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    [Endocrinology and interdisciplinary consultation in internal medicine : Illustrated using the example of polyglandular autoimmune syndrome].
    Internist (Berl) 2017 Feb 23. Epub 2017 Feb 23.
    MVZ für Rheumatologie, Planegg, Deutschland.
    Polyglandular autoimmune syndromes encompass several endocrine and nonendocrine autoimmune disorders with variable onset and phenotype. Rheumatoid and gastroenterological symptoms in patients with autoimmune polyglandular syndromes are suggestive of additional rheumatoid gastrointestinal and hepatological autoimmune diseases. Autoimmune gastritis, celiac disease, autoimmune hepatitis, rheumatoid arthritis, Sjögren syndrome, and systemic Lupus erythematodus are of particular clinical relevance. Read More

    Biomarkers in Autoimmune Salivary Gland Disorders: A Review.
    ORL J Otorhinolaryngol Relat Spec 2017 Feb 24;79(1-2):43-53. Epub 2017 Feb 24.
    Otorhinolaryngology/Head and Neck Surgery, Klinikum rechts der Isar, Technical University Munich, Munich, Germany.
    Salivary glands are frequent sites of manifestations of autoimmune disorders in the head and neck. Sjögren syndrome, sarcoidosis, granulomatosis with polyangiitis, and IgG4-related sialadenitis represent the most important autoimmune salivary gland disorders. Due to the lack of specific symptoms, diagnosis of these conditions remains a challenge. Read More

    Anti-La positive, anti-Ro negative subset of primary Sjögren's syndrome: anti-La is a reality but is the disease?
    Clin Exp Rheumatol 2017 Feb 20. Epub 2017 Feb 20.
    The Arthritis and Clinical Immunology Program, Oklahoma Med.Research Foundation; Dept.of Medicine, University of Oklahoma Health Sciences Center; and Medical Service, Dept.of Veterans Affairs Medical Center, Oklahoma City, USA.
    Objectives: To characterise the serological and clinical findings in primary Sjögren's syndrome in which anti-La was found without anti-Ro. We hypothesised that a significant portion of these are falsely negative for anti-Ro60.

    Methods: Twenty-nine sera from primary Sjögren's syndrome patients were tested for antibodies directed against La and Ro. Read More

    Correlation between ESSDAI and ClinESSDAI in a real-life cohort of patients with Sjögren's syndrome.
    Clin Exp Rheumatol 2017 Feb 10. Epub 2017 Feb 10.
    Rheumatology Clinic, Department of Medical and Biological Sciences, Azienda Ospedaliero-Universitaria 'S. Maria della Misericordia', Udine, Italy.
    ****************************************************************************. Read More

    Patient phenotypes in fibromyalgia comorbid with systemic sclerosis or rheumatoid arthritis: influence of diagnostic and screening tests. Screening with the FiRST questionnaire, diagnosis with the ACR1990 and revised ACR 2010 criteria.
    Clin Exp Rheumatol 2017 Feb 8. Epub 2017 Feb 8.
    Rheumatology Department, Cochin Hospital, Paris Descartes University, Paris, France.
    Objectives: Fibromyalgia (FM) may occur with rheumatoid arthritis (RA) and systemic sclerosis (SSc), and debate remains about its diagnosis. We aimed to use three FM tools (a screening tool (FiRST), diagnostic criteria (ACR 1990 and revised 2010), to compare FM prevalence between RA and SSc patients, to describe the phenotypes of patients with comorbid FM, and to analyze links between FM and secondary Sjögren's syndrome (SS).

