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    Systemic Lupus Erythematosus and hearing disorders: Literature review and meta-analysis of clinical and temporal bone findings.
    J Int Med Res 2017 Jan 1:300060516688600. Epub 2017 Jan 1.
    2 Department of Oral and Maxillofacial Sciences, Sapienza University of Rome, Italy.
    Objective This literature review and meta-analysis was performed to evaluate the correlations among hearing and vestibular clinical symptoms, temporal bone findings, and pathological mechanisms in patients with systemic lupus erythematosus (SLE). Study design Relevant papers in the literature were retrospectively reviewed. Clinical hearing aspects in patients with SLE and relevant temporal bone studies in the same field were analyzed. Read More

    Cystatin S-a candidate biomarker for severity of submandibular gland involvement in Sjögren's syndrome.
    Rheumatology (Oxford) 2017 Feb 16. Epub 2017 Feb 16.
    Department of Clinical and Experimental Medicine, University of Pisa.
    Objectives.: Salivary cystatin S is a defence protein mainly produced by submandibular glands and involved in innate oral immunity. This study aimed to verify whether cystatin S was diversely expressed in different disease subsets of primary Sjogren's syndrome (pSS) patients, defined on the basis of salivary flow [unstimulated salivary flow rate (USFR)], minor salivary gland (MSG) focus score and submandibular gland ultrasonography abnormalities. Read More

    MicroRNA expression profiles identify disease-specific alterations in systemic lupus erythematosus and primary Sjögren's syndrome.
    PLoS One 2017 24;12(3):e0174585. Epub 2017 Mar 24.
    Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.
    The discovery of microRNAs (miRNAs) and their critical role in genetic control opened new avenues in understanding of various biological processes including immune cell lineage commitment, differentiation, proliferation and apoptosis. However, a given miRNA may have hundreds of different mRNA targets and a target might be regulated by multiple miRNAs, thus the characterisation of dysregulated miRNA expression profiles could give a better insight into the development of immunological disturbances in autoimmune diseases. The aim of our study was to examine the changes in miRNA expression profiles in patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS). Read More

    One year in review 2017: primary Sjögren's syndrome.
    Clin Exp Rheumatol 2017 Mar-Apr;35(2):179-191. Epub 2017 Mar 15.
    Rheumatology Unit, University of Perugia, Italy.
    Primary Sjögren's syndrome (pSS) is a complex and heterogeneous disease. Last year, a great deal of basic and clinical research was carried out in pSS. Following the previous reviews of this publishing series, we will herewith provide a critical digest of the most recent literature on pSS pathogenesis, clinical manifestations and treatment. Read More

    Comparison of performance of the 2016 ACR-EULAR classification criteria for primary Sjögren's syndrome with other sets of criteria in Japanese patients.
    Ann Rheum Dis 2017 Mar 22. Epub 2017 Mar 22.
    Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan.
    Objectives: To compare the performance of the new 2016 American College of Rheumatology (ACR)-European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's syndrome (SS) with 1999 revised Japanese Ministry of Health criteria for diagnosis of SS (JPN), 2002 American-European Consensus Group classification criteria for SS (AECG) and 2012 ACR classification criteria for SS (ACR) in Japanese patients.

    Methods: The study subjects were 499 patients with primary SS (pSS) or suspected pSS who were followed up in June 2012 at 10 hospitals in Japan. All patients had been assessed for all four criteria of JPN (pathology, oral, ocular, anti-SS-A/SS-B antibodies). Read More

