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Sicca Syndrome

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https://www.readbyqxmd.com/read/29465367/proprotein-convertase-enzyme-furin-is-upregulated-in-primary-sj%C3%A3-gren-s-syndrome
#1
Noora Ranta, Atte Valli, Anna Grönholm, Olli Silvennoinen, Pia Isomäki, Marko Pesu, Marja Pertovaara
OBJECTIVES: The proprotein convertase enzyme FURIN is a critical regulator of the anti-inflammatory TGFβ-1 cytokine and peripheral immune tolerance. In T cells, FURIN is co-regulated with IFN-γ and thus highly expressed in T helper 1 type cells. Previous studies have demonstrated that FURIN is upregulated in inflammatory conditions, including atherosclerosis, rheumatoid arthritis and systemic lupus erythematosus. Here, we evaluated the levels of FURIN in the plasma and peripheral blood mononuclear cells (PBMCs) of patients with primary Sjögren's syndrome (pSS) and in healthy controls...
February 21, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29465360/serum-soluble-interleukin-2-receptor-is-a-useful-biomarker-for-disease-activity-but-not-for-differential-diagnosis-in-igg4-related-disease-and-primary-sj%C3%A3-gren-s-syndrome-adults-from-a-defined-population
#2
Mitsuhiro Akiyama, Takanori Sasaki, Yuko Kaneko, Hidekata Yasuoka, Katsuya Suzuki, Kunihiro Yamaoka, Tsutomu Takeuchi
OBJECTIVES: To identify biomarkers for disease activity in IgG4-related disease (IgG4-RD) and primary Sjögren's syndrome (pSS). METHODS: Forty-three consecutive treatment-naïve patients with IgG4-RD, 62 patients with pSS, and 5 patients with sicca syndrome were enrolled. IgG4-RD and pSS disease activity was assessed based on the IgG4-RD responder index (IgG4-RD RI) and EULAR Sjögren's Syndrome Disease Activity Index (ES- SDAI), respectively. The associations of biomarkers with disease activity were examined...
February 15, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29458245/impact-of-sleep-quality-on-clinical-features-of-primary-sj%C3%A3-gren-s-syndrome
#3
Sang Wan Chung, Jaehyung Hur, You-Jung Ha, Eun Ha Kang, Joon Young Hyon, Hyo-Jung Lee, Yeong Wook Song, Yun Jong Lee
Background/Aims: This study aimed to investigate the inf luence of poor sleep quality on clinical features of primary Sjögren's syndrome (pSS). Methods: Sleep quality was cross-sectionally assessed using the Pittsburgh Sleep Quality Index (PSQI), and demographic, clinical, and laboratory data were collected from 115 Korean patients with pSS. The patients completed questionnaires on the European League Against Rheumatism (EULAR) SS Patient Reported Index (ESSPRI), quality of life (EuroQOL five dimensions questionnaire [EQ-5D]), fatigue (fatigue severity score [FSS]), and depression (Beck Depression Inventory [BDI] II])...
February 20, 2018: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/29457375/evidence-for-alternate-modes-of-b-cell-activation-involving-fab-acquired-n-glycosylations-in-antibody-secreting-cells-infiltrating-the-labial-salivary-glands-of-sj%C3%A3-gren-s-syndrome-patients
#4
Kristi A Koelsch, Joshua Cavett, Kenneth Smith, Jacen S Moore, Sylvain D Lehoux, Nan Jia, Tim Mather, Syed M S Quadri, Astrid Rasmussen, C Erick Kaufman, David M Lewis, Lida Radfar, Teresa A Scordino, Christopher J Lessard, Biji T Kurien, Richard D Cummings, Judith A James, Kathy L Sivils, A Darise Farris, R Hal Scofield
OBJECTIVE: To better understand the role of B cells, potential mechanisms for their aberrant activation, and the production of autoantibodies in the pathogenesis of Sjögren's Syndrome (SS), we explored selection pressures and N-glycosylation acquired by somatic mutation (acN-glyc) in the immunoglobulin (Ig) variable regions (V-regions) of antibody secreting cells (ASCs) isolated from the minor salivary glands of SS patients and non-SS controls with sicca symptoms. METHODS: We report a novel method to produce and characterize recombinant monoclonal antibodies (mAbs) from SS patient and control labial salivary gland single-cell sorted ASC infiltrates that can be utilized to concurrently probe any other expressed genes...
