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    Systemic interferon type I and type II signatures in primary Sjögren's syndrome reveal differences in biological disease activity.
    Rheumatology (Oxford) 2018 Feb 20. Epub 2018 Feb 20.
    Department of Immunology, Erasmus University Medical Centre, Rotterdam, The Netherlands.
    Objective: To assess the relationships between systemic IFN type I (IFN-I) and II (IFN-II) activity and disease manifestations in primary SS (pSS).

    Methods: RT-PCR of multiple IFN-induced genes followed by principal component analysis of whole blood RNA of 50 pSS patients was used to identify indicator genes of systemic IFN-I and IFN-II activities. Systemic IFN activation levels were analysed in two independent European cohorts (n = 86 and 55, respectively) and their relationships with clinical features were analysed. Read More

    Use of Autologous Serum Tears for the Treatment of Ocular Surface Disease from Patients with Systemic Autoimmune Diseases.
    Am J Ophthalmol 2018 Feb 19. Epub 2018 Feb 19.
    Department of Ophthalmology, Ocular Surface Center of Excellence, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine; Miami, FL. USA; Department Microbiology & Immunology, University of Miami Miller School of Medicine; Miami, FL. USA. Electronic address:
    Purpose: To describe the safety and efficacy of autologous serum tears (AST) in managing ocular surface disease resistant to conventional therapy in patients with systemic autoimmune disease(s).

    Design: Retrospective, interventional case series.

    Methods: Records of patients from 2009 to 2015 with systemic autoimmune disease treated with AST (20-50%) for chronic surface disease were analyzed. Read More

    Primary cutaneous amyloidosis associated with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome and Sjögren syndrome: A case report.
    Medicine (Baltimore) 2018 Feb;97(8):e0004
    Department of Hepatology, The First Hospital of Jilin University, Changchun, Jilin, China.
    Rationale: Primary cutaneous amyloidosis (PCA) is a localized skin disorder characterized by the abnormal deposition of amyloid in the extracellular matrix of the dermis. The association between PCA and other diseases, although rare, has been documented for various autoimmune diseases. PCA associated with autoimmune hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjögren syndrome (SS) has not been previously reported in the literature. Read More

    Proprotein convertase enzyme FURIN is upregulated in primary Sjögren's syndrome.
    Clin Exp Rheumatol 2018 Feb 21. Epub 2018 Feb 21.
    Department of Internal Medicine, Centre for Rheumatic Diseases, Tampere University Hospital; and Faculty of Medicine and Life Sciences, University of Tampere, Finland.
    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. Read More

    Serum soluble interleukin-2 receptor is a useful biomarker for disease activity but not for differential diagnosis in IgG4-related disease and primary Sjögren's syndrome adults from a defined population.
    Clin Exp Rheumatol 2018 Feb 15. Epub 2018 Feb 15.
    Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
    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. Read More

    Immune response against the coiled coil domain of Sjögren's syndrome associated autoantigen Ro52 induces salivary gland dysfunction.
    Clin Exp Rheumatol 2018 Jan 31. Epub 2018 Jan 31.
    Arthritis and Clinical Immunology Program, Oklahoma Medical Research Foundation, Oklahoma City, USA.
    Objectives: The structural domains of Ro52, termed the RING, B-box, coiled coil (CC) and B30.2/SPRY are targets of anti-Ro52 in multiple autoimmune disorders. In Sjögren's syndrome patients, the presence of anti-Ro52 is associated with higher disease severity, and in mice, they induce salivary gland hypofunction. Read More

    Autoantibodies against the Immunoglobulin-Binding Region of Ro52 Link its Autoantigenicity with Pathogen Neutralization.
    Sci Rep 2018 Feb 20;8(1):3345. Epub 2018 Feb 20.
    Sjögren's Syndrome and Salivary Gland Dysfunction Unit, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD, 20892, USA.
    Ro52/TRIM21 plays a key role in antibody-dependent pathogen neutralization and is a major autoantigen in systemic lupus erythematosus, Sjögren's syndrome (SS), and other autoimmune diseases. Here we evaluated immunoreactivity against Ro52-related molecules in SS and healthy volunteers. Although most proteins examined were not antigenic, several TRIM paralogs, including TRIM22, and TRIM38, showed sporadic immunoreactivity in SS. Read More