    Insight into pathogenesis of Sjögren's syndrome: Dissection on autoimmune infiltrates and epithelial cells.
    Clin Immunol 2017 Mar 19. Epub 2017 Mar 19.
    Department of Pathophysiology & Academic Joint Rheumatology Program, School of Medicine, National University of Athens, Greece. Electronic address:
    Sjögren's syndrome (SS) is a chronic autoimmune disease with broad clinical spectrum, extending from benign exocrinopathy to severe systemic disease and lymphoma development. The glandular and extraglandular dysfunction of SS is associated with lymphocytic infiltrates that invade the epithelial structures of affected organs. The in-depth study of autoimmune lesions in the minor salivary glands (MSG), which are the major target-organ of SS responses, revealed that the lymphocytic infiltrates vary in severity and composition among SS-patients, are full-blown at diagnosis and remain unchanged thereafter. Read More

    [Dental implantation in a patient with Sjögren's syndrome: a case report].
    Hua Xi Kou Qiang Yi Xue Za Zhi 2017 Feb;35(1):108-111
    Center of Oral Medicine, Foshan Hospital of TCM, Guangzhou University of Chinese Medicine, Foshan 528000, China.
    We reported one case of a patient with Sjögren's syndrome and dental implant. This patient was followed up for more than one year. The patient was diagnosed with Sjögren's syndrome for 5 years and treated with long-term corticosteroid. Read More

    A Potential of sFasL in Preventing Gland Injury in Sjogren's Syndrome.
    Biomed Res Int 2017 23;2017:5981432. Epub 2017 Feb 23.
    Department of Rheumatology and Clinical Immunology, The First Hospital of Xiamen University, Xiamen 361003, China; Xiamen Key Laboratory of Rheumatology and Clinical Immunology, Xiamen, China.
    Fas and its ligand FasL, members of tumor necrosis factor receptor superfamily, have been implicated in the process of cell apoptosis. FasL consists of two forms, membrane FasL (mFasL) and soluble FasL (sFasL). sFasL can be produced by mFasL cleaved by matrix metalloproteinases (MMP) and also reveals a role for binding to Fas which is expressed on cell surface. Read More

    Prevalence and characterization of non-sicca onset primary Sjögren syndrome with interstitial lung involvement.
    Clin Rheumatol 2017 Mar 21. Epub 2017 Mar 21.
    Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Via del Pozzo, 71 41121, Modena, Italy.
    Primary Sjögren syndrome (pSS)-related interstitial lung disease (ILD) involved about 10-20% of patients. In 20% of cases, ILD can be diagnosed before pSS; anyway, few studies have investigated the frequency of ILD as the first clinically relevant manifestation of pSS, generally referred to retrospective studies. Aim of our prospective study was to describe prevalence, clinical, serological, and instrumental features of non-sicca onset pSS patients with interstitial lung involvement. Read More

    Diffusional kurtosis imaging of parotid glands in Sjögren's syndrome: Initial findings.
    J Magn Reson Imaging 2017 Mar 21. Epub 2017 Mar 21.
    Department of Radiology, Nanjing Drum Tower Hospital, Affiliated Hospital of Nanjing University Medical School, Nanjing, P.R. China.
    Purpose: To explore the role of diffusion kurtosis imaging (DKI) of parotid glands in diagnosing Sjögren's syndrome (SS).

    Materials And Methods: A total of 40 patients with SS and 40 healthy volunteers underwent 3.0T magnetic resonance imaging (MRI) including DKI, which generated the apparent diffusion coefficient (ADC), corrected diffusion (D), and diffusional kurtosis (K) values. Read More

    Gastrointestinal and liver lesions in primary childhood Sjögren syndrome.
    Clin Rheumatol 2017 Mar 20. Epub 2017 Mar 20.
    Department of Pediatrics, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, Japan.
    Sjögren syndrome (SS) is characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands, leading to keratoconjunctivitis sicca and xerostomia. SS is one of the most common autoimmune rheumatic diseases in adults; however, few cases of primary childhood SS with gastrointestinal and liver lesions have been reported in the literature. We report five cases of primary childhood SS with gastrointestinal and liver lesions. Read More