    Methods: Consecutive adult patients with confirmed RA or SSc from four university hospitals were tested with the three FM tools. Read More

    Compact Bone-Derived Multipotent Mesenchymal Stromal Cells (MSCs) for the Treatment of Sjogren's-like Disease in NOD Mice.
    Methods Mol Biol 2017 ;1553:25-39
    Craniofacial Tissue Engineering and Stem Cells Laboratory, Faculty of Dentistry, McGill University, 3640 University Street, M43, Montreal, QC, Canada.
    Compact bone (cortical or dense bone) is among the organs that contain multipotent mesenchymal stromal cells (MSCs). Unlike bone marrow plugs where MSCs were initially isolated, compact bone has minimal (amount of) hematopoietic cells and thus facilitates the MSCs isolation process. In vitro, MSCs from compact bone show multipotency and differentiation into mesenchymal tissues such as bone, adipose, and cartilage, under certain conditions. Read More

    La deletion from mouse brain alters pre-tRNA metabolism and accumulation of pre-5.8S rRNA, with neuron death and reactive astrocytosis.
    Mol Cell Biol 2017 Feb 21. Epub 2017 Feb 21.
    Commisioned Corps, U.S. Public Health Service, Rockville, Maryland
    Human La antigen (Sjögren's syndrome antigen B, SSB) is an abundant multifunctional RNA-binding protein. In the nucleoplasm, La binds to and protects from 3' exonucleases, the ends of precursor-tRNAs and other transcripts synthesized by RNA polymerase III, and facilitates their maturation, while a nucleolar isoform has been implicated in rRNA biogenesis by multiple independent lines of evidence. We showed earlier that conditional La knockout (La cKO) from mouse cortex neurons results in defective tRNA processing although pathway(s) involved in neuronal loss thereafter was unknown. Read More

    Mycobacterium chelonae keratitis in a patient with Sjogren''s syndrome.
    Eur J Ophthalmol 2008 Mar-Apr;18(2):294-296
    Department of Medical Microbiology, Center of Infectious Diseases, Leiden University Medical Center.
    Purpose: In this report a case of Mycobacterium chelonae keratitis in a patient without any previously described risk factors is described. The only risk factor found was a rheumatoid arthritis related Sjogren's syndrome.


    Case Report:

    Results: A 60-year-old woman was referred to the hospital with an infectious keratitis of the left eye of 3 months duration, unresponsive to empirical therapy with ofloxacin and tobramycin drops. Read More

    Suspension made with amniotic membrane: Clinical trial.
    Eur J Ophthalmol 2005 Jul-Aug;15(4):441-445
    Department of Ophthalmology, Eye Bank, S. Maria della Scaletta Hospital, Imola (BO) - Italy.
    Purpose: To investigate if a suspension made with amniotic membrane could have a beneficial effect on ocular surface diseases.

    Methods: In the Imola branch of the Eye Bank of Emilia Romagna, the authors prepared a suspension containing homogenized amniotic membrane previously conserved at -80 degrees Celsius. Subsequently, the authors gave this preparation to 21 patients: 8 had undergone lamellar keratoplasty, 4 had undergone penetrating keratoplasty, 2 had undergone photorefractive keratectomy with a delay of epithelialization, 3 had neurotrophic corneal ulcers, 2 had corneal burning, 1 had torpid corneal ulcer, and 1 had Sjogren syndrome. Read More

    B7-H4 deficiency in salivary gland of patients with primary Sjögren's syndrome impairs the regulatory effect on T cells.
    Int J Rheum Dis 2017 Feb 20. Epub 2017 Feb 20.
    Centre for Transplantation and Renal Research, Westmead Hospital, University of Sydney, Sydney, New South Wales, Australia.
    Aim: Our previous study confirmed the defect of B7-H4 expression in peripheral blood and salivary glands of patients with primary Sjögren's syndrome (pSS). The aim of this study was to analyze the effect of the deficit expression of B7-H4 on CD4(+) T cells.