    Biomarkers for Sjögren's syndrome.
    Biomark Med 2018 Feb 20. Epub 2018 Feb 20.
    Rheumatology Unit, Department of Clinical & Experimental Medicine, University of Pisa, 56126, Italy.
    Sjögren's syndrome (pSS) is a complex and heterogeneous disorder characterized by different clinical subsets. Recently, great efforts have been made searching for reliable biomarkers able to ameliorate the diagnostic algorithm and the prognostic stratification of pSS patients and ultimately allowing the scientific community to address some of the unmet needs for the disease. In this review, we have summarized the state of the art of 'traditional' widely acknowledged clinical, serological and histologic biomarkers for pSS with the aim of highlighting their relevance and limitations in clinical practice. Read More

    Impact of sleep quality on clinical features of primary Sjögren's syndrome.
    Korean J Intern Med 2018 Feb 20. Epub 2018 Feb 20.
    Department of Internal Medicine, Section of Dentistry, Seoul National University Bundang Hospital, Seongnam, Korea.
    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]). Read More

    Evidence for Alternate Modes of B cell Activation Involving Fab Acquired-N-Glycosylations in Antibody Secreting Cells Infiltrating the Labial Salivary Glands of Sjögren's Syndrome Patients.
    Arthritis Rheumatol 2018 Feb 18. Epub 2018 Feb 18.
    University of Oklahoma Health Sciences Center, Oklahoma City, OK.
    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. V-regions were amplified by RT-PCR, sequenced, and analyzed for incidence of N-glycosylation and selection pressure, then expressed as the native mAbs, or mutant mAbs lacking the acN-glyc for specificity testing. Read More

    Audiological Profile in Primary Sjögren's Syndrome in a Tertiary Care Setting and its Clinical Implications.
    Indian J Otolaryngol Head Neck Surg 2018 Mar 17;70(1):59-65. Epub 2015 Dec 17.
    1Department of ENT, Christian Medical College, Vellore, India.
    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. Read More

    Diminished CXCR5 expression in peripheral blood of patients with Sjögren's syndrome may relate to both genotype and salivary gland homing.
    Clin Exp Immunol 2018 Feb 17. Epub 2018 Feb 17.
    Rheumatology Unit, Department of Medicine, the Karolinska Institute, Karolinska University Hospital, Stockholm, Sweden.
    Genetic investigations of Sjögren's syndrome (SS) have identified a susceptibility locus at p23.3 of chromosome 11, which contains the CXCR5 gene. CXCR5 is a chemokine receptor expressed on B and T cell subsets, and binds the chemotactic ligand CXCL13. Read More

    [Prospective, monocentric, uncontrolled study of efficacy, tolerance and adherence of cyclosporin 0.1 % for severe dry eye syndrome].
    J Fr Ophtalmol 2018 Feb 12. Epub 2018 Feb 12.
    Service d'ophtalmologie, hôpital Édouard-Herriot, Lyon, France; Université Claude-Bernard Lyon I, Villeurbanne, France. Electronic address:
    Purpose: To evaluate the efficacy, tolerability and treatment adherence of Ikervis(Santen, SAS) (ciclosporine 0.1 %) for first line therapy or following treatment with Restasis(Allergan, Inc.) (ciclosporine 0. Read More

    Survival of adults with systemic autoimmune rheumatic diseases and pulmonary arterial hypertension after lung transplantation.
    Rheumatology (Oxford) 2018 Feb 13. Epub 2018 Feb 13.
    Department of Medicine, Columbia University Medical Center, New York, NY.
    Objectives: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in adults with systemic autoimmune rheumatic diseases (ARDs). The aim of this study was to determine whether adults with ARDs and PAH on right-sided heart catheterization (ARD-PAH) have increased mortality following lung transplantation compared with those with PAH not due to an ARD.

