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    Unique glandular ex-vivo Th1 and Th17 receptor motifs in Sjögren's syndrome patients using single-cell analysis.
    Clin Immunol 2018 Apr 18. Epub 2018 Apr 18.
    Department of Infectious Diseases and Pathology, College of Veterinary Medicine, University of Florida, Gainesville, Florida, USA; Department of Oral Biology, USA; Center of Orphaned Autoimmune Diseases, University of Florida, Gainesville, Florida, USA. Electronic address:
    Primary Sjögren's syndrome (pSS) is an autoimmune disease in which the underlying cause has yet to be elucidated. The main objective of this study was to determine the T cell receptor (TCR) repertoires of individual infiltrating T helper (Th)-1 and 17 cells of pSS patients using single-cell analysis. Single-cell analysis of ex-vivo infiltrating T cells demonstrated that pSS patients had higher frequencies of activated Th17 cells. Read More

    Diagnostic Value of Vitamin D Status and Bone Turnover Markers in Rheumatoid Arthritis Complicated by Osteoporosis.
    Ann Clin Lab Sci 2018 Mar;48(2):197-204
    Department of Clinical Laboratory, Jiangxi Province Key Laboratory of Laboratory Medicine, the Second Affiliated Hospital of Nanchang University, Nanchang, Jiangxi, China.
    Objective: To research the diagnostic performance of clinical potential bone turnover indexes in rheumatoid arthritis (RA) complicated with osteoporosis (OP).

    Methods: This study involved 87 RA patients, 48 with OP, and 39 without OP, and 204 non-RA control patients, including those with systemic lupus erythematosus, ankylosing spondylitis, primary Sjogren's syndrome, systemic sclerosis, and healthy patients. The levels of 25-hydroxyvitamin D [25(OH)D], β-crosslaps (β-CROSSL), parathyroid hormone (PTH) were measured by electrochemiluminescence (ECLIA), and the level of bone alkaline phosphatase (BALP) was measured by lectin affinity method. Read More

    TBK1: A key regulator and potential treatment target for interferon positive Sjögren's syndrome, systemic lupus erythematosus and systemic sclerosis.
    J Autoimmun 2018 Apr 16. Epub 2018 Apr 16.
    Department of Immunology, Erasmus University Medical Centre, 3015 CN, Rotterdam, The Netherlands. Electronic address:
    Objective: Upregulation of type I interferons (IFN-I) is a hallmark of systemic autoimmune diseases like primary Sjögren's syndrome (pSS), systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Expression of IFN-I is induced by three different receptor families: Toll-like receptors (TLRs), RIG-like receptors (RLRs) and DNA-sensing receptors (DSRs). TANK-binding kinase (TBK1) is an important signaling hub downstream of RLRs and DSRs. Read More

    Musculoskeletal Manifestations of Non-RA Connective Tissue Diseases: Scleroderma, Systemic Lupus Erythematosus, Still's Disease, Dermatomyositis/Polymyositis, Sjögren's Syndrome, and Mixed Connective Tissue Disease.
    Semin Musculoskelet Radiol 2018 Apr 19;22(2):166-179. Epub 2018 Apr 19.
    Department of Musculoskeletal Radiology, University Hospital of Lille, Lille Cedex, France.
    The most common systemic rheumatologic conditions are connective tissue diseases (including rheumatoid arthritis [RA]) followed by spondyloarthropathy. With the advent of biotherapies and imaging biomarkers, development in the imaging of RA and spondyloarthropathies has received substantial attention in the literature. This article details the various musculoskeletal imaging features of the other connective tissue diseases such as scleroderma and progressive systemic sclerosis, systemic lupus erythematosus, Still's disease, dermatomyositis and polymyositis, Sjögren's syndrome, and mixed connective tissue disease. Read More

    Atherosclerosis and autoimmunity: a growing relationship.
    Int J Rheum Dis 2018 Apr 19. Epub 2018 Apr 19.
    Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran.
    Atherosclerosis is regarded as one of the leading causes of mortality and morbidity in the world. Nowadays, it seems that atherosclerosis cannot be defined merely through the Framingham traditional risk factors and that autoimmunity settings exert a remarkable role in its mechanobiology. Individuals with autoimmune disorders show enhanced occurrence of cardiovascular complications and subclinical atherosclerosis. Read More