    Methods: CD4(+) T cells were purified by magnetic-activated cell sorting MACS. Read More

    Targeting BAFF and APRIL in systemic lupus erythematosus and other antibody-associated diseases.
    Int Rev Immunol 2017 Jan;36(1):3-19
    d Department of Biochemistry , University of Lausanne , Lausanne , Switzerland.
    The B cell-stimulating molecules, BAFF (B cell activating factor) and APRIL (a proliferation-inducing ligand), are critical factors in the maintenance of the B cell pool and humoral immunity. In addition, BAFF and APRIL are involved in the pathogenesis of a number of human autoimmune diseases, with elevated levels of these cytokines detected in the sera of patients with systemic lupus erythematosus (SLE), IgA nephropathy, Sjögren's syndrome, and rheumatoid arthritis. As such, both molecules are rational targets for new therapies in B cell-driven autoimmune diseases, and several inhibitors of BAFF or BAFF and APRIL together have been investigated in clinical trials. Read More

    [Headaches in autoimmune diseases].
    Wiad Lek 2016 ;69(6):773-777
    Uniwersytet Medyczny w Lublinie, Polska.
    Vasculitides in autoimmune diseases are an important cause of secondary headaches. The article discusses the incidence of headache in primary and secondary vasculitides of the central nervous system. The symptoms of primary CNS vasculitis are presented. Read More

    Neonatal lupus: Follow-up in infants with anti-SSA/Ro antibodies and review of the literature.
    Autoimmun Rev 2017 Feb 14. Epub 2017 Feb 14.
    Department of Obstetrics and Gynaecology, Catholic University of Sacred Heart, Rome, Italy.
    Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur. Read More

    Increased Interleukin-17F is Associated with Elevated Autoantibody Levels and More Clinically Relevant Than Interleukin-17A in Primary Sjögren's Syndrome.
    J Immunol Res 2017 22;2017:4768408. Epub 2017 Jan 22.
    Department of Rheumatology and Immunology, Peking University People's Hospital and Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135), Beijing, China; Center of Clinical Immunology, Peking University, Beijing 100044, China; Peking-Tsinghua Center for Life Sciences, Beijing, China.
    Th17 related immune response is pathogenic in primary Sjögren's syndrome (pSS). However, the role of IL-17F, one potent inflammatory member of IL-17 family cytokines in pSS, has not been specifically defined. We recruited one hundred and nine pSS patients and forty-two healthy controls and their serum levels of IL-17A and IL-17F were determined by multiplex cytokine assays. Read More

    Isolated Ro52 Antibodies as Immunological Marker of a Mild Phenotype of Undifferentiated Connective Tissue Diseases.
    Int J Rheumatol 2017 22;2017:3076017. Epub 2017 Jan 22.
    Immunology Unit, Central Laboratory, Complejo Hospitalario Universitario de Canarias, Tenerife, Spain.
    The term undifferentiated connective tissue disease (UCTD) is used to describe undiagnosed patients that do not fulfill classification criteria for definite connective tissue disease (Systemic Lupus, Systemic Sclerosis, Sjögren Syndrome, and Dermatomyositis/Polymyositis). It is important to find serological markers as predictors of the evolution or severity of these diseases. The objective of this retrospective study was to investigate if there was a milder subgroup of UCTD with a special clinical profile consisting only in the presence of anti-Ro52 autoantibodies. Read More

    Spontaneous Canalicular Plug Erosion After Punctal Plug Placement.
    Cornea 2017 Feb 15. Epub 2017 Feb 15.
    *Department of Ophthalmology, Marshall University, Huntington, West Virginia; and †Department of Ophthalmology, University of North Carolina at Chapel Hill, Chapel Hill, NC.
    Purpose: To describe a case of keratoconjunctivitis sicca treated with punctal plug placement with the first reported complication of subsequent plug erosion through the canaliculus and extrusion through the lid.

