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    Single agent and synergistic combinatorial efficacy of first-in-class small molecule imipridone ONC201 in hematological malignancies.
    Cell Cycle 2017 Nov 20:1-29. Epub 2017 Nov 20.
    a Oncoceutics, Inc. , Philadelphia , PA.
    ONC201, founding member of the imipridone class of small molecules, is currently being evaluated in advancer cancer clinical trials. We explored single agent and combinatorial efficacy of ONC201 in preclinical models of hematological malignancies. ONC201 demonstrated (GI50 1-8 µM) dose- and time-dependent efficacy in acute myeloid leukemia (AML), acute lymphoblastic leukemia (ALL), chronic myelogenous leukemia (CML), chronic lymphocytic leukemia (CLL), diffuse large B-cell lymphoma (DLBCL), mantle cell lymphoma (MCL), Burkitt's lymphoma, anaplastic large cell lymphoma (ALCL), cutaneous T-cell lymphoma (CTCL), Hodgkin's lymphoma (nodular sclerosis) and multiple myeloma (MM) cell lines including cells resistant to standard of care (dexamethasone in MM) and primary samples. Read More

    Work productivity in systemic sclerosis, its economic burden and association with health-related quality of life.
    Rheumatology (Oxford) 2017 Oct 9. Epub 2017 Oct 9.
    Department of Medicine, University of Melbourne at St Vincent's Hospital Melbourne.
    Objective: To evaluate work productivity and its economic burden in SSc patients.

    Methods: Consecutive SSc patients enrolled in the Australian Scleroderma Cohort Study were mailed questionnaires assessing employment (Workers' Productivity and Activity Impairment Questionnaire and a custom-made questionnaire) and health-related quality of life (HRQoL) (36-item Short Form Health Survey and Patient-Reported Outcomes Measurement Information System 29). Linear regression methods were used to determine factors associated with work productivity. Read More

    Autologous hematopoietic stem cell transplantation in systemic sclerosis: a reset to tolerance?
    Immunol Lett 2017 Nov 16. Epub 2017 Nov 16.
    Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands. Electronic address:
    Autologous hematopoietic stem cell transplantation (ASCT) is an effective therapy for refractory autoimmune disease, in particular diffuse cutaneous systemic sclerosis (dcSSc). ASCT is the only treatment that can induce long term remission in dcSSc. However, the mechanism of action of ASCT has not yet been fully elucidated. Read More

    14-3-3z sequesters cytosolic T-bet, up-regulating IL-13 in Tc2 and scleroderma CD8+ lymphocytes.
    J Allergy Clin Immunol 2017 Nov 15. Epub 2017 Nov 15.
    Department of Medicine, Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh PA 15261 USA; Department of Immunology,University of Pittsburgh School of Medicine, Pittsburgh PA 15261 USA. Electronic address:
    Background: Interleukin(IL)-13-producing CD8+ T cells have been implicated in the pathogenesis of type-2 driven inflammatory human conditions. We have shown that CD8+IL-13+ cells play a critical role in cutaneous fibrosis, the most characteristic feature of systemic sclerosis (scleroderma; SSc). However, the molecular mechanisms underlying IL-13 and other type-2 cytokine production by CD8+ T cells remain unclear. Read More

    Cardiac magnetic resonance imaging in myocardial inflammation in autoimmune rheumatic diseases: An appraisal of the diagnostic strengths and limitations of the Lake Louise criteria.
    Int J Cardiol 2017 Nov 14. Epub 2017 Nov 14.
    Department of Medicine, Radiology and Epidemiology Johns Hopkins University, Baltimore, MD, USA.
    Myocardial inflammation in autoimmune rheumatic diseases (ARDs) is the endpoint of various pathophysiologic processes. The Lake Louise-criteria is the most popular approach for the diagnosis of myocarditis. However, due to the diversity of myocardial inflammation in ARDs, some issues should be acknowledged. Read More

    The relationship between the degree of skin fibrosis by sonoelastography and the degree of pulmonary involvement in scleroderma
    Turk J Med Sci 2017 11 13;47(5):1555-1559. Epub 2017 Nov 13.
    Background/aim: This study aimed to assess the relationship between skin fibrosis as determined by sonoelastography and the degree of pulmonary involvement as determined by high-resolution computed tomography (HRCT) in patients with diffuse cutaneous systemic sclerosis (dcSSc).Materials and methods: This prospective study included 40 patients with dcSSc. All patients with HRCT scans underwent conventional ultrasonography and sonoelastography to determine skin thickness and degree of fibrosis. Read More

    Physical activity in patients with systemic sclerosis.
    Rheumatol Int 2017 Nov 18. Epub 2017 Nov 18.
    Department of Rheumatology, Leiden University Medical Centre, C1-51, PO Box 9600, 2300 RC, Leiden, The Netherlands.
    Objectives: To compare self-reported levels of physical activity (PA) of systemic sclerosis (SSc) patients with the general population. To evaluate in SSc patients factors associated with PA levels and needs and preferences regarding PA.