February 18, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29456945/audiological-profile-in-primary-sj%C3%A3-gren-s-syndrome-in-a-tertiary-care-setting-and-its-clinical-implications
#5
C V Thanooja, Ann Mary Augustine, Anjali Lepcha, Pulukool Sandhya, Amit Kumar Tyagi, Debashish Danda, Achamma Balraj
This study aims to assess the frequency and the profile of hearing loss among patients with primary Sjögren's syndrome in a tertiary care hospital in India and to look for an association between hearing loss and immunological parameters (anti-SSA antibody, anti-SSB antibody, anticardiolipin antibodies, complements C3 and C4). This prospective observational study was done from January 2011 to October 2011 on consecutive patients diagnosed with primary Sjögren's syndrome in our tertiary care hospital. All patients underwent a puretone audiogram, tympanogram and acoustic reflex testing...
March 2018: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/29449828/understanding-spreading-depression-from-headache-to-sudden-unexpected-death
#6
REVIEW
Olga Cozzolino, Maria Marchese, Francesco Trovato, Enrico Pracucci, Gian Michele Ratto, Maria Gabriella Buzzi, Federico Sicca, Filippo M Santorelli
Spreading depression (SD) is a neurophysiological phenomenon characterized by abrupt changes in intracellular ion gradients and sustained depolarization of neurons. It leads to loss of electrical activity, changes in the synaptic architecture, and an altered vascular response. Although SD is often described as a unique phenomenon with homogeneous characteristics, it may be strongly affected by the particular triggering event and by genetic background. Furthermore, SD may contribute differently to the pathogenesis of widely heterogeneous clinical conditions...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29411664/salivary-gland-ultrasonography-findings-are-associated-with-clinical-histological-and-serologic-features-of-sj%C3%A3-gren-s-syndrome
#7
J-W Kim, H Lee, S-H Park, S-K Kim, J-Y Choe, J K Kim
OBJECTIVE: Salivary gland ultrasonography (SGUS) has been applied in the diagnosis of Sjögren's syndrome (SS). The aim of this study is to investigate the association of SGUS findings with clinical, histological, and serologic features of SS. METHODS: A total of 104 patients with suspected SS underwent SGUS for evaluation of salivary gland involvement. Patients with primary SS were determined according to the classification criteria for SS. The parenchymal inhomogeneity of bilateral parotid and submandibular glands was graded from 0 (homogeneity) to 4 (gross inhomogeneity)...
February 7, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29406358/sjogren-syndrome-and-non-sjogren-sicca-syndrome-do-they-affect-the-vulva
#8
James Scurry, Tania Day
No abstract text is available yet for this article.
March 2018: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/29390329/primary-sj%C3%A3-gren-syndrome-that-initially-presented-with-repeated-hypergammaglobulinemic-purpura-after-prolonged-sitting-a-case-report
#9
Zhihua Zhou, Weiqiang Jiang, Ming Wang, Yongyuan Liu, Wei Zhang, Manping Huang, Donghui Liang
RATIONALE: Purpura is a common dermatologic manifestation in Sjögren syndrome (SS). When a patient presents with sicca symptoms, the diagnosis of SS is not difficult. PATIENT CONCERNS: Here, we reported a case of a 52-year-old Chinese woman who initially presented with nonpalpable purpura on both lower extremities, and these lesions had developed soon after prolonged sitting. In the past 2 years, she had repeated cutaneous nonpalpable purpura 4 times. She had no sicca symptoms, dry eyes, or dry mouth...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29388084/unexplained-cough-it-is-time-to-rule-out-sjogren-s-syndrome
#10
Matthew Koslow, Shaye Kivity, Vicktoria Vishnevskia-Dai, Issahar Ben-Dov
Sjogren's syndrome is associated with chronic cough, but sicca symptoms are missing from cough evaluation guidelines. We evaluated patients with unexplained cough for undiagnosed Sjogren's syndrome. Patients referred to our pulmonary clinic (Sheba Medical Center, 2009 to 2012) with unexplained cough and concomitant dry eyes were selected for evaluation. Unexplained cough was defined as chronic cough of unknown etiology despite algorithm-based evaluation and treatment. Patients were evaluated in a dedicated clinic by a pulmonologist, rheumatologist, and ophthalmologist specializing in autoimmune disease...