    Predictive factors for the placebo effect in clinical trials for dry eye: a pooled analysis of three clinical trials.
    Br J Ophthalmol 2017 Mar 18. Epub 2017 Mar 18.
    Department of Pharmacoepidemiology, Graduate School of Medicine, Kyoto University, Yoshida Konoecho, Sakyoku, Kyoto, Japan
    Background: Placebo effect is one of the methodological difficulties in dry eye clinical trials. If we could elucidate the tendencies of the placebo response and find predictors, we could reduce the placebo response in clinical trials for dry eye. In this study, we investigated the predictive factors for the placebo effect in dry eye clinical trials. Read More

    OPHTHALMIC FINDINGS IN LATE STAGE SJOGREN-LARSSON SYNDROME.
    Retin Cases Brief Rep 2017 Mar 15. Epub 2017 Mar 15.
    *Harkness Eye Institute, Columbia University Medical Center; and †Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Naples, Florida.
    Purpose: To report spectral domain optical coherence tomography and fundus autofluorescence documentation of late stage macular findings associated with Sjogren-Larsson Syndrome in three adult siblings.

    Methods: Three adult siblings with Sjogren-Larsson Syndrome underwent ophthalmic examination and imaging.

    Results: Crystalline maculopathy and subretinal deposits, presumably lipofuscin accumulation, with macular atrophy were present in varying degrees in all three adult siblings. Read More

    Frontal fibrosing alopecia in association with Sjögren's syndrome: more than a simple coincidence.
    An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):14-16
    Hospital do Servidor Público Estadual do Estado de São Paulo (HSPE) - São Paulo (SP), Brazil.
    Frontal fibrosing alopecia is a distinctive form of scarring alopecia considered to be a clinical variant of lichen planopilaris. It predominantly occurs in postmenopausal women and has a slowly progressive course. It was first described by Kossard in 1994. Read More

    Recent advances in understanding and managing IgG4-related disease.
    F1000Res 2017 23;6. Epub 2017 Feb 23.
    Massachusetts General Hospital, Allergy and Immunology Division, Cox 201, 55 Fruit Street, Boston, Massachusetts, 02114, USA.
    IgG4-related disease was only recently discovered, so its description, management, and new discoveries related to its etiology are rapidly evolving. Because IgG4 itself is a unique antibody which is intimately related to the diagnosis of the disease, the role of plasmablasts in the pathophysiology remains an active area of discussion. Recent studies have uncovered a possible role for CD4-positive cytotoxic T lymphocytes, T follicular helper cells, and M2 macrophages. Read More

    The Risk of Deep Venous Thrombosis and Pulmonary Embolism in Primary Sjögren Syndrome: A General Population-based Study.
    J Rheumatol 2017 Mar 15. Epub 2017 Mar 15.
    From Arthritis Research Canada, Richmond; Division of Rheumatology, Department of Medicine, Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada; Department of Rheumatology, Division of Rheumatology, Allergy and Immunology, Harvard Medical School, Boston, Massachusetts, USA. Funded by the Canadian Arthritis Network, The Arthritis Society of Canada, the British Columbia Lupus Society (grant 10-SRP-IJD-01), and the Canadian Institutes for Health Research (grants MOP-125960 and THC-135235). J.A. Aviña-Zubieta, MD, PhD, Associate Professor of Medicine, Division of Rheumatology, Department of Medicine, University of British Columbia, and Research Scientist, Arthritis Research Canada; M. Jansz, MD, Internal Medicine Resident, Department of Medicine, Faculty of Medicine, University of British Columbia; E.C. Sayre, PhD, Statistician, Arthritis Research Canada; H.K. Choi, MD, DrPH, Professor of Medicine, Department of Rheumatology, Division of Rheumatology, Allergy and Immunology, Harvard Medical School, and Research Scientist, Arthritis Research Canada. Address correspondence to Dr. J.A. Aviña-Zubieta, 5591 No. 3 Road, Richmond, British Columbia V6X 2C7, Canada. E-mail: Accepted for publication February 10, 2017.
    Objective: To estimate the future risk and time trends of venous thromboembolism (VTE) in individuals with newly diagnosed primary Sjögren syndrome (pSS) in the general population.