    Methods: Fifty nine SSc patients completed the Short QUestionnaire to ASsess Health-Enhancing PA. Read More

    Gastrointestinal and Hepatic Disease in Systemic Sclerosis.
    Rheum Dis Clin North Am 2018 Feb;44(1):15-28
    Department of Internal Medicine, University of Colorado, Denver, 12631 East 17th Avenue, Aurora, CO 80045, USA.
    Although classification criteria for systemic sclerosis (SSc) do not incorporate gastrointestinal tract (GIT) manifestations often present in this disease, the GIT is the most common internal organ involved. Pathophysiology of GIT involvement is thought to be similar to other organs in SSc with fibroproliferative vascular lesions of small arteries and arterioles, increased production of profibrotic growth factors, and alterations of innate, humoral, and cellular immunity. These processes result in neuropathy progressing to myopathy with eventual fibrosis. Read More

    Primary Testicular Diffuse Large B-Cell Lymphoma: A Case Report.
    World J Oncol 2013 Feb 6;4(1):61-65. Epub 2013 Mar 6.
    Private Medicalpark Bahcelievler Hospital, Department of Nuclear Medicine, Istanbul, Turkey.
    Testicular lymphoma was first reported by Malassez and Curling in 1866. Primary testicular lymphoma constitutes only 1-7% of all testicular neoplasms and less than 1% of all non Hodgkin lymphoma. We report the case of a 47-year-old man without a particular past medical history, who presented with a painful left testicular swelling that he has noticed for several weeks. Read More

    Defining a unified vascular phenotype in systemic sclerosis.
    Arthritis Rheumatol 2017 Nov 16. Epub 2017 Nov 16.
    UCL Division of Medicine, University College London, London, UK.
    Microcirculation impairment and related vasculopathy is a hallmark of systemic sclerosis. Digital ulcers are second only to Raynaud's phenomenon as a vascular complication occurring in scleroderma patients. Digital ulcers are painful and generate disability. Read More

    Identification of novel systemic sclerosis biomarkers employing aptamer proteomic analysis.
    Rheumatology (Oxford) 2017 Nov 13. Epub 2017 Nov 13.
    Jefferson Institute of Molecular Medicine and The Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, USA.
    There is an important unmet need for clinically validated non-invasive biomarkers for SSc diagnosis, assessment of disease activity, extent of internal organ involvement, therapeutic response and prognosis. There is also an unmet need for biomarkers to accurately differentiate primary RP from recent onset RP evolving into SSc. The lack of sensitive and specific biomarkers for SSc and SSc-associated RP is a limitation for the optimal clinical management of these patients. Read More

    Localized Scleroderma, Systemic Sclerosis and Cardiovascular Risk: A Danish Nationwide Cohort Study.
    Acta Derm Venereol 2017 Nov 14. Epub 2017 Nov 14.
    Department of Dermatology and Allergy, Herlev and Gentofte University Hospital, University of Copenhagen, Kildegaardsvej 28, DK-2900 Hellerup, Denmark.
    Recent findings indicate that patients with systemic sclerosis have an increased risk of cardiovascular disease. To determine whether patients with systemic sclerosis or localized scleroderma are at increased risk of cardiovascular disease, a cohort study of the entire Danish population aged ≥ 18 and ≤ 100 years was conducted, followed from 1997 to 2011 by individual-level linkage of nationwide registries. Multivariable adjusted Cox regression models were used to estimate the hazard ratios (HRs) for a composite cardiovascular disease endpoint. Read More