    Methods: We conducted a retrospective cohort study of 93 adults with ARD-PAH and 222 adults with PAH who underwent lung transplantation in the USA between 4 May 2005 and 9 March 2015 using data from the United Network for Organ Sharing. Read More

    Circulating small non-coding RNAs reflect IFN status and B cell hyperactivity in patients with primary Sjögren's syndrome.
    PLoS One 2018 15;13(2):e0193157. Epub 2018 Feb 15.
    Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.
    Background: Considering the important role of miRNAs in the regulation of post-transcriptional expression of target genes, we investigated circulating small non-coding RNAs (snc)RNA levels in patients with primary Sjögren's syndrome (pSS). In addition we assessed if serum sncRNA levels can be used to differentiate patients with specific disease features.

    Methods: Serum RNA was isolated from 37 pSS patients as well as 21 patients with incomplete Sjögren's Syndrome (iSS) and 17 healthy controls (HC) allocated to two independent cohorts: discovery and validation. Read More

    A spatial gradient of bacterial diversity in the human oral cavity shaped by salivary flow.
    Nat Commun 2018 Feb 14;9(1):681. Epub 2018 Feb 14.
    Department of Microbiology and Immunology, Stanford University School of Medicine, Stanford, CA, 94305, USA.
    Spatial and temporal patterns in microbial communities provide insights into the forces that shape them, their functions and roles in health and disease. Here, we used spatial and ecological statistics to analyze the role that saliva plays in structuring bacterial communities of the human mouth using >9000 dental and mucosal samples. We show that regardless of tissue type (teeth, alveolar mucosa, keratinized gingiva, or buccal mucosa), surface-associated bacterial communities vary along an ecological gradient from the front to the back of the mouth, and that on exposed tooth surfaces, the gradient is pronounced on lingual compared to buccal surfaces. Read More

    Validation of the ACR-EULAR criteria for primary Sjögren's syndrome in a Dutch prospective diagnostic cohort.
    Rheumatology (Oxford) 2018 Feb 12. Epub 2018 Feb 12.
    Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.
    Objectives: To validate the ACR-EULAR classification criteria for primary SS (pSS), and compare them to the American-European Consensus Group (AECG) and ACR criteria in a Dutch prospective diagnostic cohort.

    Methods: Consecutive patients (n = 129) referred for suspicion of pSS underwent a multidisciplinary evaluation, including a labial and/or parotid gland biopsy. Patients with an incomplete work-up (n = 8) or associated systemic auto-immune disease (n = 7) were excluded. Read More

    A Method for the Measurement of Salivary Gland Function in Mice.
    J Vis Exp 2018 Jan 25(131). Epub 2018 Jan 25.
    Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation;
    Patients with Sjögren's syndrome, an autoimmune disease affecting the exocrine glands, develop salivary gland inflammation and have reduced saliva production. Similarly, saliva production is severely compromised in patients receiving radiation treatment for head and neck cancers. Rodent models, developed to mimic these clinical conditions, facilitate an understanding of the disease pathogenesis and allow for the development of new therapeutic strategies. Read More

    Comparison of ESSDAI and ClinESSDAI in potential optimisation of trial outcomes in primary Sjögren's syndrome: examination of data from the UK Primary Sjögren's Syndrome Registry.
    Swiss Med Wkly 2018 Feb 14;148:w14588. Epub 2018 Feb 14.
    , UNITED KINGDOM.
    Objectives: To assess the use of the Clinical EULAR Sjögren's Syndrome Disease Activity Index (ClinESSDAI), a version of the ESSDAI without the biological domain, for assessing potential eligibility and outcomes for clinical trials in patients with primary Sjögren's syndrome (pSS), according to the new ACR-EULAR classification criteria, from the UK Primary Sjögren's Syndrome Registry (UKPSSR).

    Methods: A total of 665 patients from the UKPSSR cohort were analysed at their time of inclusion in the registry. ESSDAI and ClinESSDAI were calculated for each patient. Read More

    LC-MS-based serum metabolomics reveals a distinctive signature in patients with rheumatoid arthritis.
    Clin Rheumatol 2018 Feb 13. Epub 2018 Feb 13.
    Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, 300 Guangzhou Road, Nanjing, 210029, China.
    Metabolomics has been applied to explore altered metabolite profiles in disease and identify unique metabolic signatures in recent years. We aim to characterize the metabolic profile of rheumatoid arthritis patients and explore its underlying pathological processes using metabolomics approach. Serum samples from 30 rheumatoid arthritis (RA) patients, 30 primary Sjogren's syndrome (pSS) patients, and 32 healthy controls (HC) were collected. Read More

    Topical ocular 0.1% cyclosporine A cationic emulsion in dry eye disease patients with severe keratitis: experience through the French early-access program.
    Clin Ophthalmol 2018 5;12:289-299. Epub 2018 Feb 5.
    Research Team S12, Quinze-Vingts National Ophthalmology Hospital.
    Purpose: The objective of this study was to report the evaluation of efficacy and safety of cyclosporine A cationic emulsion (CsA CE) 0.1% for the treatment of severe keratitis in adults with dry eye disease (DED) in a French early-access program.