    A patient presenting with isolated hematuria and renal dysfunction as rare manifestation of cryoglobulinemic glomerulonephritis in the course of autoimmune diseases including Sjögren's syndrome.
    CEN Case Rep 2018 Apr 18. Epub 2018 Apr 18.
    Department of Internal Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, Japan.
    Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren's syndrome are most frequent condition among non-hepatitis C virus-related condition. Its typical renal manifestation shows high amount of proteinuria with microscopic hematuria and renal insufficiency. Read More

    Clinical characteristics and risk factors for overlapping rheumatoid arthritis and Sjögren's syndrome.
    Sci Rep 2018 Apr 18;8(1):6180. Epub 2018 Apr 18.
    Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Dongcheng, China.
    This study investigated the clinical characteristics and risk factors for overlapping rheumatoid arthritis and Sjögren's syndrome (RA/SS). Patients with RA/SS in Peking Union Medical College Hospital from January 2012 to January 2017 were retrospectively analysed and compared to those of sex- and age-matched RA or SS controls. Logistic regression analysis was used to identify risk factors. Read More

    Sex differences in pulmonary arterial hypertension: role of infection and autoimmunity in the pathogenesis of disease.
    Biol Sex Differ 2018 Apr 18;9(1):15. Epub 2018 Apr 18.
    Department of Cardiovascular Medicine, Mayo Clinic, Jacksonville, FL, USA.
    Registry data worldwide indicate an overall female predominance for pulmonary arterial hypertension (PAH) of 2-4 over men. Genetic predisposition accounts for only 1-5% of PAH cases, while autoimmune diseases and infections are closely linked to PAH. Idiopathic PAH may include patients with undiagnosed autoimmune diseases based on the relatively high presence of autoantibodies in this group. Read More

    Ectopic germinal centre-like structures in minor salivary gland biopsy predict lymphoma occurrence in patients with primary Sjögren syndrome.
    Arthritis Rheumatol 2018 Apr 18. Epub 2018 Apr 18.
    Department of Rheumatology, and Inserm UMR 1132 Lariboisière Fernand Widal Hospital AP-HP, France.
    Objective: The objective was to determine risk factors of pSS-associated lymphoma in a multicenter cohort of patients, including in analyzed variables the presence of including ectopic germinal centre-like structures in minor salivary gland biopsy (MSGB).

    Patients And Methods: 115 pSS-patients were included and their MSGB were retrospectively examined focusing on the presence of ectopic germinal centre-like structures. Epidemiological, clinical, biological, immunological and histological data were collected at pSS diagnosis. Read More

    Proximal Muscle Weakness With Overlying Hypokalemic Periodic Paralysis in Sjögren Syndrome: Report of 6 Cases.
    J Clin Rheumatol 2018 Apr 5. Epub 2018 Apr 5.
    Department of Neurology Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow, Uttar Pradesh, Department of Pathology Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow, Uttar Pradesh, India Department of Immunology Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow, Uttar Pradesh, India.

    The Association of Sjögren Syndrome and Autoimmune Thyroid Disorders.
    Front Endocrinol (Lausanne) 2018 3;9:121. Epub 2018 Apr 3.
    Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
    Sjögren's syndrome (SS) and autoimmune thyroid diseases (AITD) may frequently coexist in clinical practice, resulting in a complex overlapping disorder that represents a particular example of the expression of heterogeneity in patients with autoimmune disorders. Objective of this review was to describe the prevalence of the SS-AITD association in the most recent literature, exploring in particular to what extent the presence of AITD might influence the clinical expression of SS and . Moreover, we summarized some of the proposed genetic, biologic, and molecular mechanisms implied in the pathogenesis of AITD-SS association. Read More

    SIL1, the ER Hsp70 co-chaperone, plays a critical role in maintaining skeletal muscle proteostasis and physiology.
    Dis Model Mech 2018 Apr 9. Epub 2018 Apr 9.
    Dept. of Microbiology, Immunology, and Biochemistry, The University of Tennessee Health Science Center, Memphis, TN 38163, USA
    Mutations in , a co-factor for the endoplasmic reticular (ER) chaperone BiP, cause Marinesco-Sjögren syndrome (MSS), an autosomal recessive disorder. Using a mouse model, we characterized molecular aspects of the progressive myopathy associated with MSS. Proteomic-profiling of quadriceps at the onset of myopathy revealed that SIL1 deficiency affected multiple pathways critical to muscle physiology. Read More