    The Plasma Levels of ADAMTS-13, von Willebrand Factor, VWFpp, and Fibrin-Related Markers in Patients With Systemic Sclerosis Having Thrombosis.
    Clin Appl Thromb Hemost 2017 Jan 1:1076029617736382. Epub 2017 Jan 1.
    1 Department of Dermatology, Mie University Graduate School of Medicine, Mie, Tsu, Japan.
    This study aimed to examine the hemostatic abnormalities in patients with systemic sclerosis (SSc) and the relationship between these abnormalities and thrombotic events (THEs), focusing on the difference in diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc). The plasma levels of ADAMTS-13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13), von Willebrand factor (VWF), VWF propeptide (VWFpp), d-dimer, and soluble fibrin (SF) were measured in 233 patients with SSc. The relationship between their levels and organ involvement, including THEs and interstitial lung disease (ILD), was evaluated. Read More

    Increased frequencies of circulating CXCL10-, CXCL8- and CCL4-producing monocytes and Siglec-3-expressing myeloid dendritic cells in systemic sclerosis patients.
    Inflamm Res 2017 Nov 10. Epub 2017 Nov 10.
    Blood and Transplantation Center of Coimbra, Portuguese Institute of Blood and Transplantation, Coimbra, Portugal.
    Objective: To investigate the ex vivo pro-inflammatory properties of classical and non-classical monocytes as well as myeloid dendritic cells (mDCs) in systemic sclerosis (SSc) patients.

    Methods: Spontaneous production of CXCL10, CCL4, CXCL8 and IL-6 was intracellularly evaluated in classical, non-classical monocytes and Siglec-3-expressing mDCs from peripheral blood of SSc patients and healthy controls (HC) through flow cytometry. In addition, production of these cytokines was determined upon toll-like receptor (TLR) 4 plus Interferon-γ (IFN-γ) stimulation. Read More

    Systemic sclerosis and the gastrointestinal tract.
    Prz Gastroenterol 2017 30;12(3):163-168. Epub 2017 Sep 30.
    Clinical Department of Dermatology, Central Clinical Hospital of the MSWiA, Warsaw, Poland.
    Systemic sclerosis (SSc) is an autoimmunological disease of unknown origin with complex pathogenesis and multiple organ involvement. It is characterised by vascular and immunological abnormalities leading to fibrosis of the skin and internal organs. It is a rather rare disease with a prevalence of around 20 per 100,000. Read More

    Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP): Integrating the literature on hereditary diffuse leukoencephalopathy with spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD).
    J Clin Neurosci 2017 Nov 6. Epub 2017 Nov 6.
    Department of Pathology and Laboratory Medicine, University of Saskatchewan, Royal University Hospital, 103 Hospital Drive, Saskatoon, Saskatchewan S7N 0W8, Canada. Electronic address:
    Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a progressive degenerative white matter disorder. ALSP was previously recognized as two distinct entities, hereditary diffuse leukoencephalopathy with spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD). However, recent identification of mutations in the tyrosine kinase domain of the colony stimulating factor 1 receptor (CSF1R) gene, which regulates mononuclear cell lineages including microglia, have provided genetic and mechanistic evidence that POLD and HDLS should be regarded as a single clinicopathologic entity. Read More

    Novel lung imaging biomarkers and skin gene expression subsetting in dasatinib treatment of systemic sclerosis-associated interstitial lung disease.
    PLoS One 2017 9;12(11):e0187580. Epub 2017 Nov 9.
    Northwestern Scleroderma Program, Feinberg School of Medicine, Chicago, IL, United States of America.
    Background: There are no effective treatments or validated clinical response markers in systemic sclerosis (SSc). We assessed imaging biomarkers and performed gene expression profiling in a single-arm open-label clinical trial of tyrosine kinase inhibitor dasatinib in patients with SSc-associated interstitial lung disease (SSc-ILD).

    Methods: Primary objectives were safety and pharmacokinetics. Read More

    Bench to Bedside - and Back Again: Finding the Goldilocks Zone within the Scleroderma Universe.
    Arthritis Rheumatol 2017 Nov 9. Epub 2017 Nov 9.
    Royal Free Hospital and UCL Medical School, Centre of rRheumatology.
    Systemic sclerosis (SSc) is a complex inflammatory connective tissue disease characterized by skin thickening, organ fibrosis and vasculopathy [1, 2]. The underlying disease mechanisms and pathophysiology are not fully understood. However, it appears to be, at least in part, driven by autoimmunity and inflammation associated with microvascular dysfunction ultimately resulting in excess extracellular matrix deposition in target organs [2-4]. Read More

    Imaging findings in systemic childhood diseases presenting with dermatologic manifestations.
    Clin Imaging 2017 Oct 31;49:17-36. Epub 2017 Oct 31.
    Department of Radiology, Division of Pediatric Radiology, Montefiore Medical Center, Bronx, NY, United States. Electronic address:
    Purpose: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs.