    Methods: Patients with DED and severe keratitis (corneal fluorescein staining [CFS] score of 3-5 on the Oxford scale and/or the presence of corneal lesions [filaments or ulcers]) were enrolled in a compassionate use program (Authorization for Temporary Use [ATU]) for once-daily CsA CE, which was approved by French health authorities prior to its registration. Read More

    A systematic review of the effects of acupuncture on xerostomia and hyposalivation.
    BMC Complement Altern Med 2018 Feb 13;18(1):57. Epub 2018 Feb 13.
    Department of Oral Biochemistry, Academic Centre for Dentistry Amsterdam (ACTA), room 12N-37, Gustav Mahlerlaan 3004, 1081 LA, Amsterdam, The Netherlands.
    Background: Saliva is fundamental to our oral health and our well-being. Many factors can impair saliva secretion, such as adverse effects of prescribed medication, auto-immune diseases (for example Sjögren's syndrome) and radiotherapy for head and neck cancers. Several studies have suggested a positive effect of acupuncture on oral dryness. Read More

    Sjögren-Like Lacrimal Keratoconjunctivitis in Germ-Free Mice.
    Int J Mol Sci 2018 Feb 13;19(2). Epub 2018 Feb 13.
    Ocular Surface Center, Department of Ophthalmology, Cullen Eye Institute, Baylor College of Medicine, Houston, TX 77030, USA.
    Commensal bacteria play an important role in the formation of the immune system but their role in the maintenance of immune homeostasis at the ocular surface and lacrimal gland remains poorly understood. This study investigated the eye and lacrimal gland phenotype in germ-free and conventional C57BL/6J mice. Our results showed that germ-free mice had significantly greater corneal barrier disruption, greater goblet cell loss, and greater total inflammatory cell and CD4⁺ T cell infiltration within the lacrimal gland compared to the conventionally housed group. Read More

    Glucocorticoid receptor expression in resident and hematopoietic cells in IgG4-related disease.
    Mod Pathol 2018 Feb 12. Epub 2018 Feb 12.
    Department of Nephrology, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan.
    Immunoglobulin G4-related disease is a rare immune-mediated disease characterized by the infiltration of IgG4-positive plasma cells and unique storiform fibrosis of multiple organs. The majority of IgG4-related disease patients respond to glucocorticoids, yet the precise mechanism of their action remains unclear. Pathological sections of the submaxillary gland, kidney, and retroperitoneum from 20 patients in total diagnosed with IgG4-related disease were analyzed for glucocorticoid receptor expression and the cell types expressing glucocorticoid receptor. Read More

    Identification of a novel autoantibody against self-vimentin specific in secondary Sjögren's syndrome.
    Arthritis Res Ther 2018 Feb 12;20(1):30. Epub 2018 Feb 12.
    Department of Rheumatology & Immunology, Peking University People's Hospital, 11 Xizhimen South Street, Beijing, 100044, China.
    Background: Sjögren's syndrome (SS) is a primary autoimmune disease (pSS) or secondarily associated with other autoimmune diseases (sSS). The mechanisms underlying immune dysregulation in this syndrome remain unknown, and clinically it is difficult to diagnose owing to a lack of specific biomarkers.

    Methods: We extracted immunoglobulins (Igs) from the sera of patients with sSS associated with rheumatoid arthritis (RA) and used them to screen a phage display library of peptides with random sequences. Read More

    Pilocarpine and Artificial Saliva for the Treatment of Xerostomia and Xerophthalmia of Sjögren's Syndrome: A double blind control trial.
    Br J Dermatol 2018 Feb 12. Epub 2018 Feb 12.
    Department of Dermatology, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
    Background: Sjögren's Syndrome (SS) is associated with xerostomia and xerophthalmia. Pilocarpine stimulates the secretion of saliva.