    Methods: Using a population database that includes all residents of British Columbia, Canada, we created a study cohort of all patients with incident SS and up to 10 controls from the general population matched for age, sex, and entry time. We compared incidence rates (IR) of pulmonary embolism (PE), deep vein thrombosis (DVT), and VTE between the 2 groups according to SS disease duration. Read More

    Randomized Controlled Trial of Rituximab and cost-effectiveness analysis in treating fatigue and oral dryness in primary Sjogren's Syndrome.
    Arthritis Rheumatol 2017 Mar 10. Epub 2017 Mar 10.
    William Harvey Research Institute, Barts and The London School of Medicine and Dentistry, Lower Ground Floor, Old Anatomy Building, Charterhouse Square, London, EC1M 6BQ, UK.
    Objective: We investigated whether rituximab, an anti-B-cell therapy, improved symptoms of fatigue and oral dryness in patients with Primary Sjögren's Syndrome (PSS).

    Methods: Multicentre, randomised, double-blind, parallel-group placebo-controlled trial, including Health Economic Analysis. Anti-Ro positive patients with PSS, symptomatic fatigue and oral dryness were recruited from 25 UK rheumatology clinics from August 2011 to January 2014. Read More

    Extrahepatic Manifestations of Primary Biliary Cholangitis.
    Gut Liver 2017 Mar 16. Epub 2017 Mar 16.
    Department of Medicine, University of California, Los Angeles, CA, USA.
    Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren's syndrome, thyroid dysfunction and systemic sclerosis. Read More

    Criteria sets for primary Sjogren's syndrome are not adequate for those presenting with extraglandular organ involvements as their dominant clinical features.
    Rheumatol Int 2017 Mar 13. Epub 2017 Mar 13.
    Rheumatology, Gazi Universitesi Tıp Fakültesi, Ankara, Turkey.
    Patients with primary Sjogren's syndrome (pSS) may go undiagnosed or be misclassified due to the insidious nature and wide spectrum of the disease. The available several classification criteria emphasize glandular findings. We aimed to analyze the efficiency of various classification criteria sets in patients diagnosed on the clinical basis by expert opinion and to compare those pSS patients who fulfilled these criteria with those who did not. Read More

    Clinical features and management of non-gastrointestinal non-ocular extranodal mucosa associated lymphoid tissue (ENMALT) marginal zone lymphomas.
    Best Pract Res Clin Haematol 2017 Mar - Jun;30(1-2):99-108. Epub 2016 Nov 5.
    Department of Hematology, MD Anderson Cancer Center, Madrid, Spain. Electronic address:
    Extranodal mucosa associated lymphoid tissue (ENMALT) marginal zone lymphomas may arise at any site of the body. The most frequent localizations other than gastrointestinal and eye are salivary gland, skin, lung and thyroid. These lymphomas usually arise in a setting of inflammation due to a persistent infection or autoimmune diseases such as Sjogren syndrome in salivary MALT lymphomas and Hashimoto's thryroiditis in thyroid lymphomas. Read More

    Marginal zone lymphoma: Associated autoimmunity and auto-immune disorders.
    Best Pract Res Clin Haematol 2017 Mar - Jun;30(1-2):65-76. Epub 2016 Nov 5.
    Histopathology Department, Royal Marsden Hospital, London, UK.
    Large epidemiological studies have shown a consistent increased risk for developing lymphoma in the setting of autoimmune disorders (AID). It is known that this link appears to be stronger for some AID and certain non-Hodgkin lymphoma subtypes e.g. Read More

    Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren's syndrome.
    Reumatol Clin 2017 Mar 8. Epub 2017 Mar 8.
    Department of Pediatric Hematology/Oncology and Blood & Marrow Transplant, Cleveland Clinic, OH, USA.
    Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes into exocrine glands, resulting in the typical sicca symptoms. Unlike adults, primary SS is a very rare condition in childhood, and the risk of malignancy in juvenile SS (JSS) has not been defined. We report the detection of extranodal marginal zone B-cell lymphoma (EMZL) occurring in two children with SS. Read More

    Autodisplay of the La/SSB protein on LPS-free E. coli for the diagnosis of Sjögren's syndrome.
    Enzyme Microb Technol 2017 May 24;100:1-10. Epub 2017 Jan 24.
    Department of Materials Science and Engineering, Yonsei University, 50 Yonsei-ro, Seo-dae-mun-gu, Seoul 120-749, Republic of Korea. Electronic address:
    The objective of this study was to present an immunoassay for the diagnosis of Sjögren's syndrome based on the autodisplayed La/SSB protein on the outer membrane of intact E. coli (strain UT-5600) and LPS-free E. coli (ClearColi™). Read More

    The Potential Role for Early Biomarker Testing as Part of a Modern, Multidisciplinary Approach to Sjögren's Syndrome Diagnosis.
    Adv Ther 2017 Mar 10. Epub 2017 Mar 10.
    Division of Allergy, Immunology and Rheumatology, Department of Medicine, SUNY at Buffalo School of Medicine, Buffalo, NY, USA.
    Sjögren's syndrome (SS) is a chronic and progressive multisystem autoimmune disease typically managed by rheumatologists. Diagnostic delays are common, due in large part to the non-specific and variable nature of SS symptoms and the slow progression of disease. The hallmark characteristics of SS are dry eye and dry mouth, but there are a broad range of other possible symptoms such as joint and muscle pain, skin rashes, chronic dry cough, vaginal dryness, extremity numbness or tingling, and disabling fatigue. Read More

    Is it Sjögren's syndrome or burning mouth syndrome? Distinct pathoses with similar oral symptoms.
    Oral Surg Oral Med Oral Pathol Oral Radiol 2017 Apr 24;123(4):482-495. Epub 2017 Jan 24.
    Department of Oral Diagnostics Sciences, State University of New York at Buffalo, Buffalo, NY, USA; Oral Biology, State University of New York at Buffalo, Buffalo, NY, USA. Electronic address:
    Sjögren's syndrome (SS) and burning mouth syndrome (BMS) typically occur in postmenopausal women. Although these conditions have significantly different etiopathogeneses, patients with SS or BMS often present with analogous oral complaints. The similarities between the two conditions have led to considerable confusion on the part of medical and dental practitioners, and those with BMS or SS often wait years to receive a diagnosis. Read More

    Alterations in the Salivary Proteome and N-Glycome of Sjögren's Syndrome Patients.
    J Proteome Res 2017 Mar 24. Epub 2017 Mar 24.
    Department of Obstetrics, Gynecology and Reproductive Sciences, University of California, San Francisco , San Francisco, California 94143, United States.
    We used isobaric mass tagging (iTRAQ) and lectin affinity capture mass spectrometry (MS)-based workflows for global analyses of parotid saliva (PS) and whole saliva (WS) samples obtained from patients diagnosed with primary Sjögren's Syndrome (pSS) who were enrolled in the Sjögren's International Collaborative Clinical Alliance (SICCA) as compared with two control groups. The iTRAQ analyses revealed up- and down-regulation of numerous proteins that could be involved in the disease process (e.g. Read More

    What is the clinical significance of anti-Sm antibodies in systemic lupus erythematosus? A comparison with anti-dsDNA antibodies and C3.
    Clin Exp Rheumatol 2017 Mar 3. Epub 2017 Mar 3.
    Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Germany.
    Objectives: To investigate the clinical value of anti-Sm antibodies in diagnosis and monitoring of systemic lupus erythematosus (SLE) and their ability to predict lupus flares compared with that of anti-dsDNA antibody and complement (C3) assays.