    Methods: We review the imaging findings in childhood diseases associated with dermatologic manifestations. Read More

    Orthopedic complications of linear morphea: Implications for early interdisciplinary care.
    Pediatr Dermatol 2017 Nov 9. Epub 2017 Nov 9.
    Department of Dermatology, Mayo Clinic, Rochester, MN, USA.
    Linear morphea of the limb primarily affects children, and extracutaneous manifestations are common. Orthopedic surgeons are often essential in the care of patients with linear morphea, yet there are few reports outlining specific orthopedic complications in this population. We sought to improve the understanding of orthopedic complications in linear morphea of the limb. Read More

    Determinants of mortality in systemic sclerosis: a focused review.
    Rheumatol Int 2017 Nov 7. Epub 2017 Nov 7.
    Division of Rheumatology, University of Pennsylvania School of Medicine, 3400 Spruce Street, Philadelphia, PA, 19104, USA.
    Scleroderma (systemic sclerosis) is an autoimmune rheumatic disorder that is characterized by fibrosis, vascular dysfunction, and autoantibody production that involves most visceral organs. It is characterized by a high morbidity and mortality rate, mainly due to disease-related complications. Epidemiological data describing mortality and survival in this population have been based on both population and observational studies. Read More

    Can fibrinolytic system components explain cognitive impairment in multiple sclerosis?
    J Neurol Sci 2017 Nov 23;382:66-72. Epub 2017 Sep 23.
    Department of Neurology, Hospital Dr. José Molina Orosa, Ctra. Arrecife. Lanzarote. Las Palmas de Gran Canaria, Spain.
    Background: The fibrinolytic system is capable of modulating inflammatory and degenerative events within the central nervous system. Specifically, the plasminogen activator inhibitor-1 (PAI-1) has been associated with different pathological conditions in multiple sclerosis (MS) and its role in cognitive functioning is also known.

    Objectives And Methods: To study the association between plasma levels and the polymorphic variants of the PAI-1 gene and cognitive performance in MS. Read More

    Abnormal esophageal acid exposure on high-dose proton pump inhibitor therapy is common in systemic sclerosis patients.
    Neurogastroenterol Motil 2017 Nov 6. Epub 2017 Nov 6.
    Division of Gastroenterology and Hepatology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
    Background: Esophageal dysfunction and gastro-esophageal reflux disease (GERD) are common among patients with systemic sclerosis (SSc). Although high-dose proton pump inhibitors (PPIs) typically normalize esophageal acid exposure, the effectiveness of PPI therapy has not been systematically studied in SSc patients. The aim of this study was to characterize reflux in SSc patients on high-dose PPI using esophageal pH-impedance testing. Read More

    Graph Theoretical Framework of Brain Networks in Multiple Sclerosis: A Review of Concepts.
    Neuroscience 2017 Nov 1. Epub 2017 Nov 1.
    Department of Neurology and Neuroimaging Center (NIC) of the Focus Program Translational Neuroscience (FTN), University Medical Center of the Johannes Gutenberg University Mainz, Germany. Electronic address:
    Network science provides powerful access to essential organizational principles of the human brain. It has been applied in combination with graph theory to characterize brain connectivity patterns. In multiple sclerosis (MS), analysis of the brain networks derived from either structural or functional imaging provides new insights into pathological processes within the gray and white matter. Read More