    CP-25 Alleviates Experimental Sjögren's Syndrome Features in NOD/Ltj Mice and Modulates T Lymphocyte Subsets.
    Basic Clin Pharmacol Toxicol 2018 Apr 17. Epub 2018 Apr 17.
    Institute of Clinical Pharmacology, Anhui Medical University;Key Laboratory of Anti-inflammatory and Immune Medicine, Ministry of Education;Anhui Collaborative Innovation Center of Anti-inflammatory and Immune Medicine, Hefei, Anhui, 230032, China.
    Primary Sjögren's syndrome (pSS) is a chronic inflammatory autoimmune illness of the moisture-producing glands such as salivary glands that is characterized by various immune abnormalities. The aetiology of pSS remains unclear and there is no curative agent. In this study, we investigated the putative therapeutic effects on a NOD/Ltj mouse model of Sjögren's syndrome-like disorders of an ester derivative of paeoniflorin, Paeoniflorin-6'O-benzene (termed CP-25). Read More

    Salivary gland stem cells: A review of development, regeneration and cancer.
    Genesis 2018 Apr 16. Epub 2018 Apr 16.
    Program in Craniofacial Biology, Department of Cell and Tissue Biology, University of California, 513 Parnassus Avenue, San Francisco, CA, 94143, USA.
    Salivary glands are responsible for maintaining the health of the oral cavity and are routinely damaged by therapeutic radiation for head and neck cancer as well as by autoimmune diseases such as Sjögren's syndrome. Regenerative approaches based on the reactivation of endogenous stem cells or the transplant of exogenous stem cells hold substantial promise in restoring the structure and function of these organs to improve patient quality of life. However, these approaches have been hampered by a lack of knowledge on the identity of salivary stem cell populations and their regulators. Read More

    Associations of chronic urticaria with atopic and autoimmune comorbidities: a nationwide population-based study.
    Int J Dermatol 2018 Apr 16. Epub 2018 Apr 16.
    Department of Health Services Administration, China Medical University, Taichung, Taiwan.
    Background: Most cases of chronic urticaria (CU) are idiopathic. Circumstantial evidence suggests that some CU cases have an autoimmune pathogenesis. Previous research indicates that a substantial percentage of patients with CU have an atopic background. Read More

    The rheumatic disease-associated FAM167A-BLK locus encodes DIORA-1, a novel disordered protein highly expressed in bronchial epithelium and alveolar macrophages.
    Clin Exp Immunol 2018 Apr 17. Epub 2018 Apr 17.
    Unit of Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
    Objectives: Triggering of autoimmunity that leads to rheumatic disease has been suggested to depend on gene-environment interactions occurring in epithelial barriers and associated immune cells. Genetic studies have identified associations of the FAM167A-BLK locus with rheumatoid arthritis, SLE, and Sjögren's syndrome. While BLK (B lymphocyte kinase) has a well-established role in B cells, family with sequence similarity 167 member A (FAM167A) and its gene family remain uncharacterized. Read More

    Acquisition of N-Glycosylation Sites in Immunoglobulin Heavy Chain Genes During Local Expansion in Parotid Salivary Glands of Primary Sjögren Patients.
    Front Immunol 2018 12;9:491. Epub 2018 Mar 12.
    Department of Rheumatology and Clinical Immunology, University of Groningen and University Medical Center Groningen, Groningen, Netherlands.
    Previous studies revealed high incidence of acquired N-glycosylation sites acquired N-glycosylation sites in RNA transcripts encoding immunoglobulin heavy variable region (IGHV) 3 genes from parotid glands of primary Sjögren's syndrome (pSS) patients. In this study, next generation sequencing was used to study the extent of ac-Nglycs among clonally expanded cells from all IGVH families in the salivary glands of pSS patients. RNA was isolated from parotid gland biopsies of five pSS patients and five non-pSS sicca controls. Read More

