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    Long-term follow-up of finger passive range of motion in Japanese systemic sclerosis patients treated with self-administered stretching.
    Mod Rheumatol 2018 Apr 18:1-18. Epub 2018 Apr 18.
    b Department of Dermatology , School of Medicine, Institute of Medical, Pharmaceutical, and Health Sciences, Kanazawa University , Ishikawa , Japan.
    Objective: Severe skin sclerosis in patients with systemic sclerosis (SSc) can result in a loss of hand function. The aim of this study is to examine the long-term changes of finger passive range of motion (ROM) in Japanese SSc patients treated with self-administered stretching.

    Methods: This is a single center, retrospective, observational cohort study. Read More

    From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution.
    Dermatol Res Pract 2018 30;2018:1284687. Epub 2018 Jan 30.
    Scleroderma Unit, Chair of Rheumatology, University of Modena and Reggio Emilia, Modena, Italy.
    Background: Systemic sclerosis (SSc) and localized scleroderma (LoS) are two different diseases that may share some features. We evaluated the relationship between SSc and LoS in our case series of SSc patients.

    Methods: We analysed the clinical records of 330 SSc patients, in order to find the eventual occurrence of both the two diseases. Read More

    Pathogenic Effects of IFIT2 and Interferon-β during Fatal Systemic Candida albicans Infection.
    MBio 2018 Apr 17;9(2). Epub 2018 Apr 17.
    Department of Molecular Genetics and Microbiology, Stony Brook University, Stony Brook, New York, USA
    A balanced immune response to infection is essential to prevent the pathology and tissue damage that can occur from an unregulated or hyperactive host defense. Interferons (IFNs) are critical mediators of the innate defense to infection, and in this study we evaluated the contribution of a specific gene coding for IFIT2 induced by type I IFNs in a murine model of disseminated Invasive candidiasis is a frequent challenge during immunosuppression or surgical medical interventions, and is a common culprit that leads to high rates of mortality. When IFIT2 knockout mice were infected systemically with , they were found to have improved survival and reduced fungal burden compared to wild-type mice. Read More

    MDA-5 associated rapidly progressive interstitial lung disease with recurrent Pneumothoraces: a case report.
    BMC Pulm Med 2018 Apr 17;18(1):59. Epub 2018 Apr 17.
    Rheumatology Department, Cork University Hospital, Wilton, Cork, Ireland.
    Background: Clinically hypomyopathic dermatomyositis is a rare disease that is important to recognize, investigate and treat early as it is associated with poor prognosis. In a proportion of patients, myositis specific antibodies could be negative, but with high clinical suspicion, myositis associated antibodies should be ordered. Anti-MDA-5 antibodies was reported in literature to be associated with severe and rapidly progressive interstitial lung disease, with few case reports of pneumothorax and/or pneumomediastinum. Read More

    Oxidative stress in the pathogenesis of systemic scleroderma: An overview.
    J Cell Mol Med 2018 Apr 17. Epub 2018 Apr 17.
    Center for Gender-Specific Medicine, Biomarkers Unit, Rome, Italy.
    Systemic sclerosis (SSc) is a rare disorder of the connective tissue characterized by fibrosis of the skin, skeletal muscles and visceral organs. Additional manifestations include activation of the immune system and vascular injury. SSc causes disability and death as the result of end-stage organ failure. Read More

    Coexistence of systemic lupus erythematosus and multiple sclerosis. A case report and literature review.
    Mult Scler J Exp Transl Clin 2018 Apr-Jun;4(2):2055217318768330. Epub 2018 Apr 8.
    Department of Neurology, Hospital Carlos Andrade Marín, Ecuador.
    Multiple sclerosis (MS) and systemic lupus erythematous (SLE) are autoimmune diseases, the coexistence of which is uncommon in patients. Owing to the rarity of this condition, the distinction between MS and SLE is a diagnostic challenge for neurologists. We present a case report in which MS and SLE were present in the same patient. Read More

    Transcription factors operate across disease loci, with EBNA2 implicated in autoimmunity.
    Nat Genet 2018 Apr 16. Epub 2018 Apr 16.
    Center for Autoimmune Genomics and Etiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
    Explaining the genetics of many diseases is challenging because most associations localize to incompletely characterized regulatory regions. Using new computational methods, we show that transcription factors (TFs) occupy multiple loci associated with individual complex genetic disorders. Application to 213 phenotypes and 1,544 TF binding datasets identified 2,264 relationships between hundreds of TFs and 94 phenotypes, including androgen receptor in prostate cancer and GATA3 in breast cancer. Read More