    Pomalidomide in Patients with Interstitial Lung Disease due to Systemic Sclerosis: A Phase II, Multicenter, Randomized, Double-blind, Placebo-controlled, Parallel-group Study.
    J Rheumatol 2017 Nov 1. Epub 2017 Nov 1.
    From the Robert Wood Johnson (RWJ) Medical School Scleroderma Program, New Brunswick; Celgene Corp., Summit, New Jersey; Department of Rheumatology, University of Pittsburgh, Pittsburgh, Pennsylvania; David Geffen School of Medicine at the University of California at Los Angeles (UCLA), Los Angeles, California; Georgetown University Medical Center, Washington, DC, USA; Centre for Rheumatology and Connective Tissue Disease, London, UK; The Queen Elizabeth Hospital and University of Adelaide, Adelaide, Australia; Research Rheumatology Institute n.a. V.A. Nassonova, Moscow, Russia; Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany. Funded by Celgene Corp. V.M. Hsu, MD, RWJ Medical School Scleroderma Program; C.P. Denton, MBBS, MRCP, Centre for Rheumatology and Connective Tissue Disease; R.T. Domsic, MD, MPH, Department of Rheumatology, University of Pittsburgh; D.E. Furst, MD, David Geffen School of Medicine at UCLA; M. Rischmueller, MD, FRACP, The Queen Elizabeth Hospital and University of Adelaide; M. Stanislav, MD, PhD, DMSc, Research Rheumatology Institute n.a. V.A. Nassonova; V.D. Steen, MD, Georgetown University Medical Center; J.H. Distler, MD, Department of Internal Medicine 3, University of Erlangen-Nuremberg; S. Korish, MD, Celgene Corp.; A. Cooper, Celgene Corp.; S. Choi, PhD, Celgene Corp.; P.H. Schafer, PhD, Celgene Corp.; G. Horan, PhD, Celgene Corp.; D.R. Hough, MD, Celgene Corp. Address correspondence to Dr. V.M. Hsu, RWJ Medical School Scleroderma Program, 51 French St., P.O. Box 19, New Brunswick, New Jersey 08903, USA. E-mail: Accepted for publication August 9, 2017.
    Objective: To evaluate the safety and efficacy of pomalidomide (POM) on forced vital capacity (FVC), modified Rodnan skin score (mRSS), and gastrointestinal (GI) symptomatology over 52 weeks of treatment in patients with interstitial lung disease due to systemic sclerosis (SSc).

    Methods: Twenty-three adult patients diagnosed with SSc were randomized 1:1 POM:placebo (PBO).

    Results: Mean change at Week 52 from baseline in predicted FVC% -5. Read More

    Association between cortisol levels and pain threshold in systemic sclerosis and major depression.
    Rheumatol Int 2017 Oct 30. Epub 2017 Oct 30.
    Division of Rheumatology, Department of Medicine, University of Louisville, Kentucky, USA.
    Pain perception and threshold show complex interactions with the inflammatory, psychiatric and neuroendocrine stimuli. This study aims to test whether lower serum cortisol levels are associated with lower pain thresholds and higher degree of depression in systemic sclerosis (SSc) and major depression with atypical features (MD-AF) patients compared to controls. 180 female subjects (SSc = 60, MD-AF = 60, healthy controls = 60) participated in this observational, cross-sectional, parallel group study. Read More

    Body mass index (BMI) and body surface area (BSA) in scleroderma patients.
    J Eur Acad Dermatol Venereol 2017 Oct 28. Epub 2017 Oct 28.
    Department of Dermatology, School of Medicine in Katowice Medical University of Silesia, Poland.
    Dysfunctions of the digestive tract in the course of systemic sclerosis were observed in as many as 90% cases [1]. It causes fluctuations of body composition and consequently lead to body mass disorders. Among the methods of assessing the nutritional condition, the most traditional is BMI (Body Mass Index) technique. Read More

    Patient acceptable symptom state in scleroderma: results from the tocilizumab compared with placebo trial in active diffuse cutaneous systemic sclerosis.
    Rheumatology (Oxford) 2017 Oct 25. Epub 2017 Oct 25.
    Schulich School of Medicine and Dentistry, Western University, London, ON, Canada.
    Objectives: Patient acceptable symptom state (PASS) as an absolute state of well-being has shown promise as an outcome measure in many rheumatologic conditions. We aimed to assess whether PASS may be effective in active diffuse cutaneous SSc differentiating active from placebo.

    Methods: Data from the phase 2 Safety and Efficacy of Subcutaneous Tocilizumab in Adults with Systemic Sclerosis (faSScinate) trial were used, which compared tocilizumab (TCZ) vs placebo over 48 weeks followed by an open-label TCZ period to 96 weeks. Read More

    A systematic review of the epidemiology, disease characteristics and management of systemic sclerosis in Australian adults.
    Int J Rheum Dis 2017 Oct 27. Epub 2017 Oct 27.
    Department of Medicine, The University of Melbourne at St Vincent's Hospital, Melbourne, Vic., Australia.
    Objective: Australia has one of the highest prevalence rates of systemic sclerosis (SSc) worldwide. In order to highlight management deficiencies and key areas for further research, it is essential to understand its local epidemiological patterns, natural history, prognosis and mortality trends over time.