    Objectives: Investigate and compare the efficacy of pilocarpine and artificial saliva as symptomatic treatments for xerostomia and xerophthalmia in patients with SS. Read More

    The imprint of salivary secretion in autoimmune disorders and related pathological conditions.
    Autoimmun Rev 2018 Feb 9. Epub 2018 Feb 9.
    Department of Pharmacology, Institute of New Drug Development, School of Medicine, Chonbuk National University, Jeonju, Republic of Korea. Electronic address:
    Xerostomia is a state of oral dryness associated with salivary gland dysfunction and is induced by stress, radiation and chemical therapy, various systemic and autoimmune diseases, and specific medications. Fluid secretion is interrupted by the stimulation of neurotransmitter-induced increase in cytosolic calcium ([Ca]) in salivary gland acinar cells, prompting the mobilization of ion channels and their transporters. Salivary fluid and protein secretion are principally dependent on parasympathetic and sympathetic nerves. Read More

    TCRαβCD3CD4CD8(double negative) T cells in autoimmunity.
    Autoimmun Rev 2018 Feb 9. Epub 2018 Feb 9.
    Division of Pediatric Rheumatology and Immunology, Children's Hospital Dresden, Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany; Department of Women's & Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, UK; Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK. Electronic address:
    TCRαβCD3CD4CD8"double negative" (DN) T cells comprise a small subset of mature peripheral T cells. The origin and function of DN T cells are somewhat unclear and discussed controversially. While DN T cells resemble a rare and heterogeneous T cell subpopulation in healthy individuals, numbers of TCRαβDN T cells are expanded in several inflammatory conditions, where they also exhibit distinct effector phenotypes and infiltrate inflamed tissues. Read More

    Anti-tubulin-α-1C autoantibody in systemic lupus erythematosus: a novel indicator of disease activity and vasculitis manifestations.
    Clin Rheumatol 2018 Feb 9. Epub 2018 Feb 9.
    Department of Rheumatology and Immunology, Peking University People's Hospital, 11 Xizhimen South St, Beijing, 100044, China.
    A variety of autoantibodies has been involved in the pathogenesis of systemic lupus erythematosus (SLE), some of which are well known and applied as disease biomarkers. This study aimed to determine the prevalence of a novel autoantibody, anti-tubulin-α-1C, in patients with SLE and investigate its clinical significance. Anti-tubulin-α-1C autoantibody levels were determined by enzyme-linked immunosorbent assay (ELISA) in 128 SLE patients, 38 primary Sjögren's syndrome (pSS) patients, and 106 healthy controls (HCs). Read More

    [Etiological profile of secondary Raynaud's phenomenon in an internal medicine department. About 121 patients].
    J Med Vasc 2018 Feb 20;43(1):29-35. Epub 2017 Dec 20.
    Service de médecine interne, La Rabta, Tunis, Tunisie.
    Introduction: Raynaud's phenomenon is a reversible episodic vasospastic disorder triggered by cold or emotion. Two types of Raynaud's phenomenon were distinguished: Raynaud's disease and secondary Raynaud's phenomenon. The purpose of this study was to determine the etiologic profile of secondary Raynaud's phenomenon in an internal medicine department. Read More

    The prognosis of pulmonary arterial hypertension associated with primary Sjögren's syndrome: a cohort study.
    Lupus 2018 Jan 1:961203318756287. Epub 2018 Jan 1.
    1 Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
    Pulmonary arterial hypertension (PAH) is a rare and severe complication of primary Sjögren's syndrome (pSS). Cohort studies indicate that the underlying diseases of PAH associated with connective tissue disease (CTD-PAH) in Asian countries are different from that in the United States and in Europe. This study investigated the clinical characteristics, survival, and prognostic factors of pSS-PAH in Chinese patients. Read More

    On chronic fatigue syndrome and nosological categories.
    Clin Rheumatol 2018 Feb 7. Epub 2018 Feb 7.
    Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, 5265601, Tel-Hashomer, Israel.
    Chronic fatigue syndrome (CFS) is a heterogeneous disease which presents with pronounced disabling fatigue, sleep disturbances, and cognitive impairment that negatively affects patients' functional capability. CFS remains a poorly defined entity and its etiology is still in question. CFS is neither a novel diagnosis nor a new medical condition. Read More