    MIF functional polymorphisms (-794 CATT and -173 G>C) are associated with MIF serum levels, severity and progression in male multiple sclerosis from western Mexican population.
    J Neuroimmunol 2018 Apr 10. Epub 2018 Apr 10.
    Instituto de Investigación en Ciencias Biomédicas, Centro Universitario de Ciencias de la Salud, Universidad de Guadalajara, Guadalajara, Jalisco, Mexico. Electronic address:
    Macrophage migration inhibitory factor (MIF) is a cytokine associated with tissue damage in multiple autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis and psoriatic arthritis. The role of MIF in multiple sclerosis (MS) and the contribution of its polymorphisms are unknown in our population. Therefore, we decided to investigate the genetic association of -794 CATT (rs5844572) and -173 G>C (rs755622) MIF polymorphisms with MS, clinical variables and MIF serum levels in the population of western Mexico. Read More

    Evaluation of membrane-bound and soluble forms of HLA-G in Systemic Sclerosis.
    Clin Exp Immunol 2018 Apr 16. Epub 2018 Apr 16.
    Department of Internal Medicine, Clinical Immunology Unit, University of Genoa and Ospedale Policlinico San Martino, Genoa (Italy).
    Systemic sclerosis (SSc) is a complex disease characterized by immune dysregulation, extensive vascular damage and wide-spread fibrosis. Human leukocyte antigen-G (HLA-G) is a non-classic class I major histocompatibility complex (MHC) molecule characterized by complex immuno-modulating properties. HLA-G is expressed on the membrane of different cell lineages in both physiological and pathological conditions. Read More

    Venous Thromboembolism in Systemic Sclerosis: Prevalence, Risk Factors, and Effect on Survival.
    J Rheumatol 2018 Apr 15. Epub 2018 Apr 15.
    From the Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital; Division of Rheumatology, Department of Medicine, University of Toronto; Institute of Health Policy, Management and Evaluation; Department of Anesthesia and Institute of Health Policy, Management and Evaluation, University of Toronto; Department of Anesthesia and Pain Management, Toronto General Hospital, Toronto, Ontario, Canada; King Fahad Central Hospital, Ministry of Health, Jizan, Saudi Arabia. Dr. Johnson is supported by a Canadian Institutes of Health Research Clinician Scientist Award, the Oscar and Eleanor Markovitz Fund for Scleroderma Research, and the Freda Fejer Fund of the Arthritis Research Foundation. Dr. Wijeysundera is supported in part by a New Investigator Award from the Canadian Institutes of Health Research and a Merit Award from the Department of Anesthesia at the University of Toronto. S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, University of Toronto, and Institute of Health Policy, Management and Evaluation, University of Toronto; N. Hakami, MD, Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, University of Toronto, and King Fahad Central Hospital, Ministry of Health; Z. Ahmad, MD, Toronto Scleroderma Program, Mount Sinai Hospital, Division of Rheumatology, Department of Medicine, University of Toronto; D.N. Wijeysundera, MD, PhD, Department of Anesthesia and Pain Management, Toronto General Hospital, and Department of Anesthesia and Institute of Health Policy, Management and Evaluation, University of Toronto. Address correspondence to Dr. S. Johnson, Division of Rheumatology, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst St., Toronto, Ontario M5T 2S8, Canada. E-mail: Accepted for publication December 14, 2017.
    Objective: Whether systemic sclerosis (SSc) confers increased risk of venous thromboembolism (VTE) is uncertain. We evaluated the prevalence, risk factors, and effect of VTE on SSc survival.

    Methods: A cohort study was conducted of subjects with SSc who fulfilled the American College of Rheumatology/European League Against Rheumatism classification criteria between 1970 and 2017. Read More

    [Autologous stem cell transplantation with a myeloablative regimen for treatment of severe systemic sclerosis].
    Z Rheumatol 2018 Apr 13. Epub 2018 Apr 13.
    Zentrum für Interdisziplinäre Rheumatologie, klinische Immunologie und Autoimmunerkrankungen (INDIRA) und Innere Medizin II (Onkologie, Hämatologie, Rheumatologie, Immunologie und Pulmologie), Universitätsklinik Tübingen, Otfried-Müller-Str. 10, 72076, Tübingen, Deutschland.