    Methods: To identify Australian SSc-specific information through a systematic review focusing on areas of epidemiology, disease characteristics, treatment, functional ability, disease burden and health-related quality of life (HRQoL). Read More

    Toll-like receptor 9 in systemic sclerosis patients: relation to modified Rodnan skin score, disease severity, and functional status.
    Clin Rheumatol 2017 Oct 26. Epub 2017 Oct 26.
    Clinical and Chemical Pathology Department (Immunology), Faculty of Medicine, Helwan University, Helwan, Egypt.
    The objective of this study is to assess toll-like receptor-9 (TLR9) expression in CD3-positive T lymphocytes and CD19-positive B lymphocytes in systemic sclerosis (SSc) patients and to study their relation to the extent of skin fibrosis, disease characteristics, and severity as well as the functional status. Fifty-five female SSc patients and 30 matched controls were included. Skin thickness was scored according to the modified Rodnan skin score (mRss). Read More

    Belimumab for the Treatment of Early Diffuse Systemic Sclerosis: Results of a Randomized, Double-Blind, Placebo-Controlled, Pilot Trial.
    Arthritis Rheumatol 2017 Oct 26. Epub 2017 Oct 26.
    Department of Rheumatology, Hospital for Special Surgery, New York, NY.
    Objective: To assess the safety and efficacy of treatment with belimumab in patients with early diffuse cutaneous systemic sclerosis (dcSSc) treated with background mycophenolate mofetil (MMF).

    Methods: In this 52-week, investigator-initiated, single-center, double-blind, placebo-controlled, pilot study, 20 patients recently started on MMF were randomized 1:1 to additionally receive belimumab 10 mg/kg intravenously or placebo. We assessed safety, efficacy and differential gene expression (NCT01670565). Read More

    Environmental Pollution by Benzene and PM10 and Clinical Manifestations of Systemic Sclerosis: A Correlation Study.
    Int J Environ Res Public Health 2017 Oct 26;14(11). Epub 2017 Oct 26.
    Institute of Public Health-Hygiene Section, Università Cattolica del Sacro Cuore, 00168 Roma, Italy.
    Atmospheric air pollution has been associated with a range of adverse health effects. The environment plays a causative role in the development of Systemic Sclerosis (SSc). The aim of the present study is to explore the association between particulate (PM10) and benzene (B) exposure in Italian patients with systemic sclerosis and their clinical characteristics of the disease. Read More

    Case Report: Glatiramer Acetate-Induced Serum Sickness.
    Int J MS Care 2017 Sep-Oct;19(5):263-264
    Multiple sclerosis (MS) is a central nervous system demyelinating disease with a prevalence of approximately 400,000 individuals in the United States. Glatiramer acetate is a frequently prescribed disease-modifying therapy used for the management of relapsing forms of the disease. A 40-year-old woman with relapsing-remitting MS presented with symptomatic concerns of vomiting, fever, diffuse rash, joint and low back pain, and distal lower-limb paresthesia and was subsequently admitted to the hospital for investigation and treatment. Read More

    Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate).
    Ann Rheum Dis 2017 Oct 24. Epub 2017 Oct 24.
    University of California, Los Angeles, California, USA.
    Objectives: Assess the efficacy and safety of tocilizumab in patients with systemic sclerosis (SSc) in a phase II study.

    Methods: Patients with SSc were treated for 48 weeks in an open-label extension phase of the faSScinate study with weekly 162 mg subcutaneous tocilizumab. Exploratory end points included modified Rodnan Skin Score (mRSS) and per cent predicted forced vital capacity (%pFVC) through week 96. Read More

    Discovery and pharmacological characterization of a new class of prolyl-tRNA synthetase inhibitor for anti-fibrosis therapy.
    PLoS One 2017 24;12(10):e0186587. Epub 2017 Oct 24.
    Immunology Unit, Pharmaceutical Research Division, Takeda Pharmaceutical Company Limited, Fujisawa, Kanagawa, Japan.
    Scleroderma has clinical characteristics including skin and other tissue fibrosis, but there is an unmet need for anti-fibrotic therapy. Halofuginone (HF) is a well-known anti-fibrosis agent in preclinical and clinical studies which exerts its effect via inhibition of TGF-β/Smad3 signaling pathway. Recently, prolyl-tRNA synthetase (PRS) was elucidated as a target protein for HF that binds to the proline binding site of the catalytic domain of PRS. Read More