    B cells in the pathogenesis of primary Sjögren syndrome.
    Nat Rev Rheumatol 2018 Mar 8;14(3):133-145. Epub 2018 Feb 8.
    Department of Rheumatology, Université Paris-Sud, INSERM U1184: Centre for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpitaux Universitaires Paris-Sud Bicêtre, Le Kremlin-Bicêtre, France.
    Primary Sjögren syndrome (pSS) is a prototypical autoimmune disease. The involvement of B cells in the pathogenesis of pSS has long been suspected on the basis of clinical observations that include the presence of serum autoantibodies, hypergammaglobulinaemia, increased levels of free light chains and increased risk of B cell lymphoma. Moreover, the composition of the B cell subset is altered in pSS. Read More

    Salivary gland ultrasonography findings are associated with clinical, histological, and serologic features of Sjögren's syndrome.
    Scand J Rheumatol 2018 Feb 7:1-8. Epub 2018 Feb 7.
    b Department of Otolaryngology Head and Neck Surgery , Catholic University of Daegu School of Medicine , Daegu , Korea.
    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. Read More

    Clinical practice guideline for Sjögren's syndrome 2017.
    Mod Rheumatol 2018 Feb 7:1-74. Epub 2018 Feb 7.
    a Department of Internal Medicine, Faculty of Medicine , University of Tsukuba , Ibaraki , Japan.
    Objectives: To develop clinical practice guideline (CPG) for Sjögren's syndrome (SS) based on recently available clinical and therapeutic evidences.

    Methods: The CPG committee for SS was organized by the Research Team for Autoimmune Diseases, Research Program for Intractable Disease of the Ministry of Health, Labor and Welfare (MHLW), Japan. The committee completed a systematic review of evidences for several clinical questions and developed CPG for SS 2017 according to the procedure proposed by the Medical Information Network Distribution Service (Minds). Read More

    A mixed-methods study identifying key intervention targets to improve participation in daily living activities in primary Sjögren's syndrome patients.
    Arthritis Care Res (Hoboken) 2018 Feb 6. Epub 2018 Feb 6.
    Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK.
    Objective: Functional ability and participation in life situations are compromised in many primary Sjögren's syndrome (PSS) patients. This study aims to identify the key barriers and priorities to participation in daily living activities, in order to develop potential future interventions.

    Methods: Group concept mapping (GCM), a semi-quantitative, mixed-methods, approach was used to identify and structure ideas from UK PSS patients, adults living with a PSS patient (AHMs) and health care professionals (HCPs). Read More

    Targeting the enhanced ER stress response in Marinesco-Sjögren syndrome.
    J Neurol Sci 2018 Feb 9;385:49-56. Epub 2017 Dec 9.
    Department of Pediatrics, Developmental Biology, Tokyo Medical and Dental University, Tokyo, Japan. Electronic address:
    Background And Objective: Marinesco-Sjögren syndrome (MSS) is an autosomal recessive infantile-onset disorder characterized by cataracts, cerebellar ataxia, and progressive myopathy caused by mutation of SIL1. In mice, a defect in SIL1 causes endoplasmic reticulum (ER) chaperone dysfunction, leading to unfolded protein accumulation and increased ER stress. However, ER stress and the unfolded protein response (UPR) have not been investigated in MSS patient-derived cells. Read More

    Granulomatous diseases of the breast and axilla: radiological findings with pathological correlation.
    Insights Imaging 2018 Feb 5;9(1):59-71. Epub 2018 Feb 5.
    Department of Radiology, Mayo Clinic, Rochester, MN, USA.
    Objectives: This article reviews our experience and describes the literature findings of granulomatous diseases of the breast and axilla.