    Clinicopathological investigation of odontogenic fibroma in tuberous sclerosis complex.
    Int J Oral Maxillofac Surg 2018 Apr 10. Epub 2018 Apr 10.
    Department of Radiation Oncology, Gunma University Graduate School of Medicine, Gunma, Japan.
    Tuberous sclerosis complex (TSC) is an autosomal dominant inherited disease characterized by systemic hamartoma and diverse systemic features. TSC1 and TSC2 are the causative genes, and mental retardation, epileptic seizures, and facial angiofibroma develop in many patients with the disease. The case of a patient with TSC who developed a central odontogenic fibroma of the mandible is reported here. 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

    Prediction of organ involvement in systemic sclerosis by serum biomarkers and peripheral endothelial function.
    Clin Exp Rheumatol 2018 Apr 12. Epub 2018 Apr 12.
    Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
    Objectives: To identify prognostic factors among serum biomarkers and endothelial vasodilator function findings in patients with systemic sclerosis (SSc).

    Methods: This is a clinical observational study. We assessed 60 consecutive SSc patients (44 limited cutaneous-type, 16 diffuse cutaneous-type). Read More

    Heptamer peptide disassembles native amyloid in human plasma via Heat Shock Protein 70.
    Rejuvenation Res 2018 Apr 13. Epub 2018 Apr 13.
    Drexel University College of Medicine, Neurobiology & Anatomy, Philadelphia, Pennsylvania, United States ;
    Proteostasis, which includes the repair and disposal of misfolded proteins, depends in part on the activity of heat shock proteins, a well-known class of chaperone molecules. When this process fails abnormally folded proteins may accumulate in cells, tissues, and blood. These species are a hallmark of protein aggregation diseases but also amass during aging, often in the absence of an identified clinical disorder. Read More

    How to conduct research of rare autoimmune diseases.
    Mod Rheumatol 2018 Apr 13:1-10. Epub 2018 Apr 13.
    a Laboratory for Statistical Analysis , RIKEN Center for Integrative Medical Sciences , Yokohama 230-0045 , Japan.
    Rare autoimmune diseases are difficult to conduct researches in spite of present era with advanced scientific progress. Research using genetic approach is a promising way since genetic findings implicate causality of diseases. Still, there are multiple obstacles preventing genetic studies of rare diseases. Read More

    Articular Surgery of the Ischemic Hand in Systemic Scleroderma: A Vascular Basis for Arthrodesis and Arthroplasty.
    J Hand Surg Am 2018 Apr 9. Epub 2018 Apr 9.
    Division of Plastic and Reconstructive Surgery, Brigham & Women's Hospital/Harvard Medical School, Boston, MA.
    Hallmark deformities of systemic scleroderma are early onset and progressively disabling flexion contractures of the proximal interphalangeal (PIP) joints often in conjunction with extension or, less frequently, flexion contractures of the metacarpophalangeal (MCP) joints. Although surgical correction is generally recommended, a prevailing reluctance for operative treatment exists owing to the inherent ischemia of the disease with its potentially compromised healing capacity. Nonetheless, with recognition and preservation of the tenuous but well-defined and constant periarticular vascular networks of the PIP and MCP joints, articular reconstruction with uncomplicated wound healing can prove consistently successful for patients with scleroderma. Read More

    Pulmonary paracoccidioidomycosis associated with the use of natalizumab in multiple sclerosis.
    Mult Scler 2018 Apr 1:1352458518763091. Epub 2018 Apr 1.
    Division of Neurosurgery, Department of Neurology, University of São Paulo, São Paulo, Brazil.
    Background: Natalizumab (NTZ) is a monoclonal antibody with an immunosuppressive effect that reduces the inflammation of the central nervous system, and it has been used for the treatment of relapsing-remitting multiple sclerosis (RRMS). In patients with low cellular immune response, systemic mycosis arising from endemic areas may occur.