    Toll-Like Receptor-4 Signaling Drives Persistent Fibroblast Activation and Prevents Fibrosis Resolution in Scleroderma.
    Adv Wound Care (New Rochelle) 2017 Oct;6(10):356-369
    Northwestern Scleroderma Program, Feinberg School of Medicine, Chicago, Illinois.
    Significance: This review provides current overview of the emerging role of innate immunity in driving fibrosis, and preventing its resolution, in scleroderma (systemic sclerosis, SSc). Understanding the mechanisms of dysregulated innate immunity in fibrosis and SSc will provide opportunities for therapeutic interventions using novel agents and repurposed existing drugs. Recent Advances: New insights from genomic and genetic studies implicate components of innate immune signaling such as pattern recognition receptors (PRRs), downstream signaling intermediates, and endogenous inhibitors, in fibrosis in SSc. Read More

    Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort.
    Ann Rheum Dis 2017 Oct 23. Epub 2017 Oct 23.
    Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
    Objective: Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc). In this longitudinal study, we aimed to identify factors associated with an unfavourable outcome in patients with SSc with early PAH (SSc-PAH) from the DETECT cohort.

    Methods: Patients with SSc-PAH enrolled in DETECT were observed for up to 3 years. Read More

    The involvement of galectin-3 in skin injury in systemic lupus erythematosus patients.
    Lupus 2017 Jan 1:961203317736144. Epub 2017 Jan 1.
    1 Department of Dermatology, 56713 Sun Yat-sen Memorial Hospital , Guangzhou, China.
    Objective Our previous research suggested that anti-galectin-3 antibody was highly associated with the development of lupus skin lesions in systemic lupus erythematosus (SLE). In this study we aimed to investigate the involvement of galectin-3 in SLE skin damage. Methods The study consisted of 49 patients with SLE, 16 with dermatomyositis and 11 with systemic scleroderma and 20 healthy controls. Read More

    Combined platelet-rich plasma and lipofilling treatment provides great improvement in facial skin-induced lesion regeneration for scleroderma patients.
    Stem Cell Res Ther 2017 Oct 23;8(1):236. Epub 2017 Oct 23.
    Cellular and Molecular Pathophysiology Laboratory, Department of Surgical Oncological and Stomatological Sciences, University of Palermo, Via del Vespro 131, Palermo, 90127, Italy.
    Background: The use of stem cells, including mesenchymal stem cells (MSCs), for regenerative medicine is gaining interest for the clinical benefits so far obtained in patients. This study investigates the use of adipose autologous tissue in combination with platelet-rich plasma (PRP) to improve the clinical outcome of patients affected by systemic sclerosis (SSc).

    Methods: Adipose-derived mesenchymal stem cells (AD-MSCs) and PRPs were purified from healthy donors and SSc patients. Read More

    PTP4A1 promotes TGFβ signaling and fibrosis in systemic sclerosis.
    Nat Commun 2017 Oct 20;8(1):1060. Epub 2017 Oct 20.
    Division of Rheumatology, Allergy and Immunology, Department of Medicine, University of California San Diego, 9500 Gilman Dr, La Jolla, CA, 92093, USA.
    Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and internal organs. Protein tyrosine phosphatases have received little attention in the study of SSc or fibrosis. Here, we show that the tyrosine phosphatase PTP4A1 is highly expressed in fibroblasts from patients with SSc. Read More

    Assessment of hospitalization and mortality of scleroderma in-patients: a thirteen-year study.
    Reumatologia 2017 31;55(4):163-168. Epub 2017 Aug 31.
    Division of Rheumatology, Departments of Internal Medicine, Shiraz University of Medical Sciences, Iran.
    Objective: Systemic sclerosis (SSc) is an uncommon non-hereditary sporadic disease that increases the risk of premature death, especially in diffuse type. We determined the prevalence of SSc in the last 13 years in our rheumatologic hospitals as a referral center for southern Iranian patients, the causes of hospitalization, the average length of stay (LOS), the mortality rate, and the reason for their mortality.