January 31, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29387196/decreased-microrna-181a-and-16-expression-levels-in-the-labial-salivary-glands-of-sj%C3%A3-gren-syndrome-patients
#11
Yuhua Wang, Guohua Zhang, Lingling Zhang, Miansong Zhao, Hongdong Huang
Sjögren syndrome (SS) is characterized by dysfunction of the exocrine glands, particularly the salivary and lacrimal glands. Thus, labial salivary gland biopsy is useful method for diagnosing SS. The aim of the present study was to investigate the microRNA (miRNA or miR) profile of labial salivary glands obtained from SS patients and to examine the correlation of miR-181a and -16 levels with the pathological grade in SS. miRNA expression in labial salivary gland tissues was profiled in 3 female patients with primary SS and 3 female patients with non-SS sicca syndrome using microarray analysis...
January 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29361207/blood-t-follicular-regulatory-cells-t-follicular-helper-cells-ratio-marks-ectopic-lymphoid-structure-formation-and-pd-1-icos-t-follicular-helper-cells-indicate-disease-activity-in-primary-sj%C3%A3-gren-s-syndrome
#12
Valter R Fonseca, Vasco C Romão, Ana Agua-Doce, Mara Santos, Dolores López-Presa, Ana Cristina Ferreira, João Eurico Fonseca, Luis Graca
OBJECTIVES: To investigate whether the balance of blood T follicular helper (Tfh) and Foxp3+ T follicular regulatory (Tfr) cells can inform on ectopic lymphoid neogenesis and disease activity in primary Sjögren's syndrome (SS). METHODS: We prospectively recruited 56 patients with clinical suspicion of SS, 16 of which subsequently fulfilled American European Consensus Group primary SS classification criteria and were compared with 16 patients with non-Sjögren sicca syndrome...
January 23, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29318645/endometriosis-increased-the-risk-of-bladder-pain-syndrome-interstitial-cystitis-a-population-based-study
#13
Chia-Chang Wu, Shiu-Dong Chung, Herng-Ching Lin
OBJECTIVE: Previous studies have suggested an association between bladder pain syndrome/interstitial cystitis (BPS/IC) and endometriosis. However, no nation-wide population study has yet reported an association between them. In this study, we examined the risk of BPS/IC among subjects with endometriosis during a 3-year follow-up in Taiwan using a population-based dataset. STUDY DESIGN: This study comprised 9191 subjects with endometriosis, and 27 573 subjects randomly selected as controls...
January 10, 2018: Neurourology and Urodynamics
https://www.readbyqxmd.com/read/29246416/hepatobiliary-involvement-in-systemic-sclerosis-and-the-cutaneous-subsets-characteristics-and-survival-of-patients-from-the-spanish-rescle-registry
#14
Begoña Marí-Alfonso, Carmen Pilar Simeón-Aznar, Alfredo Guillén-Del Castillo, Manuel Rubio-Rivas, Luis Trapiella-Martínez, José Antonio Todolí-Parra, Mónica Rodríguez Carballeira, Adela Marín-Ballvé, Nerea Iniesta-Arandia, Dolores Colunga-Argüelles, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, José Antonio Vargas Hitos, Antonio-J Chamorro, Ana Belen Madroñero-Vuelta, Isabel Perales-Fraile, Xavier Pla-Salas, Rafael A Fernández-De-La-Puebla, Vicent Fonollosa-Pla, Carles Tolosa-Vilella
OBJECTIVE: To assess the prevalence and causes of hepatobiliary involvement (HBI) in systemic sclerosis (SSc), to investigate the clinical characteristics and prognosis of SSc patients with HBI (SSc-HBI) and without HBI (SSc-non-HBI), and to compare both groups according to the cutaneous SSc subsets. METHODS: In all, 1572 SSc patients were collected in the RESCLE registry up to January 2015, and all hepatobiliary disturbances were recorded. We investigated the HBI-related characteristics and survival from the entire SSc cohort and according to the following cutaneous subsets: diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc sine scleroderma (ssSSc)...
October 6, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29243035/systemic-lupus-erythematosus-and-ocular-involvement-an-overview
#15
REVIEW
Rosanna Dammacco
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical features. Virtually any organ system can be affected, including the eye. SLE-related eye involvement can be diagnosed in approximately one-third of the patients and is usually indicative of disease activity. An early diagnosis and the adoption of suitable therapeutic measures are necessary to prevent sight-threatening consequences, especially in patients with juvenile SLE. Periocular lesions, such as eyelid involvement and orbital inflammation, are relatively rare and, in case of orbital masses, may require a biopsy control...