    Methods: Punctal plugs were placed in a patient with Sjogren syndrome for treatment of keratoconjunctivitis sicca. More than 12 years later, the plug was noted to have eroded through the canaliculus and extruded through the conjunctiva and lid. Read More

    Interleukin-33 produced by M2 macrophages and other immune cells contributes to Th2 immune reaction of IgG4-related disease.
    Sci Rep 2017 Feb 13;7:42413. Epub 2017 Feb 13.
    Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, Fukuoka, Japan.
    IgG4-related disease (IgG4-RD) is characterized by elevated serum IgG4 and marked infiltration of IgG4-positive cells in multiple organs. Interleukin-33 (IL-33) is a recently described cytokine that is secreted by damaged epithelial cells, macrophages, and dendritic cells, and potently activates helper T type 2 (Th2) immune responses, which have been suggested to play a major role in IgG4 production of IgG4-RD. Here, we assessed the expression of IL-33 and related molecules in the salivary glands (SGs) of patients with IgG4-RD versus that in patients with Sjögren's syndrome (SS) and controls. Read More

    Ectopic lymphoid neogenesis in rheumatic autoimmune diseases.
    Nat Rev Rheumatol 2017 Mar 9;13(3):141-154. Epub 2017 Feb 9.
    Centre for Experimental Medicine and Rheumatology, John Vane Science Centre, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Charterhouse Square, London EC1M 6BQ, UK.
    Ectopic lymphoid neogenesis often occurs in the target tissues of patients with chronic rheumatic autoimmune diseases such as rheumatoid arthritis, Sjögren syndrome and other connective tissue disorders, including systemic lupus erythematosus and myositis. However, the mechanisms of ectopic lymphoid-like structure (ELS) formation and function are not entirely understood. For example, it is unclear whether ELSs indicate distinct disease phenotypes or whether they are evolutionary manifestations of chronic inflammation. Read More

    Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome.
    Intern Med 2017 15;56(4):419-423. Epub 2017 Feb 15.
    Department of Internal Medicine, Teikyo University School of Medicine, Japan.
    We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Read More

    Carbonic Anhydrase I and II Autoantibodies in Behçet's Disease.
    Acta Reumatol Port 2016 Dec 3. Epub 2016 Dec 3.
    Faculty of Engineering and Natural Sciences, Gumushane University.
    Background: Behçet's disease is a vasculitis, seen more frequently around the Mediterranean and the Far East, and evinces with oral and genital ulcerations, skin lesions and uveitis. Carbonic anhydrase (CA) is a metalloenzyme which is widely distributed in the living world, and it is essential for the regulation of acid-base balance. Anti-CA antibodies have been reported in many disorders, such as systemic lupus erythematosus, Sjögren's syndrome, rheumatoid arthritis, endometriosis, idiopathic chronic pancreatitis, type 1 diabetes and Graves' disease. Read More

    Prevalence of secondary Sjögren's syndrome in Indian patients with rheumatoid arthritis: a single-center study.
    Int J Rheum Dis 2017 Feb 15. Epub 2017 Feb 15.
    Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
    Aim: To assess the prevalence of secondary Sjögren's syndrome (sSS) and its association with joint damage in patients with rheumatoid arthritis (RA) from northern India.

    Methods: Patients included had RA, fulfilling the 1987 American College of Rheumatology criteria, with disease duration of more than 1 year. They were administered a sicca questionnaire that included six questions from subjective criteria of American European Consensus Group (AECG) criteria. Read More

    Granulomatous interstitial nephritis associated with Primary Sjögren's syndrome.
    Z Rheumatol 2017 Feb 14. Epub 2017 Feb 14.
    Internal Medicine/Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey.
    Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by lymphocytic and plasmacytic infiltration of the exocrine glands. Tubulointerstitial nephritis (TIN) is the most common type of renal involvement in pSS. However, clinically significant renal involvement is uncommon. Read More

    Extrahepatic Autoimmune Diseases in Patients with Autoimmune Liver Diseases: A Phenomenon Neglected by Gastroenterologists.
    Gastroenterol Res Pract 2017 16;2017:2376231. Epub 2017 Jan 16.
    Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, Tianjin 300052, China.
    Autoimmune liver diseases (AILDs) often coexist with other extrahepatic autoimmune diseases (EHAIDs). The spectrum of EHAIDs in patients with AILDs is similar, whereas the incidence is different. Notably, autoimmune thyroid disease and Sjogren's syndrome are the most common EHAIDs. Read More