    Results And Conclusion: In this article, we will describe a case of paracoccidioidomycosis as a complication to treatment with NTZ in an RRMS patient. 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

    Lower-limb Ulcers in Systemic Sclerosis: A Multicentre Retrospective Case-control Study.
    Acta Derm Venereol 2018 Apr 12. Epub 2018 Apr 12.
    Department of Dermatology, Tenon Hospital, Public Assistance - Paris Hospitals (AP-HP), Paris, France.
    Lower-limb ulcers in systemic sclerosis patients are rarely reported. The aim of this study was to describe the main causes and outcomes of lower-limb ulcers in systemic sclerosis patients and to assess factors associated with ischemic causes (arterial disease and/or microvascular impairment). A retrospective, multicentre, case-control study was conducted in 2013 and 2014, including 45 systemic sclerosis patients presenting lower-limb ulcers between 2008 and 2013. Read More

    The glucocorticoid receptor in recipient cells keeps cytokine secretion in acute graft-versus-host disease at bay.
    Oncotarget 2018 Mar 2;9(21):15437-15450. Epub 2018 Mar 2.
    Institute for Cellular and Molecular Immunology, University Medical Center, Georg-August-University Göttingen, Göttingen, Germany.
    Graft-versus-host disease (GvHD) is a life-threatening complication of hematopoietic stem cell transplantation (HSCT), which is caused by allogeneic T cells recognizing molecules of the recipient as foreign. Endogenous glucocorticoids (GC) released from the adrenal gland are crucial in regulating such inflammatory diseases. Here we demonstrate that genetically engineered mice, that are largely unresponsive to GC, suffer from aggravated clinical symptoms and increased mortality after HSCT, effects that could be tempered by neutralization of IL-6. Read More

    The challenge of establishing treatment efficacy for cutaneous vascular manifestations of systemic sclerosis.
    Expert Rev Clin Immunol 2018 Apr 11. Epub 2018 Apr 11.
    a Royal National Hospital for Rheumatic Diseases, Royal United Hospitals NHS Foundation Trust , Bath , BA1 1RL , UK.
    Introduction: The cutaneous vascular manifestations of systemic sclerosis (SSc) comprise Raynaud's phenomenon, cutaneous ulceration, telangiectasia formation and critical digital ischaemia; each of which are associated with significant disease-related morbidity. Despite the availability of multiple classes of vasodilator therapy, many of which have been the subject of RCTs, a limited number of pharmacological interventions are currently approved for the management of cutaneous vascular manifestations of SSc. Areas covered: A major challenge has been demonstrating treatment efficacy with examples of promising therapies yielding contrasting results in controlled trial settings. Read More

    Oesophageal diameter is associated with severity but not progression of systemic sclerosis-associated interstitial lung disease.
    Respirology 2018 Apr 11. Epub 2018 Apr 11.
    Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
    Background And Objective: It is unknown whether oesophageal disease is associated with systemic sclerosis-associated interstitial lung disease (SSc-ILD) severity, progression or mortality.

    Methods: High-resolution computed tomography (HRCT) scans from 145 SSc-ILD patients were scored for fibrosis score, oesophageal diameter and presence of hiatal hernia. Fibrosis asymmetry was calculated as: (most affected side - least affected side)/(most affected side + least affected side). Read More

    Ajulemic acid: potential treatment for chronic inflammation.
    Pharmacol Res Perspect 2018 Apr;6(2):e00394
    Department of Biochemistry & Molecular Pharmacology, University of Massachusetts Medical School, Worcester, MA, USA.
    Ajulemic acid (AJA, CT-3, IP-751, JBT-101, anabasum) is a first-in-class, synthetic, orally active, cannabinoid-derived drug that preferentially binds to the CB2 receptor and is nonpsychoactive. In preclinical studies, and in Phase 1 and 2 clinical trials, AJA showed a favorable safety, tolerability, and pharmacokinetic profile. It also demonstrated significant efficacy in preclinical models of inflammation and fibrosis. Read More

    Efficacy and safety of rituximab in systemic sclerosis: French retrospective study and literature review.
    Autoimmun Rev 2018 Apr 7. Epub 2018 Apr 7.
    Department of Internal Medicine, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique - Hôpitaux de Paris, Hôpital Saint-Antoine, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Sorbonne Universités, UMPC University Paris 06, INSERM U938, Centre de Recherche Saint-Antoine (CRSA), Paris, France. Electronic address:
    Objective: To describe safety and efficacy of rituximab in patients with systemic sclerosis.