    Methods: Autoantibodies against Smith antigen (Sm) and double-stranded DNA (dsDNA) in sera from SLE (n=232), myositis (n=26), systemic sclerosis (n=81), Sjögren's syndrome (n=88), and rheumatoid arthritis patients (n=165) and healthy donors (n=400) were determined by using enzyme-linked immunosorbent assays (both from Euroimmun). New thresholds for both autoantibodies were calculated by receiver operating characteristics (ROC) curve analysis. Read More

    Type I interferon signature may influence the effect of belimumab on immunoglobulin levels, including rheumatoid factor in Sjögren's syndrome.
    Clin Exp Rheumatol 2017 Mar 8. Epub 2017 Mar 8.
    Department of Biological and Medical Sciences, Rheumatology Clinic, University of Udine, Italy.
    ****************************************************************************. Read More

    Inflammation in Sjögren's syndrome: Cause or consequence?
    Autoimmunity 2017 Feb 14:1-10. Epub 2017 Feb 14.
    a Department of Biochemistry , Faculty of Medicine, University of Porto , Porto , Portugal and.
    Sjögren's syndrome (SS) is an autoimmune disease most commonly characterized by ocular and oral dryness. Despite the high prevalence of SS, generation and perpetuation of this disease is still unclear in many aspects. Inflammation, nonetheless, seems to play a central role in this pathology especially in the form of Th-1, Th-2 and Th-17 cytokines release within different aspects, concentrations and connections involved in the maintenance of the syndrome. Read More

    Thymic hyperplasia associated with primary Sjogren's syndrome cured by thymectomy.
    J Thorac Dis 2017 Feb;9(2):E130-E132
    Department of Thoracic Surgery, the First Hospital of Jilin University, Changchun 130021, China.
    Thymus hyperplasia associated with Sjogren's syndrome is a rare morbid state. The present study described a 55-year-old woman who presented with a dryness of the oral cavity, and itchy eyes. Chest computed tomography identified a mass, measuring 4×2. Read More

    Elevated serum autoantibodies against co-inhibitory PD-1 facilitate T cell proliferation and correlate with disease activity in new-onset systemic lupus erythematosus patients.
    Arthritis Res Ther 2017 Mar 9;19(1):52. Epub 2017 Mar 9.
    Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, No. 197 Ruijin Second Road, Shanghai, 200025, China.
    Background: Programmed cell death protein 1 (PD-1) plays an important role in immune response regulation as a co-inhibitory signal during T cell activation. However, there is little known about the serum autoantibody profile of PD-1 in systemic lupus erythematosus (SLE), a disease characterized by the breakdown of immune tolerance to self-antigens and an excessive production of autoantibodies. Thus, we aim to investigate the serum levels and function of anti-PD-1 in patients with new-onset SLE. Read More

    Optic Neuropathy Associated with Primary Sjögren's Syndrome: A Case Series.
    Optom Vis Sci 2017 Apr;94(4):519-526
    *MD †MD, PhD Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Gyeonggi-do, Republic of Korea (all authors).
    Purpose: To determine the diverse clinical features of optic neuropathy associated with primary Sjögren's syndrome in Korean patients.

    Methods: Five women with acute and/or chronic optic neuropathy who were diagnosed as primary Sjögren's syndrome were retrospectively evaluated. Primary Sjögren's syndrome was diagnosed by signs and symptoms of keratoconjunctivitis sicca, positive serum anti-Ro/SSA and/or anti-La/SSB antibodies, and/or minor salivary gland biopsy. Read More

    Advances in Mouse Models of Sjögren's Syndrome.
    Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2017 Feb;39(1):156-161
    Department of Traditional Chinese Medicine,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.
    Sjögren's syndrome (SS) is a common chronic systemic autoimmune disease;however,its pathogenic mechanisms remain unclear. Proper mouse models of SS are essential for experiments. This article summarizes the recent advances in spontaneous mouse models of SS,genetically engineered mouse models of SS,and experimentally induced mouse models of SS. Read More