    Deterioration in saliva quality in patients with Sjögren's syndrome: impact of decrease in salivary epidermal growth factor on the severity of intraoral manifestations.
    Inflamm Regen 2018 9;38. Epub 2018 Apr 9.
    1Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501 Japan.
    Background: Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, especially the salivary and lacrimal glands. As a result of salivary gland dysfunction, most patients with SS have xerostomia related to a reduced salivary flow rate. In addition to the discomfort due to xerostomia, dry mouth can cause various intraoral manifestations such as refractory stomatitis, ulcer, and atrophic changes in the oral mucosa and tongue, and the patient's quality of life (QoL) is severely impaired. Read More

    Experience of sleep disruption in primary Sjögren's syndrome: A focus group study.
    Br J Occup Ther 2018 Apr 11;81(4):218-226. Epub 2018 Jan 11.
    Professor of Applied Health Care Research, Department of Social Work, Education & Community Wellbeing, Northumbria University, UK.
    Introduction: Primary Sjögren's syndrome is the third most common systemic autoimmune rheumatic disease, following rheumatoid arthritis and systemic lupus erythematosus, and results in dryness, fatigue, discomfort and sleep disturbances. Sleep is relatively unexplored in primary Sjögren's syndrome. We investigated the experiences of sleep disturbances from the viewpoint of primary Sjögren's syndrome patients and their partners and explored the acceptability of cognitive behavioural therapy for insomnia. Read More

    Distal renal tubular acidosis in Sjögren's syndrome.
    Saudi J Kidney Dis Transpl 2018 Mar-Apr;29(2):470-473
    Department of Neurology, M. E. S. Medical College, Perinthalmanna, Kerala, India.
    Interstitial nephritis and immune complex-mediated glomerulonephritis are the two common renal manifestations of primary Sjögren's syndrome (SS). Here, we discuss three cases of primary SS where presenting manifestation was distal renal tubular acidosis. The possibility of an underlying autoimmune disorder should be considered in a patient presenting with distal tubular acidosis or recurrent hypokalemic periodic paralysis as treatment of primary disease improves the outcome of illness. Read More

    Systemic lupus erythematosus with Sjögren's syndrome and renal tubular acidosis presenting as nephrogenic diabetes insipidus.
    Saudi J Kidney Dis Transpl 2018 Mar-Apr;29(2):440-442
    Division of Internal Medicine, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India.
    Systemic lupus erythematosus (SLE) presenting as diabetes insipidus (DI) is a rare association; there is a case report of neurogenic DI in patients of SLE; however, SLE and nephrogenic DI has not been reported in literature. We present a case of SLE presenting as nephrogenic DI. We report a case who presented with DI (nephrogenic) and fulfilled criteria for SLE and Sjögren's syndrome with renal tubular acidosis. Read More

    The prevalence of meibomian gland dysfunction, tear film and ocular surface parameters in an Austrian dry eye clinic population.
    Acta Ophthalmol 2018 Apr 15. Epub 2018 Apr 15.
    Department of Ophthalmology, Medical University of Graz, Graz, Austria.
    Purpose: The purpose of this study was to assess the prevalence of meibomian gland dysfunction (MGD) and its association with tear film and ocular surface parameters in an Austrian clinical population of dry eye patients.

    Methods: The records of 1372 consecutive patients from a dry eye unit were analysed retrospectively. Symptoms and objective tear film and ocular surface parameters were evaluated. Read More

    How Are Ocular Signs and Symptoms of Dry Eye Associated with Depression in Women with and without Sjögren's Syndrome?
    Am J Ophthalmol 2018 Apr 12. Epub 2018 Apr 12.
    F.I. Proctor Foundation, Department of Ophthalmology, University of California, San Francisco, 95 Kirkham Street, San Francisco, CA 94143.
    Purpose: To determine whether ocular phenotypic features of keratoconjunctivitis sicca (KCS) and/or participant-reported symptoms of dry eye disease are associated with depression in women participants enrolled in the Sjögren's International Collaborative Clinical Alliance (SICCA).

    Design: Cross-sectional study.

