gut dysbiosis, contributed to the occurrence or development of a range of rheumatic diseases, including rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis, systemic sclerosis, and Sjogren's syndrome, through profoundly affecting the balance between pro- and anti-inflammatory immune responses...
September 15, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28914367/no-association-of-single-nucleotide-polymorphisms-within-h19-and-hox-transcript-antisense-rna-hotair-with-genetic-susceptibility-to-systemic-lupus-erythematosus-rheumatoid-arthritis-and-primary-sj%C3%A3-gren-s-syndrome-in-a-chinese-han-population
#17
An-Fang Huang, Lin-Chong Su, Hong Jia, Yi Liu, Wang-Dong Xu
The H19 (rs2839698, rs3741219) and HOTAIR (rs920778) polymorphisms were related to many kinds of cancers. However, these polymorphisms have been scarcely explored in different autoimmune diseases. Here, we aimed to examine the association of the polymorphisms with susceptibility to or protection against systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and primary Sjögren's syndrome (pSS) among Chinese Han patients. We conducted a case-control study including 800 patients (300 with SLE, 350 with RA, and 150 with pSS) and 350 healthy control individuals...
September 15, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#18
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
September 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28904462/clinical-spectrum-therapeutic-outcomes-and-prognostic-predictors-in-sjogren-s-syndrome-associated-neuropathy
#19
Ajith Sivadasan, Karthik Muthusamy, Bimal Patel, Rohit Ninan Benjamin, A T Prabhakar, Vivek Mathew, Sanjith Aaron, Mathew Alexander
OBJECTIVES: There are limited data regarding long-term follow-up and therapeutic outcomes in Sjogren's syndrome (SS)-associated peripheral neuropathy. In this study, we aim to study the clinical, electrophysiological spectrum and therapeutic responses among the different subtypes of SS-associated neuropathy. The predictors of suboptimal treatment response will be identified. METHODS: The study included a retrospective cohort of patients with SS-associated neuropathy between January 2012 and November 2015...
July 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28904255/predictors-of-favorable-responses-to-immunosuppressive-treatment-in-pulmonary-arterial-hypertension-associated-with-connective-tissue-disease
#20
Hidekata Yasuoka, Yuichiro Shirai, Yuichi Tamura, Tsutomu Takeuchi, Masataka Kuwana
BACKGROUND: The potential efficacy of immunosuppressive (IS) treatment has been reported in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD), but its positioning in the treatment algorithm remains uncertain. The aim of this study was to identify predictors of favorable responses to first-line IS treatment.Methods and Results:This single-center retrospective study included 30 patients with PAH accompanied by systemic lupus erythematosus (SLE), mixed CTD (MCTD), or primary Sjögren's syndrome (SS) who received first-line IS treatment alone or in combination with pulmonary vasodilators...
September 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
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