    Cigarette smoking and the risk of primary Sjögren's syndrome: a nested case control study.
    Arthritis Res Ther 2017 Mar 7;19(1):50. Epub 2017 Mar 7.
    Department of Clinical sciences, Malmö, Rheumatology, Lund University, Malmö, Sweden.
    Background: Smoking is reported to affect the risk of a number of chronic disorders, including rheumatic diseases. Previous cross-sectional studies have shown a lower frequency of smoking in patients with primary Sjögren's syndrome (pSS). The aim of this study was to investigate the impact of smoking and socioeconomic status on the risk of subsequent diagnosis of pSS in a nested case-control study. Read More

    Analysis of novel Sjogren's syndrome autoantibodies in patients with dry eyes.
    BMC Ophthalmol 2017 Mar 7;17(1):20. Epub 2017 Mar 7.
    Department of Medicine, SUNY at Buffalo School of Medicine, Buffalo, NY, USA.
    Background: Dry eye is a common problem in Ophthalmology and may occur for many reasons including Sjogren's syndrome (SS). Recent studies have identified autoantibodies, anti-salivary gland protein 1 (SP1), anti-carbonic anhydrase 6 (CA6) and anti-parotid secretory protein (PSP), which occur early in the course of SS. The current studies were designed to evaluate how many patients with idiopathic dry eye and no evidence of systemic diseases from a dry eye practice have these autoantibodies. Read More

    Primary Sjögren's syndrome and bronchiolitis. An unusual association.
    Reumatol Clin 2017 Mar 3. Epub 2017 Mar 3.
    Departamento de Neumología, Hospital Regional de Alta Especialidad de la Península de Yucatán, Mérida, Yucatán, México. Electronic address:
    Sjögren's syndrome (SS) is a systemic autoimmune disease that mainly affects the exocrine glands. It has been reported that the association between SS and respiratory involvement occurs in 11% and 45% of cases, and can be the initial manifestation of the disease. The diagnosis is based on the integration of clinical, immunological and histopathological criteria. Read More

    Intrathecal Baclofen Therapy for the Treatment of Spasticity in Sjögren-Larsson Syndrome.
    J Child Neurol 2017 Jan 6;32(1):100-103. Epub 2016 Oct 6.
    1 Division of Pediatric Neurosurgery, Department of Neurosurgery, NYU Langone Medical Center, NY, USA.
    Intrathecal baclofen therapy is widely accepted as a treatment option for patients with severe spasticity. The current treatment of spasticity in patients with Sjögren-Larsson syndrome is largely symptomatic, given that no effective causal therapy treatments are available. We report the outcome of 2 patients with Sjögren-Larsson syndrome who had pump implantation for intrathecal baclofen. Read More

    An unexpected role for the yeast nucleotide exchange factor Sil1 as a reductant acting on the molecular chaperone BiP.
    Elife 2017 Mar 3;6. Epub 2017 Mar 3.
    Department of Molecular Medicine, Cornell University, Ithaca, United States.
    Unfavorable redox conditions in the endoplasmic reticulum (ER) can decrease the capacity for protein secretion, altering vital cell functions. While systems to manage reductive stress are well-established, how cells cope with an overly oxidizing ER remains largely undefined. In previous work (Wang et al. Read More

    The clinical and pathological characteristics of nephropathies in connective tissue diseases in the Japan Renal Biopsy Registry (J-RBR).
    Clin Exp Nephrol 2017 Mar 2. Epub 2017 Mar 2.
    Division of Nephrology, Kanazawa Medical University School of Medicine, Uchinada, Japan.
    Background: In connective tissue diseases, a wide variety of glomerular, tubulointerstitial, and vascular lesions of the kidney are observed. Nonetheless, recent information is limited regarding renal lesions in connective tissue diseases, except in systemic lupus erythematosus (SLE).