    Methods: Women enrolled in the SICCA registry from 9 international research sites. Read More

    Transcription profiling of peripheral B cells in antibody-positive primary Sjögren's syndrome reveals upregulated expression of CX3CR1 and a type I and type II interferon signature.
    Scand J Immunol 2018 May;87(5):e12662
    Department of Medical Sciences, Rheumatology and Science for Life Laboratory, Uppsala University, Uppsala, Sweden.
    B cells play a key role in the pathogenesis of primary Sjögren's syndrome (pSS). The aim of this study was to analyse the transcriptome of CD19+ B cells from patients with pSS and healthy controls to decipher the B cell-specific contribution to pSS. RNA from purified CD19+ B cells from 12 anti-SSA antibody-positive untreated female patients with pSS and 20 healthy blood donors was subjected to whole transcriptome sequencing. Read More

    Sympathetic Nerve Hyperactivity in the Spleen: Causal for Nonpathogenic-Driven Chronic Immune-Mediated Inflammatory Diseases (IMIDs)?
    Int J Mol Sci 2018 Apr 13;19(4). Epub 2018 Apr 13.
    College of Arts and Sciences, Kent State University, Kent, OH 44304, USA.
    Immune-Mediated Inflammatory Diseases (IMIDs) is a descriptive term coined for an eclectic group of diseases or conditions that share common inflammatory pathways, and for which there is no definitive etiology. IMIDs affect the elderly most severely, with many older individuals having two or more IMIDs. These diseases include, but are not limited to, type-1 diabetes, obesity, hypertension, chronic pulmonary disease, coronary heart disease, inflammatory bowel disease, and autoimmunity, such as rheumatoid arthritis (RA), Sjőgren's syndrome, systemic lupus erythematosus, psoriasis, psoriatic arthritis, and multiple sclerosis. Read More

    Epigenetic alterations in primary Sjögren's syndrome - an overview.
    Clin Immunol 2018 Apr 9. Epub 2018 Apr 9.
    Department of Medical Sciences, Rheumatology and Science for Life Laboratory, Uppsala University, Uppsala, Sweden.
    Primary Sjögren's syndrome (pSS) is a chronic autoimmune rheumatic disease characterized by inflammation of exocrine glands, mainly salivary and lacrimal glands. In addition, pSS may affect multiple other organs resulting in systemic manifestations. Although the precise etiology of pSS remains elusive, pSS is considered to be a multi-factorial disease, where underlying genetic predisposition, environmental factors and epigenetic mechanisms contribute to disease development. Read More

    Excess Productivity Costs of Systemic Lupus Erythematosus, Systemic Sclerosis, and Sjogren's Syndrome: A General Population-Based Study.
    Arthritis Care Res (Hoboken) 2018 Apr 12. Epub 2018 Apr 12.
    University of British Colmbia, Faculty of Pharmaceutical Sciences, Arthritis Research Centre of, Canada.
    Objective: Determine excess productivity losses and costs of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and Sjogren's syndrome (SjS) at the population level.

    Methods: Administrative databases from the province of British Columbia, Canada, were used to establish population-based cohorts of SLE, SSc, and SjS, and matched comparison cohorts selected from the general population. Random samples from these cohorts were surveyed about time absent from paid and unpaid work, and working at reduced levels/efficiency (presenteeism), using validated labour questionnaires. Read More

    Salivary gland ultrasound is linked to the autoimmunity profile in patients with primary Sjögren's syndrome.
    J Int Med Res 2018 Jan 1:300060518767031. Epub 2018 Jan 1.
    1 Department of Rheumatology, 16483 Hospital General Universitario Gregorio Marañón , Madrid, Spain.
    Objective Salivary gland ultrasound (SGU) is a reliable technique for assessing the salivary glands in patients with primary Sjögren's syndrome (pSS). The aim of this study was to elucidate the relationship between SGU findings and autoimmunity in patients with pSS. Methods Patients with pSS underwent an SGU assessment. Read More

    Identification of novel biomarker as citrullinated inter-alpha-trypsin inhibitor heavy chain 4, specifically increased in sera with experimental and rheumatoid arthritis.
    Arthritis Res Ther 2018 Apr 10;20(1):66. Epub 2018 Apr 10.
    Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, 305-8575, Japan.
    Background: Anticitrullinated protein antibodies (ACPA) and citrullinated proteins play key roles in the pathogenesis of rheumatoid arthritis (RA). Many candidate citrullinated antigens have been identified in joints, but citrullinated proteins in sera are mostly uncertain in patients with RA. We explored the expression of citrullinated proteins in joints and sera of experimental arthritis, and we further investigated their specific expression correlated with the disease activity in patients with RA. Read More