    Mutations in INPP5K Cause a Form of Congenital Muscular Dystrophy Overlapping Marinesco-Sjögren Syndrome and Dystroglycanopathy.
    Am J Hum Genet 2017 Feb 1. Epub 2017 Feb 1.
    Department of Pharmacology and Physiology, The George Washington University School of Medicine and Health Science, Washington, DC 20037, USA. Electronic address:
    Congenital muscular dystrophies display a wide phenotypic and genetic heterogeneity. The combination of clinical, biochemical, and molecular genetic findings must be considered to obtain the precise diagnosis and provide appropriate genetic counselling. Here we report five individuals from four families presenting with variable clinical features including muscular dystrophy with a reduction in dystroglycan glycosylation, short stature, intellectual disability, and cataracts, overlapping both the dystroglycanopathies and Marinesco-Sjögren syndrome. Read More

    Subacute Cutaneous Lupus Erythematosus: Clinical Characteristics, Disease Associations, Treatments, and Outcomes in a Series of 90 Patients at Mayo Clinic, 1996-2011.
    Mayo Clin Proc 2017 Feb 6. Epub 2017 Feb 6.
    Department of Dermatology, Mayo Clinic, Rochester, MN. Electronic address:
    Objective: To characterize the clinical presentation, laboratory studies, disease associations, and treatments of subacute cutaneous lupus erythematosus (SCLE).

    Patients And Methods: A retrospective review of 90 patients with SCLE at Mayo Clinic from January 1, 1996, through October 28, 2011, was performed.

    Results: The mean patient age at diagnosis was 61 years; 64 patients (71%) were women, and 11 cases (12%) were drug induced (1996-2000, no drug-induced cases; 2001-2005, 2 cases; 2006-2011, 9 cases). Read More

    Ten-year incidence and prevalence of clinically diagnosed blepharitis in South Korea: A nationwide population-based cohort study.
    Clin Exp Ophthalmol 2017 Feb 9. Epub 2017 Feb 9.
    Department of Ophthalmology, Severance Hospital, Institute of Vision Research, Yonsei University College of Medicine, Seoul, Korea.
    Importance: Blepharitis is one of the most common conditions. However, no study has yet evaluated the epidemiology by evaluating a large population-based sample.

    Background: To evaluate the incidence and prevalence of clinically diagnosed blepharitis in South Korea. Read More

    It's more than dryness and fatigue: The patient perspective on health-related quality of life in Primary Sjögren's Syndrome - A qualitative study.
    PLoS One 2017 9;12(2):e0172056. Epub 2017 Feb 9.
    Division of Rheumatology and Immunology, Department of Internal Medicine, Medical University Graz, Graz, Austria.
    Objectives: In Primary Sjögren's Syndrome (PSS), there is an apparent lack of data concerning the perspectives of patients, their needs, preferences and difficulties of daily life. This qualitative study was conducted to explore perspectives and needs of patients with PSS that influence health related quality of life (HRQL).

    Methods: We recruited 20 PSS patients fulfilling the American-European consensus classification criteria out of the PSS cohort of the Medical University Graz, Austria. Read More

    A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome.
    Case Rep Neurol Med 2017 15;2017:5696512. Epub 2017 Jan 15.
    Neurology Department, Hospital Professor Doutor Fernando Fonseca, Amadora, Portugal.
    A 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and rest tremor of the upper limbs, cogwheel rigidity of the four limbs, retropulsion on standing position, and inability to walk. During the following 2 weeks she developed xerostomia and unilateral parotiditis that improved with steroids. Read More

    Association between rheumatic diseases and cancer: results from a clinical practice cohort study.
    Intern Emerg Med 2017 Feb 8. Epub 2017 Feb 8.
    Division of Internal Medicine, Immunorheumatology Unit, "Maggiore della Carità" Hospital, Corso Mazzini 18, 28100, Novara, Italy.
    The association between cancer and immune-mediated rheumatic conditions is controversial, especially as far as polymyalgia rheumatica (PMR) is concerned. Furthermore, no clinical feature has been shown to be suggestive of a paraneoplastic rheumatic syndrome. With the present study, we aim to address both these issues. Read More