December 14, 2017: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/29231165/polyvalent-immunoglobulins-with-vitamin-d3-and-vitamin-b12-in-the-treatment-of-sjogren-s-syndrome-in-a-vegetarian-with-stomatitis-glossodynia-xerostomia-and-elevated-antinuclear-antibodies-case-report%C3%A2
#16
Clemens Cuny, Barbara Vaerst, Jennis Gabrielpillai, Aykut Tahtali, Sven Balster, Reinhard Lissner, Barry G Woodcock
BACKGROUND: Sjogren's syndrome, involving sicca symptoms with xerostomia, stomatitis, and considerable pain is a difficult-to-treat autoimmune disease where the treatment options are limited and, as in the case of methotrexate, have a low therapeutic index. CASE REPORT: This case report concerns a male patient, aged 75 years and vegetarian, with Sjogren's syndrome subsequently confirmed by salivary gland biopsy. Serum antinuclear antibodies (ANA) were elevated (1 : 320)...
December 12, 2017: International Journal of Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/29209436/the-potential-relationship-between-flammer-and-sj%C3%A3-gren-syndromes-the-chime-of-dysfunction
#17
REVIEW
Babak Baban, Olga Golubnitschaja
Flammer syndrome (FS) is a term to blanket a cluster of vascular and nonvascular signs and symptoms linked to primary vascular dysregulation (PVD), increased sensitivity to various stimuli (stress, drugs, etc.) and altered sense regulation such as pain, smell and thirst perception. On one hand, disruption of blood barrier and homeostasis of the body are the main targets of vascular irregularity. Inflammation and immune disorders including autoimmunity are considered as a consequence of the abnormal vascular regulation processes...
December 2017: EPMA Journal
https://www.readbyqxmd.com/read/29208023/comparison-of-2002-aecg-and-2016-acr-eular-classification-criteria-and-added-value-of-salivary-gland-ultrasonography-in-a-patient-cohort-with-suspected-primary-sj%C3%A3-gren-s-syndrome
#18
Maëlle Le Goff, Divi Cornec, Sandrine Jousse-Joulin, Dewi Guellec, Sebastian Costa, Thierry Marhadour, Rozenn Le Berre, Steeve Genestet, Béatrice Cochener, Sylvie Boisrame-Gastrin, Yves Renaudineau, Jacques-Olivier Pers, Alain Saraux, Valérie Devauchelle-Pensec
BACKGROUND: The objective was to evaluate concordance between 2002 American-European Consensus Group (AECG) and 2016 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's syndrome (pSS) and to assess how salivary gland ultrasonography (SGUS) might improve the classification of patients. METHODS: Patients with suspected pSS underwent a standardised evaluation, including SGUS, at inclusion into the single-centre Brittany DIApSS cohort...
December 6, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29148407/minor-salivary-gland-fibrosis-in-sj%C3%A3-gren-s-syndrome-is-elevated-associated-with-focus-score-and-not-solely-a-consequence-of-aging
#19
Kerry M Leehan, Nathan P Pezant, Astrid Rasmussen, Kiely Grundahl, Jacen S Moore, Lida Radfar, David M Lewis, Donald U Stone, Christopher J Lessard, Nelson L Rhodus, Barbara M Segal, R Hal Scofield, Kathy L Sivils, Courtney Montgomery, A Darise Farris
OBJECTIVES: Evaluate the presence of minor salivary gland (SG) fibrosis in primary Sjögren's syndrome (pSS) as a function of disease pathology or a consequence of ageing. METHODS: Subjects with sicca symptoms attending a Sjögren's research clinic were classified by American European Consensus Group (AECG) criteria as either pSS or non-SS (nSS). Discovery (n=34 pSS, n=28 nSS) and replication (n=35 pSS, n=31 nSS) datasets were evaluated. Minor SG cross-sections from haematoxylin and eosin stained slides were imaged, digitally reconstructed and analysed for percent area fibrosis...
October 23, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29130141/sj%C3%A3-gren-s-syndrome-initially-presented-as-thrombotic-thrombocytopenic-purpura-in-a-male-patient-a-case-report-and-literature-review
#20
REVIEW
Xiaohan Xu, Tienan Zhu, Di Wu, Lu Zhang
Thrombotic thrombocytopenic purpura (TTP) is a potentially lethal multisystem disorder which could be caused by autoimmune diseases. However, the concomitant occurrence of TTP and Sjögren's syndrome (SS) is an extremely uncommon scenario, especially in male patients. A 56-year-old Chinese male was admitted for the appearance of diffuse ecchymosis. Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever. Laboratory investigations suggested anemia, thrombocytopenia, significantly increased lactic dehydrogenase, schistocytes in peripheral blood smear, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers...
November 13, 2017: Clinical Rheumatology
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