    Autoantibodies Associated With Connective Tissue Diseases: What Meaning for Clinicians?
    Front Immunol 2018 26;9:541. Epub 2018 Mar 26.
    Department of Internal Medicine, Infectious Diseases, and Clinical Immunology, Reims Teaching Hospitals, Robert Debré Hospital, Reims, France.
    Connective tissue diseases (CTDs) such as systemic lupus erythematosus, systemic sclerosis, myositis, Sjögren's syndrome, and rheumatoid arthritis are systemic diseases which are often associated with a challenge in diagnosis. Autoantibodies (AAbs) can be detected in these diseases and help clinicians in their diagnosis. Actually, pathophysiology of these diseases is associated with the presence of antinuclear antibodies. Read More

    Meibomian Gland Dysfunction in Primary and Secondary Sjögren Syndrome.
    Ophthalmic Res 2018 Apr 6. Epub 2018 Apr 6.
    Schepens Eye Research Institute, Boston, Massachusetts, USA.
    Purpose: We hypothesized that women with primary (pSS) and secondary Sjögren syndrome (sSS; with systemic lupus erythematosus [SLE] or rheumatoid arthritis [RA]) have meibomian gland dysfunction (MGD). We sought to test our hypothesis.

    Methods: Subjects with pSS, sSS + SLE, sSS + RA, and non-SS-related MGD were recruited from the Sjögren's Syndrome Foundation or outpatient clinics at Tufts University School of Dental Medicine or Brigham and Women's Hospital. Read More

    Elastographic ultrasound: an additional image tool in Sjögren's syndrome.
    Int J Rheum Dis 2018 Apr 6. Epub 2018 Apr 6.
    Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
    Aim: To evaluate the stiffness of parotid and submandibular glands using elastography ultrasound and to correlate it with B-mode ultrasonographical, clinical and serological features, salivary profibrotic and inflammatory chemokines, and salivary gland fibrosis.

    Methods: We performed B-mode and elastography ultrasound of major salivary glands of 26 patients with primary Sjögren's syndrome. We registered the shear wave velocity (SWV) and correlated it with the morphologic ultrasonographic changes assessed by the Hocevar scale. Read More

    Characteristics and risk factors of pulmonary arterial hypertension in patients with primary Sjögren's syndrome.
    Int J Rheum Dis 2018 Apr 6. Epub 2018 Apr 6.
    Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
    Aim: To describe baseline characteristics of patients with primary Sjögren's syndrome (pSS) with right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH) and explore risk factors for PAH in pSS.

    Methods: This case-control study included consecutive patients hospitalized with pSS-PAH from 2007 to 2015, and pSS patients without PAH (in a 4 : 1 ratio) as controls. All patients fulfilled the 2002 American-European Consensus Group classification criteria for pSS-PAH was defined according to RHC-based European Society of Cardiology/European Respiratory Society guidelines. Read More

    Neurofilament light protein levels in cerebrospinal fluid predict long-term disability of Guillain-Barré syndrome: A pilot study.
    Acta Neurol Scand 2018 Apr 6. Epub 2018 Apr 6.
    Department of Clinical Neuroscience, Institute of Neuroscience and Physiology, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
    Objectives: Although the recovery from Guillain-Barré syndrome (GBS) is good in most patients, some develop permanent severe disability or even die. Early predictors would increase the likelihood to identify patients at risk for poor outcome at the acute stage, allowing them intensified therapeutic intervention.

    Materials And Method: Eighteen patients with a history of GBS 9-17 years ago were reassessed with scoring of neurological disability and quality of life assessment (QoL). Read More

    Association between memory B-cells and clinical and immunological features of primary Sjögren's syndrome and Sicca patients.
    Rheumatol Int 2018 Apr 6. Epub 2018 Apr 6.
    CEDOC, Chronic Diseases Research Center, Immunology, NOVA Medical School|FCM, Universidade Nova de Lisboa, Lisbon, Portugal.
    B-cells play a pivotal role in primary Sjögren's syndrome (pSS) pathogenesis. We aim to (1) evaluate the distribution of B-lymphocyte subpopulations in pSS and Sicca patients, (2) establish cut-off points that discriminate pSS from controls, (3) evaluate the association between memory B-cells and phenotypic features in pSS. We included 57 pSS patients, 68 Sicca and 24 healthy controls. Read More