    Predictable biomarkers of developing lymphoma in patients with Sjögren syndrome: a nationwide population-based cohort study.
    Oncotarget 2017 Feb 4. Epub 2017 Feb 4.
    Department of Medical Research, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
    Sjögren syndrome (SS) is commonly known to be correlated with lymphoma. This study included 16,396 individuals in the SS cohort and 65,584 individuals in the non-SS cohort, all of whom were enrolled in the Taiwan National Health Insurance database between 2000 and 2010. We evaluated the risk factors of non-Hodgkin's lymphoma (NHL) in the primary SS cohort by applying a Cox multivariable proportional-hazards model. Read More

    Effect of Tobacco Smoking on The Clinical, Histopathological, and Serological Manifestations of Sjögren's Syndrome.
    PLoS One 2017 6;12(2):e0170249. Epub 2017 Feb 6.
    Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, United States of America.
    Objectives: To assess the association of smoking habits with the clinical, serological, and histopathological manifestations of Sjögren's syndrome (SS) and non-Sjögren's sicca (non-SS sicca).

    Methods: Cross-sectional case-control study of 1288 patients with sicca symptoms (587 SS and 701 non-SS sicca) evaluated in a multi-disciplinary research clinic. Smoking patterns were obtained from questionnaire data and disease-related clinical and laboratory data were compared between current, past, ever, and never smokers. Read More

    [The value of different antibodies detection in diagnosis of rheumatism with uveitis].
    Zhonghua Yi Xue Za Zhi 2017 Jan;97(4):285-290
    Department of Clinical Laboratory, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.
    Objective: To investigate the value of HLA-B27 antigen, antinuclear antibody (ANA), anti-dsDNA and Anti-Neutrophil Cytoplasmic Antibodies (ANCA) detection in diagnosis of rheumatism with uveitis. Methods: Seven hundred and twenty four newly diagnosed patients with non-infection uveitis in Beijing Tongren Hospital from March 2012 to March 2016 who long-term lived in Beijing and its surrounding areas were continuously enrolled. HLA-B27 antigen expressions in peripheral blood lymphocytes and ANA, anti-dsDNA and ANCA levels in serum were tested. Read More

    Clinical characteristics and survival of pulmonary arterial hypertension associated with three major connective tissue diseases: A cohort study in China.
    Int J Cardiol 2017 Jan 18. Epub 2017 Jan 18.
    Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Peking Union Medical College & Chinese Academy of Medical Sciences, No. 1 Shuaifuyuan, Beijing 100730, China. Electronic address:
    Objective: Pulmonary arterial hypertension (PAH) is a major cause of death in connective tissue disease patients. This study investigated the clinical characteristics and survival of CTD-PAH in Chinese patients.

    Methods: This cohort study enrolled 190 consecutive PAH patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or primary Sjögren's syndrome (pSS) who visited our referral center between May 2006 and December 2014. Read More

    Uncovering stem cell differentiation factors for salivary gland regeneration by quantitative analysis of differential proteomes.
    PLoS One 2017 3;12(2):e0169677. Epub 2017 Feb 3.
    Department of Oral and Maxillofacial Diagnostic Sciences, University of Florida College of Dentistry, Gainesville, FL, United States of America.
    Severe xerostomia (dry mouth) compromises the quality of life in patients with Sjögren's syndrome or radiation therapy for head and neck cancer. A clinical management of xerostomia is often unsatisfactory as most interventions are palliative with limited efficacy. Following up our previous study demonstrating that mouse BM-MSCs are capable of differentiating into salivary epithelial cells in a co-culture system, we further explored the molecular basis that governs the MSC reprogramming by utilizing high-throughput iTRAQ-2D-LC-MS/MS-based proteomics. Read More