    Methods: We included 13 patients with systemic sclerosis treated with rituximab and pooled with 40 additional patients from the literature. SSc rituximab untreated patients were matched to rituximab treated ones. Read More

    Systemic lupus erythematosus and systemic sclerosis: All roads lead to platelets.
    Autoimmun Rev 2018 Apr 7. Epub 2018 Apr 7.
    Laboratoire d'Immunologie et Immunogénétique, FHU ACRONIM, Hôpital Pellegrin, Centre Hospitalier Universitaire, Place Amélie Raba Léon, 33076 Bordeaux, France; Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France; CNRS-UMR 5164, ImmunoConcept, Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France. Electronic address:
    Systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) are two phenotypically distincts inflammatory systemic diseases. However, SLE and SSc share pathogenic features such as interferon signature, loss of tolerance against self-nuclear antigens and increased tissue damage such as fibrosis. Recently, platelets have emerged as a major actor in immunity including auto-immune diseases. 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

    Exercise Hemodynamics for the Diagnosis of Diastolic Dysfunction in Dyspneic Patients with Systemic Sclerosis.
    Isr Med Assoc J 2018 Apr;20(4):245-249
    Leviev Heart Center, Sheba Medical Center, Tel Hashomer, Israel.
    Objectives: To assess the added diagnostic value of using exercise hemodynamics during RHC in assessment of patients with symptomatic SSc.

    Methods: We performed 22 RHCs in 17 SSc patients with dyspnea and/or pulmonary arterial hypertension (PAH). Exercise was performed in 15 RHCs using isotonic arm exercises while holding a 1 kg weight in each hand. Read More

    Lung transplantation for scleroderma lung disease: An international, multicenter, observational cohort study.
    J Heart Lung Transplant 2018 Mar 15. Epub 2018 Mar 15.
    Service de Chirurgie Thoracique, Vasculaire et Transplantation Cardio-pulmonaire, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France; Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France; UMR_S 999, Université Paris-Sud, INSERM, Hôpital Marie Lannelongue, Le Plessis Robinson, France. Electronic address:
    Background: Due to its multisystemic nature, scleroderma is considered a relative contraindication to lung transplantation at many centers. However, recent studies suggest similar post-transplant outcomes in patients with scleroderma compared to those with other causes of interstitial lung disease (ILD). Furthermore, it remains unknown whether scleroderma-associated pulmonary arterial hypertension (PAH) influences post-transplant outcomes. Read More

    Sleep disturbance and symptom burden in sarcoidosis.
    Respir Med 2018 Mar 24. Epub 2018 Mar 24.
    University of California, San Francisco, Division of Pulmonary and Critical Care, 505 Parnassus Ave, San Francisco, CA 94143, USA. Electronic address:
    Introduction: Sarcoidosis is a systemic inflammatory disease associated with myriad symptoms, including fatigue. It can affect physiological processes like sleep, leading to poor sleep quality and excessive daytime sleepiness. We hypothesized that sarcoidosis patients would report more severe sleep disturbance than healthy controls and that relationships would be found with sleep disturbance and the severity of other symptoms. 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

    Autoantibodies in Serum of Systemic Scleroderma Patients: Peptide-Based Epitope Mapping Indicates Increased Binding to Cytoplasmic Domains of CXCR3.
    Front Immunol 2018 22;9:428. Epub 2018 Mar 22.
    Department of Rheumatology, University of Lübeck, Lübeck, Germany.
    Systemic sclerosis (SSc) is a severe chronic autoimmune disease with high morbidity and mortality. Sera of patients with SSc contain a large variety of autoantibody (aab) reactivities. Among these are functionally active aab that bind to G protein-coupled receptors (GPCR) such as C-X-C motif chemokine receptor 3 (CXCR3) and 4 (CXCR4). Read More

    Applied Bayesian Methods in the Rheumatic Diseases.
    Rheum Dis Clin North Am 2018 May;44(2):361-370
    Institute of Health Policy, Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada; Division of Rheumatology, Department of Paediatrics, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.
    The use of applied Bayesian methods is increasing in rheumatology. Using the Bayes theorem, past evidence is updated with new data. Preexisting data are expressed as a prior probability distribution or prior. Read More