    The role of genetics and epigenetics in rheumatic diseases: are they really a target to be aimed at?
    Rheumatol Int 2018 Apr 5. Epub 2018 Apr 5.
    Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, N15W7, Kita-Ku, Sapporo, 060-8638, Japan.
    To date, numerous genetic and epigenetic studies have been performed and provided a crucial step forward in our understanding of the pathogenesis of rheumatic diseases. However, most of the recent advances in the treatment of rheumatic diseases including biological therapies are not based on or even discrepant from these genetic and epigenetic findings. For example, tumor necrosis factor inhibitors are quite successful in the treatment of rheumatoid arthritis (RA), Behçet's disease (BD), ankylosing spondylitis (AS) and psoriatic arthritis (PsA) but not in that of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), Sjögren's syndrome (SS) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV), conversely, RA shares genetic backgrounds more with SLE, SSc, SS and AAV than BD, AS and PsA. Read More

    Routine and Advanced Diffusion Imaging Modules of the Salivary Glands.
    Neuroimaging Clin N Am 2018 May;28(2):245-254
    Department of Diagnostic Radiology, Mansoura University, Elgomheryia Street, Mansoura 35512, Egypt. Electronic address:
    This article reviews the role of routine and advanced diffusion imaging modules of the salivary glands. Routine and advanced diffusion imaging modules have a role in differentiation of malignant from benign salivary gland tumors, characterization of some benign salivary gland tumors, and staging of salivary gland cancer. The role of advanced diffusion modules in patients with salivary gland cancer after surgery, radiation therapy, or radioiodine therapy is discussed. Read More

    Imaging of Sjögren Syndrome and Immunoglobulin G4-Related Disease of the Salivary Glands.
    Neuroimaging Clin N Am 2018 May;28(2):183-197
    Department of Radiology, Jichi Medical University School of Medicine, 3311-1, Yakushiji, Shimotsuke, Tochigi 329-0498, Japan. Electronic address:
    The salivary glands are commonly affected in systemic autoimmune disease and diseases of unknown pathogenesis. Sjögren syndrome (SjS) can be affected by other systemic diseases. Immunoglobulin G4-related disease (IgG4-RD) commonly affects salivary glands. Read More

    [MALT lymphoma with t (X;14) (p11.2;q32) developing during the course of cutaneous leukocytoclastic angitis].
    Rinsho Ketsueki 2018 ;59(3):269-274
    Department of Internal Medicine, Division of Hematology and Oncology, St. Marianna University School of Medicine.
    A 73-year-old man with left parotid gland swelling over 2 months was referred to our hospital in March 201X. Purpura on the lower legs had been recurrent for >20 years. Biopsy of the parotid gland demonstrated diffuse infiltration of abnormal lymphocytes that were negative for CD10 and positive for CD19, CD20, and κ-chain. Read More

    Vasoactive Intestinal Peptide Protects Salivary Glands against Structural Injury and Secretory Dysfunction via IL-17A and AQP5 Regulation in a Model of Sjögren Syndrome.
    Neuroimmunomodulation 2018 Apr 4. Epub 2018 Apr 4.
    The First College of Clinical Medicine, Nanjing University of Chinese Medicine, Nanjing, China.
    Objective: Sjögren syndrome (SS) is an autoimmune disease involving exocrine glands. Currently, drugs that can improve both abnormal immunity and exocrine gland function are needed. The study aimed to investigate the effect and mechanism of vasoactive intestinal peptide (VIP) on the immune response and exocrine gland function in SS. Read More

    Combined classification system based on ACR/EULAR and ultrasonographic scores for improving the diagnosis of Sjögren's syndrome.
    PLoS One 2018 3;13(4):e0195113. Epub 2018 Apr 3.
    Department of Radiology and Cancer Biology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
    We retrospectively evaluated the effectiveness of combined use of salivary gland ultrasonography (US) and the 2016 American College of Rheumatology/European League Against Rheumatic Disease (ACR/EULAR) classification criteria for improving the diagnostic efficiency in patients with Sjögren's syndrome (SS). A US-based salivary gland disease grading system was developed using a cohort comprising 213 SS or non-SS patients who fulfilled the minimum requirements for classifying SS based on the American-European Consensus Group (AECG) and ACR criteria. Using 62 SS or non-SS patients from the 213 patients and who had also undergone all the 5 examinations needed for the ACR/EULAR classification, we compared the diagnostic accuracy of various combinations of the ACR/EULAR and US classifications for diagnosing SS, using the clinical diagnosis of SS by rheumatologists as the gold standard. Read More