    Neutralizing antibodies against adeno-associated viruses in Sjögren's patients: implications for gene therapy.
    Gene Ther 2017 Feb 2. Epub 2017 Feb 2.
    Molecular Physiology and Therapeutics Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD, USA.
    One potential setback to the use of gene therapy for the treatment of Sjögren's syndrome is the presence of neutralizing antibodies (nAb) against adeno-associated virus (AAV) serotypes. In order to evaluate the efficacy of this treatment option, nAb titers were measured in both healthy individuals and Sjögren's patients. Several serotypes with known transduction activity in mouse salivary glands were tested and only AAV5 showed a statistically significant change in the prevalence of nAbs between Sjögren's and healthy participants. Read More

    A novel mutation in the proteolytic domain of LONP1 causes atypical CODAS syndrome.
    J Hum Genet 2017 Feb 2. Epub 2017 Feb 2.
    Department of Pediatric Neurology, Miyagi Children's Hospital, Miyagi, Japan.
    Cerebral, ocular, dental, auricular, skeletal (CODAS) syndrome is a rare autosomal recessive multisystem disorder caused by mutations in LONP1. It is characterized by intellectual disability, cataracts, delayed tooth eruption, malformed auricles and skeletal abnormalities. We performed whole-exome sequencing on a 12-year-old Japanese male with severe intellectual disability, congenital bilateral cataracts, spasticity, hypotonia with motor regression and progressive cerebellar atrophy with hyperintensity of the cerebellar cortex on T2-weighted images. Read More

    Work Disability in Newly Diagnosed Patients with Primary Sjögren Syndrome.
    J Rheumatol 2017 Feb 15;44(2):209-215. Epub 2016 Nov 15.
    From the Section of Rheumatology, Skåne University Hospital, Department of Clinical Sciences Malmö, Lund University, Malmö, Sweden; and the Parker Institute, Copenhagen University Hospital, Bispebjerg and Frederiksberg, Copenhagen, Denmark.
    Objective: To study longterm work disability and possible predictors in newly diagnosed patients with primary Sjögren syndrome (pSS).

    Methods: Because we wanted to include only patients with full work availability potential, eligible patients were aged 18-62 years. Fifty-one patients (mean age 46 yrs, range 18-61 yrs, 50 women) diagnosed with pSS between January 2001 and December 2012 were included in the study. Read More

    Hypokalaemia in Sjögren's syndrome: the missing piece.
    Clin Med (Lond) 2017 Feb;17(1):40-42
    National University Health System, Singapore and Yong Loo Lin School of Medicine, National University Singapore, Singapore.
    A 58-year-old Chinese woman with well controlled type 1 diabetes mellitus presented with acute and progressive bilateral lower limb weakness. Investigations revealed severe hypokalaemia (1.3 mmol/L) and hypophosphataemia (<0. Read More

    Autoantibody to MDM2: A potential serological marker of primary Sjogren's syndrome.
    Oncotarget 2017 Jan 28. Epub 2017 Jan 28.
    Department of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Xiamen University, Xiamen, China.
    Introduction: Primary Sjogren's Syndrome (pSS) is one of the autoimmune diseases characterized by polyclonal autoantibody production. The human homologue of the mouse double minute 2 (MDM2) is an important negative regulator of p53. Our previous study indicated that autoantibody to MDM2 can be detected in systemic lupus erythematosus patients. Read More

    Flagellar filament structural protein induces Sjögren's syndrome-like sialadenitis in mice.
    Oral Dis 2017 Jan 31. Epub 2017 Jan 31.
    Department of Microbiology and Immunology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan.
    Objective: Sjögren's syndrome (SS) is a systemic autoimmune disease that primarily affects lacrimal and salivary glands. We previously reported that FliC derived from E. coli could induce autoimmune pancreatitis-like lesions. Read More