    Propensity Score Methods for Bias Reduction in Observational Studies of Treatment Effect.
    Rheum Dis Clin North Am 2018 May;44(2):203-213
    Institute of Health Policy, Management and Evaluation, University of Toronto, 155 College Street, Toronto, Ontario M5T 3M6, Canada; Dalla Lana School of Public Health, University of Toronto, 155 College Street, Toronto, Ontario M5T 3M7, Canada; Division of Rheumatology, Department of Paediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.
    A challenge to the use of observational data to study treatment effects is the issue of confounding. Noncomparability of exposed and nonexposed subjects can lead to biased estimation of the treatment effect. The propensity score is a balancing score that can be used to form matched groups, or pairs, that are not systematically different and enable nonbiased comparisons between groups. Read More

    Autoimmune/inflammatory syndrome induced by mineral oil: a health problem.
    Clin Rheumatol 2018 Apr 4. Epub 2018 Apr 4.
    Unidad Médica de Alta Especiad, Hospital de Especialidades "Dr. Antonio Fraga Mouret," Centro Médico Nacional "La Raza", Instituto Mexicano del Seguro Social, Mexico City, Mexico.
    Autoimmune/inflammatory syndrome induced by adjuvant (ASIA) includes the following conditions: siliconosis, Gulf War syndrome, macrophagic myofasciitis syndrome, and post-vaccination phenomena. Afterward, other syndromes have been recognized, such as in ASIA by mineral oil (ASIA-MO). These conditions are triggered by adjuvants and they are the result of the interplay of genetic and environmental factors. Read More

    TNFSF14 (LIGHT) Exhibits Inflammatory Activities in Lung Fibroblasts Complementary to IL-13 and TGF-β.
    Front Immunol 2018 19;9:576. Epub 2018 Mar 19.
    Division of Immune Regulation, La Jolla Institute for Allergy and Immunology, La Jolla, CA, United States.
    The cytokine TNFSF14 [homologous to Lymphotoxin, exhibits Inducible expression and competes with HSV Glycoprotein D for binding to HVEM, a receptor expressed on T lymphocytes (LIGHT)] has been shown in mouse models to be important for development of lung tissue remodeling that is characteristic of asthma, idiopathic pulmonary fibrosis (IPF), and systemic sclerosis (SSc). However, its cellular targets are not fully delineated. In the present report, we show that LTβR and HVEM, the receptors for LIGHT, are constitutively expressed in primary human lung fibroblasts (HLFs). 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

    Ozanimod (RPC1063), a selective S1PR1 and S1PR5 modulator, reduces chronic inflammation and alleviates kidney pathology in murine systemic lupus erythematosus.
    PLoS One 2018 2;13(4):e0193236. Epub 2018 Apr 2.
    Receptos, a wholly owned subsidiary of Celgene Corporation, San Diego, California, United States of America.
    Ozanimod (RPC1063) is a specific and potent small molecule modulator of the sphingosine 1-phosphate receptor 1 (S1PR1) and receptor 5 (S1PR5), which has shown therapeutic benefit in clinical trials of relapsing multiple sclerosis and ulcerative colitis. Ozanimod and its active metabolite, RP-101075, exhibit a similar specificity profile at the S1P receptor family in vitro and pharmacodynamic profile in vivo. The NZBWF1 mouse model was used in therapeutic dosing mode to assess the potential benefit of ozanimod and RP-101075 in an established animal model of systemic lupus erythematosus. Read More

    Pathophysiology and management of glaucoma associated with phakomatoses.
    J Neurosci Res 2018 Apr 1. Epub 2018 Apr 1.
    Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois.
    The phakomatoses, encephalotrigeminal angiomatosis (ETA; Sturge-Weber Syndrome), neurofibromatosis type 1 (NF1 or von Recklinghausen disease), Von Hippel-Lindau (VHL) disease, tuberous sclerosis (TSC), oculodermal melanocytosis (ODM), and phakomatosis pigmentovascularis are a group of neurocutaneous disorders that have characteristic systemic and ocular manifestations. Through many different mechanisms, they may cause glaucomatous damage of the optic nerve and subsequent vision loss varying from mild to severe. Glaucoma commonly affects patients with ETA (43-72%), orbito-facial NF1 (23-50%), and ODM (10%). Read More