    Methods: After approval of the Institutional Review Board was obtained, the surgical pathological records from January 2000 to January 2017 were searched for the keyword granulomatous. Clinical, imaging and histology findings were reviewed by both a fellowship-trained radiologist and a breast-imaging consultant radiologist, reviewing 127 patients (age range, 32-86 years; 126 women and 1 man). Read More

    Immune Tolerance to Apoptotic Self Is Mediated Primarily by Regulatory B1a Cells.
    Front Immunol 2017 19;8:1952. Epub 2018 Jan 19.
    MRC Centre for Inflammation Research, Queen's Medical Research Institute, University of Edinburgh, Edinburgh, United Kingdom.
    The chronic autoimmune inflammatory diseases, systemic lupus erythematosus and Sjogren's syndrome, develop when tolerance to apoptotic cells (ACs) is lost. We have previously reported that this tolerance is maintained by innate-like, IL-10 secreting regulatory B cells. Two questions remained. Read More

    Increased risk of coronary heart disease among patients with primary Sjögren's syndrome: a nationwide population-based cohort study.
    Sci Rep 2018 Feb 2;8(1):2209. Epub 2018 Feb 2.
    Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
    To investigate the association between primary Sjögren's syndrome (pSS) and coronary heart disease (CHD), and the influence of medications for pSS patients on risk of CHD. The authors identified 4175 patients with a new diagnosis of pSS between 2002 and 2013 from the National Health Insurance Research database. The control-to-case ratio was 4:1. Read More

    Assessing health-related quality of life in primary Sjögren's syndrome-The PSS-QoL.
    Semin Arthritis Rheum 2017 Nov 27. Epub 2017 Nov 27.
    Division of Rheumatology and Immunology, Department of Internal Medicine, Medical University Graz, Auenbruggerplatz 15, 8036 Graz, Austria; Rheumatology Service, South Tyrolean Health Trust, Hospital of Bruneck, Spitalstraße 11, 39031 Bruneck, Italy.
    Objectives: To develop a questionnaire for the assessment of health-related quality of life (HRQL) in primary Sjögren's syndrome (PSS), and to test its psychometric properties.

    Methods: Based on the concepts of a previous qualitative study, a questionnaire for the assessment of HRQL in PSS (PSS-QoL) was developed. Psychometric testing of PSS-QoL was performed after revising the first draft with feedback of patients (n = 6) and clinicians (n = 4). Read More

    Social-economic analysis of patients with Sjogren's syndrome dry eye in East China: a cross-sectional study.
    BMC Ophthalmol 2018 Feb 2;18(1):23. Epub 2018 Feb 2.
    Department of Ophthalmology, Eye & ENT Hospital of Fudan University, No.83 Fenyang Road, Shanghai, 200031, China.
    Background: Sjogren's syndrome is the leading cause for aqueous tear-deficiency dry eye. Little is known regarding the relationship between Sjogren's syndrome dry eye (SSDE) and patients' medical expenditure, clinical severity and psychological status changes.

    Methods: Thirty-four SSDE patients and thirty non-Sjogren's syndrome dry eye (non-SSDE) subjects were enrolled. Read More

    Sjögren's syndrome manifesting as clinicopathological features of TAFRO syndrome: A case report.
    Medicine (Baltimore) 2017 Dec;96(50):e9220
    Department of Hematology and Immunology, Kanazawa Medical University.
    Rationale: TAFRO syndrome is a newly proposed disorder that manifests as thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. In this report, we describe the development of severe TAFRO syndrome-like systemic symptoms during the clinical course of juvenile-onset Sjögren's syndrome in a 32-year-old woman.

    Patient Concerns: The patient was admitted due to dyspnea, fever, polyarthralgia, and generalized edema. Read More

    Health-related quality of life and psychological status of women with primary Sjögren's syndrome: A cross-sectional study of 304 Chinese patients.
    Medicine (Baltimore) 2017 Dec;96(50):e9208
    Department of Traditional Chinese Medicine, Translational Medicine Center, Peking Union Medical College Hospital, Chinese Academy of Medical Science.
    Patients with primary Sjögren syndrome (pSS) always suffer from dryness, pain, and fatigue caused by the involvement of multiple different systems or organs. The uncomfortable disease symptoms, the consequent disability, and the side effects of therapeutic drugs decrease the quality of life and lead to emotional problems. We investigated the health-related quality of life and psychological status of a large cohort of women patients with pSS and associated factors. Read More

    Primary Sjögren syndrome that initially presented with repeated hypergammaglobulinemic purpura after prolonged sitting: A case report.
    Medicine (Baltimore) 2017 Dec;96(50):e9187
    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. Read More

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