    Enhanced Bruton's tyrosine kinase activity in peripheral blood B lymphocytes of autoimmune disease patients.
    Arthritis Rheumatol 2017 Jan 31. Epub 2017 Jan 31.
    Department of Pulmonary Medicine, Erasmus MC, Rotterdam, the Netherlands.
    Objective: Bruton's tyrosine kinase (BTK) transmits crucial survival signals from the B cell receptor (BCR) in B cells. Pharmacological BTK inhibition effectively diminishes disease symptoms in mouse models of autoimmunity and, conversely, transgenic BTK overexpression induces systemic autoimmunity in mice. We investigated BTK expression and activity in human B cells in the context of autoimmune disease. Read More

    Obstetric and perinatal outcome in anti-Ro/SSA-positive pregnant women: a prospective cohort study.
    Immunol Res 2017 Jan 30. Epub 2017 Jan 30.
    Obstetric and Gynecology Department, High Risk Pregnancy Unit, Autoimmune diseases and pregnancy clinic, University Hospital La Paz, Paseo de la Castella 261, 28046, Madrid, Spain.
    Anti-Ro/SS-A is one specific type of antinuclear antibodies. They are in the majority of cases associated with primary Sjögren syndrome (SS) but also in Systemic Lupus Erythematosus (SLE), rheumatoid arthritis (RA), and in healthy people. During pregnancy, they are mainly associated to congenital heart block (CHB) and neonatal lupus (NL). Read More

    A missense variant in NCF1 is associated with susceptibility to multiple autoimmune diseases.
    Nat Genet 2017 Mar 30;49(3):433-437. Epub 2017 Jan 30.
    Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, South Carolina, USA.
    Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease with a strong genetic component characterized by autoantibody production and a type I interferon signature. Here we report a missense variant (g.74779296G>A; p. Read More

    Mechanisms by Which B Cells and Regulatory T Cells Influence Development of Murine Organ-Specific Autoimmune Diseases.
    J Clin Med 2017 Jan 26;6(2). Epub 2017 Jan 26.
    Department of Molecular Microbiology & Immunology, University of Missouri, Columbia, MO 65212, USA.
    Experiments with B cell-deficient (B-/-) mice indicate that a number of autoimmune diseases require B cells in addition to T cells for their development. Using B-/- Non-obese diabetic (NOD) and NOD.H-2h4 mice, we demonstrated that development of spontaneous autoimmune thyroiditis (SAT), Sjogren's syndrome and diabetes do not develop in B-/- mice, whereas all three diseases develop in B cell-positive wild-type (WT) mice. Read More

    Polyneuropathy as Novel Initial Manifestation in a Case of "Nonsecretory" POEMS Syndrome with Sjögren's Syndrome.
    Case Rep Med 2017 4;2017:1276759. Epub 2017 Jan 4.
    Nursing Department, Huashan Hospital, Fudan University, Shanghai 200040, China.
    POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a paraneoplastic syndrome driven by plasma cell dyscrasias. We report a patient with novel initial manifestation of polyneuropathy, which was considered due to Sjögren's syndrome but with poor response to methylprednisolone (120 mg/d) and intravenous immunoglobulin (IVIg). Further investigation by imaging tests and following biopsy eventually confirmed the diagnosis of POEMS syndrome secondary to solitary plasmocytoma. Read More

    A case of neuromyelitis optica spectrum disorder (NMOSD) with Sjögren's syndrome manifested only brain involvement by preceding parotitis.
    Rinsho Shinkeigaku 2017 Jan 28. Epub 2017 Jan 28.
    Department of Neurology, Institute of Biomedical Sciences, Tokushima University Graduate School.
    A 33 year-old woman presented with intentional incontinence, motor aphasia, supranuclear gaze palsy, and spasticity after parotitis. Brain magnetic resonance images (MRI) showed abnormal signaling in long corticospinal tract involving internal capsules and cerebral peduncles, middle cerebellar peduncle, and frontal subcortical white matter lesions. She had a long history of dry eye and mouth. Read More

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