    Mycophenolate Mofetil in a Lupus Patient with Pulmonary Hypertension.
    Cureus 2018 Jan 29;10(1):e2121. Epub 2018 Jan 29.
    Medicine, The Johns Hopkins University School of Medicine.
    Pulmonary hypertension (PH) is a life-threatening complication of several, different connective tissue diseases, including systemic lupus erythematous (SLE), systemic sclerosis, and rheumatoid arthritis. PH can present early in SLE. The severity does not correlate with other organ disease activity or with disease duration. Read More

    Automated structure and flow measurement - a promising tool in nailfold capillaroscopy.
    Microvasc Res 2018 Mar 29;118:173-177. Epub 2018 Mar 29.
    Division of Musculoskeletal & Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK; NIHR Manchester Musculoskeletal Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. Electronic address:
    Objectives: Despite increasing interest in nailfold capillaroscopy, objective measures of capillary structure and blood flow have been little studied. We aimed to test the hypothesis that structural measurements, capillary flow, and a combined measure have the predictive power to separate patients with systemic sclerosis (SSc) from those with primary Raynaud's phenomenon (PRP) and healthy controls (HC).

    Methods: 50 patients with SSc, 12 with PRP, and 50 HC were imaged using a novel capillaroscopy system that generates high-quality nailfold images and provides fully-automated measurements of capillary structure and blood flow (capillary density, mean width, maximum width, shape score, derangement and mean flow velocity). Read More

    Neutrophils and redox stress in the pathogenesis of autoimmune disease.
    Free Radic Biol Med 2018 Mar 29. Epub 2018 Mar 29.
    Department of Musculoskeletal Biology, Institute of Ageing and Chronic Disease, University of Liverpool, 6 West Derby Street, L7 8TX Liverpool, UK. Electronic address:
    Polymorphonuclear leukocytes, or neutrophils, are specialist phagocytic cells of the innate immune system. Their primary role is host defence against micro-organisms, which they kill via phagocytosis, followed by release of reactive oxygen species (ROS) and proteolytic enzymes within the phagosome. ROS are generated via the action of the NADPH oxidase (also known as NOX2), in a process termed the 'Respiratory Burst'. Read More

    From molecules to patients: exploring the therapeutic role of soluble guanylate cyclase stimulators.
    Biol Chem 2018 Mar 1. Epub 2018 Mar 1.
    1Bayer AG, Drug-Discovery, Pharma Research Center Wuppertal, Aprather Weg 18a, D-42069 Wuppertal, Germany and Hannover Medical School, Department of Pharmacology, Hannover, Germany.
    Nitric Oxide (NO) signaling represents one of the major regulatory pathways for cardiovascular function. After the discovery of NO, awarded with the Nobel Prize in 1998, this signaling cascade was stepwise clarified. We now have a good understanding of NO production and NO downstream targets such as the soluble guanylyl cyclases (sGCs) which catalyze cGMP production. Read More

    Does nailfold capillaroscopy help predict future outcomes in systemic sclerosis? A systematic literature review.
    Semin Arthritis Rheum 2018 Feb 14. Epub 2018 Feb 14.
    Department of Pharmacy and Pharmacology, University of Bath, Bath, UK; Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Upper Borough Walls, Bath, Bath BA1 1RL, UK. Electronic address:
    Background: Nailfold capillaroscopy (NC) is an important diagnostic tool in systemic sclerosis (SSc). Confirmation of NC as a prognostic factor could facilitate earlier intervention and slow disease progression in SSc. We undertook a systematic literature review to evaluate the prognostic value of NC in predicting SSc disease progression. Read More

    Use of tocilizumab in systemic sclerosis: A brief literature review.
    Rev Clin Esp 2018 Mar 27. Epub 2018 Mar 27.
    Servicio de Medicina Interna, Hospital Universitario Central de Asturias, Oviedo, España.
    The available treatments for systemic sclerosis (SS) have limited effectiveness. Treatment with tocilizumab (TCZ), a biological drug that inhibits interleukin 6 (IL-6), has recently been proposed. In this study, we conducted a literature review to assess the safety and efficacy of TCZ in SS. Read More

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