    Sjögren's Syndrome Associated with Fanconi's Syndrome and Osteomalacia.
    Am J Case Rep 2018 Apr 3;19:392-396. Epub 2018 Apr 3.
    Department of Rheumatology, Unidad de Enfermedades Reumáticas y Autoinmunes [UNERA] (Unit of Rheumatology and Autoimmune Diseases), Guayaquil, Ecuador.
    BACKGROUND Sjögren's syndrome is a chronic inflammatory autoimmune disease, which is also known as sicca syndrome, due to the symptoms of dry eyes and dry mouth, and is associated with other connective tissue diseases and autoimmune diseases. Sjögren's syndrome can also be associated with renal involvement. Fanconi's syndrome is associated with impaired reabsorption in the proximal renal tubule associated with tubulointerstitial nephritis and is associated with renal tubular acidosis and hypophosphatemia. Read More

    Diagnostic value of screening enzyme immunoassays compared to indirect immunofluorescence for anti-nuclear antibodies in patients with systemic rheumatic diseases: A systematic review and meta-analysis.
    Semin Arthritis Rheum 2018 Mar 30. Epub 2018 Mar 30.
    Department of Preventive Medicine, College of Medicine, Korea University, Anam-ro 145, Seong Buk Gu, Seoul 02841, Republic of Korea. Electronic address:
    Objective: This study aimed to review and compare the diagnostic accuracy of the screening enzyme immunoassay (SEIA) and indirect immunofluorescence (IIF) as anti-nuclear antibody (ANA) screening assays for patients with systemic rheumatic diseases (SRDs), including systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), and systemic sclerosis (SSc).

    Methods: A systematic literature search was conducted in the Medline, Embase, Cochrane, Web of Science, and Scopus databases for articles published before August 2017. A bivariate random effects model was used to calculate pooled diagnostic values. Read More

    B-cell activity markers are associated with different disease activity domains in primary Sjögren's syndrome.
    Rheumatology (Oxford) 2018 Mar 28. Epub 2018 Mar 28.
    Rheumatology Research Group, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK.
    Objectives: B-cell activating factor (BAFF), β-2 microglobulin (β2M) and serum free light chains (FLCs) are elevated in primary SS (pSS) and associated with disease activity. We aimed to investigate their association with the individual disease activity domains of the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) in a large well-characterized pSS cohort.

    Methods: Sera from pSS patients enrolled in the UK Primary Sjögren's Syndrome Registry (UKPSSR) (n = 553) and healthy controls (n = 286) were analysed for FLC (κ and λ), BAFF and β2 M. Read More

    [A case of limbic encephalitis associated with Sjögren's syndrome mimicking anti N-methyl D-aspartate receptor encephalitis].
    Rinsho Shinkeigaku 2018 Mar 31. Epub 2018 Mar 31.
    Department of Neurology, Osaka City General Hospital.
    A 25-year-old woman in her 37 weeks and 5 days pregnant presented with abnormal behavior and memory impairment following a high fever. Her manifestations were diagnosed as limbic encephalitis, and she delivered a baby by Cesarean section. In the operation, bilateral ovarian tumors were found and resected, though they were revealed as non-teratoma afterward. Read More

    Epigenome-wide association studies for systemic autoimmune diseases: The road behind and the road ahead.
    Clin Immunol 2018 Mar 29. Epub 2018 Mar 29.
    Pfizer - University of Granada - Andalusian Government Center for Genomics and Oncological Research (GENYO), Granada, Spain; Unit of Inflammatory Chronic Diseases, Institute of Environmental Medicine, Karolinska Institutet, Solna, Sweden. Electronic address:
    Epigenetics is known to be an important mechanism in the pathogenesis of autoimmune diseases. Epigenetic variations can act as integrators of environmental and genetic exposures and propagate activated states in immune cells. Studying epigenetic alterations by means of genome-wide approaches promises to unravel novel molecular mechanisms related to disease etiology, disease progression, clinical manifestations and treatment responses. Read More

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