    Methods: In this study, we used a nationwide database of biopsy-confirmed renal diseases in Japan (J-RBR) (UMIN000000618). Read More

    Time-course of ultrasound abnormalities of major salivary glands in suspected Sjögren's syndrome.
    Joint Bone Spine 2017 Feb 27. Epub 2017 Feb 27.
    Rheumatology Unit, Hôpital de la Cavale Blanche, Brest 29609, France; INSERM U1227, Université de Brest, LabEx IGO, 29609 Brest, France.
    Objective: To evaluate whether major salivary-gland ultrasonography (SGUS) abnormalities change over time in patients with suspected primary Sjögren's syndrome (pSS) during the natural course of the disease.

    Methods: We studied patients with suspected pSS who were included in the Brittany cohort of suspected pSS and underwent SGUS at least twice, as part of the routine diagnostic workup done at baseline then at least 1 year later as an additional investigation deemed appropriate by the physician. The evaluation criteria were the semi-quantitative SGUS score (0-4) for each parotid and submandibular gland, the highest SGUS score among the four glands, and the sum of SGUS scores for the four glands. Read More

    Sex differential association of dermatomyositis with Sjögren syndrome.
    CMAJ 2017 Feb;189(5):E187-E193
    Department of Internal Medicine (Tseng), Kaohsiung Municipal Ta-Tung Hospital; Department of Kinesiology (Chang), Health and Leisure Studies, National University of Kaohsiung; Division of Rheumatology (Tsai, Ou, Wu, Sung, Yen), Department of Internal Medicine, Kaohsiung Medical University Hospital; Graduate Institute of Medicine (Sung, Yen), College of Medicine, Kaohsiung Medical University; Division of Endocrinology and Metabolism, Department of Internal Medicine (Hsieh), Changhua Christian Hospital, Changhua, Taiwan; Graduate Institute of Integrated Medicine (Hsieh), China Medical University, Taichung, Taiwan
    Background: Although dermatomyositis and Sjögren syndrome share serologic autoantibodies and genetic polymorphisms, population data about the incidence of Sjögren syndrome in patients with dermatomyositis is unavailable. We performed a nationwide cohort study to explore the potential relation between dermatomyositis and Sjögren syndrome and, if an association exists, to elucidate whether it varies by sex.

    Methods: We identified all patients with newly diagnosed dermatomyositis from the Registry of Catastrophic Illness Database in Taiwan between Jan. Read More

    Autoantibodies to box A of high mobility group box 1 in systemic lupus erythematosus.
    Clin Exp Immunol 2017 Feb 28. Epub 2017 Feb 28.
    Departments of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
    Autoantibodies to nuclear structures are a hallmark of systemic lupus erythematosus (SLE), including autoantibodies to nuclear protein high mobility group box 1 (HMGB1). HMGB1 consists of three separate domains: box A, box B and an acidic tail. Recombinant box A acts as a competitive antagonist for HMGB1 and might be an interesting treatment option in SLE. Read More

    Autologous serum eye drops for dry eye.
    Cochrane Database Syst Rev 2017 02 28;2:CD009327. Epub 2017 Feb 28.
    Wilmer Eye Institute, Johns Hopkins University School of Medicine, 327 Maumenee Bldg, 600 N. Wolfe St., Baltimore, Maryland, USA, 21287-9238.
    Background: Theoretically, autologous serum eye drops (AS) offer a potential advantage over traditional therapies on the assumption that AS not only serve as a lacrimal substitute to provide lubrication but contain other biochemical components that allow them to mimic natural tears more closely. Application of AS has gained popularity as second-line therapy for patients with dry eye. Published studies on this subject indicate that autologous serum could be an effective treatment for dry eye. Read More

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