    Material And Methods: A cross-sectional study was performed in Shiraz University of Medical Sciences, Iran. Read More

    In vivo characterization of cortical and white matter neuroaxonal pathology in early multiple sclerosis.
    Brain 2017 Nov;140(11):2912-2926
    Athinoula A. Martinos Center for Biomedical Imaging, Department of Radiology, Massachusetts General Hospital, Charlestown, MA, USA.
    Neuroaxonal pathology is a main determinant of disease progression in multiple sclerosis; however, its underlying pathophysiological mechanisms, including its link to inflammatory demyelination and temporal occurrence in the disease course are still unknown. We used ultra-high field (7 T), ultra-high gradient strength diffusion and T1/T2-weighted myelin-sensitive magnetic resonance imaging to characterize microstructural changes in myelin and neuroaxonal integrity in the cortex and white matter in early stage multiple sclerosis, their distribution in lesional and normal-appearing tissue, and their correlations with neurological disability. Twenty-six early stage multiple sclerosis subjects (disease duration ≤5 years) and 24 age-matched healthy controls underwent 7 T T2*-weighted imaging for cortical lesion segmentation and 3 T T1/T2-weighted myelin-sensitive imaging and neurite orientation dispersion and density imaging for assessing microstructural myelin, axonal and dendrite integrity in lesional and normal-appearing tissue of the cortex and the white matter. Read More

    Neuroinflammation and its relationship to changes in brain volume and white matter lesions in multiple sclerosis.
    Brain 2017 Nov;140(11):2927-2938
    Division of Brain Sciences, Imperial College London, UK.
    Brain magnetic resonance imaging is an important tool in the diagnosis and monitoring of multiple sclerosis patients. However, magnetic resonance imaging alone provides limited information for predicting an individual patient's disability progression. In part, this is because magnetic resonance imaging lacks sensitivity and specificity for detecting chronic diffuse and multi-focal inflammation mediated by activated microglia/macrophages. Read More

    Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity.
    J Investig Med High Impact Case Rep 2017 Oct-Dec;5(4):2324709617734012. Epub 2017 Oct 10.
    Eastern Virginia Medical School, Norfolk, VA, USA.
    Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. Read More

    [Changes of serum Krebs von den Lungen-6 levels in interstitial lung disease associated with dermatomyositis and secondary Sjögren's syndrome: a case report].
    Beijing Da Xue Xue Bao Yi Xue Ban 2017 Oct;49(5):910-914
    Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China.
    Interstitial lung diseases (ILDs) are a diverse group of pulmonary disorders characterized by various patterns of inflammation and fibrosis in the interstitium of the lung. The underlying pathogenesis of ILDs is complex and associated with multiple rheumatologic conditions, such as systemic sclerosis, rheumatoid arthritis, pollymyositis and dermatomyositis, Sjögren's syndrome, and systemic lupus erythematosus. As the disease progresses, excessive pulmonary fibrosis impairs alveolar gas exchange and damages pulmonary function. Read More

    Possible pro-inflammatory role of heparin-binding epidermal growth factor-like growth factor in the active phase of systemic sclerosis.
    J Dermatol 2017 Oct 17. Epub 2017 Oct 17.
    Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan.
    Heparin-binding epidermal growth factor (EGF)-like growth factor (HB-EGF) is a member of the EGF family growth factors, which affects multiple aspects of the wound healing process such as epithelialization, wound contraction and angiogenesis. In our study, we measured the serum HB-EGF levels of 51 systemic sclerosis (SSc) patients, which showed a significant increase compared with those of 20 normal subjects. Further analysis revealed a positive correlation between the HB-EGF level and pulmonary ground-glass score but no correlation between the former and pulmonary fibrosis score. Read More

    Rheopheresis as a causal therapy option for systemic scleroderma (SSc).
    Clin Hemorheol Microcirc 2017 Oct 7. Epub 2017 Oct 7.
    Department of Dermatology, University Medicine Greifswald, Greifswald, Germany.
    A complex pathomechanism accounts systemic sclerosis as a form of collagenosis. A triad of vasculopathy, autoinflammation, and dysbalance of the fibroblast function can be seen as cause, as well as symptomatic appearance. Comparative with other collagenoses, e. Read More

    Cardio-pulmonary involvement in systemic sclerosis: A study at a tertiary care center.
    Indian J Dermatol Venereol Leprol 2017 Nov-Dec;83(6):677-682
    Department of DVL, Gandhi Medical College and Hospital, Hyderabad, Telangana, India.
    Background: Systemic sclerosis is a multisystem disorder characterized by microangiopathy, dysregulation of the immune system and massive deposition of collagen in the connective tissue of the skin, blood vessels and various internal organs. Although the mortality from renal crises has dropped significantly due to the use of angiotensin-converting enzyme inhibitors, cardiac and pulmonary involvement accounts for significant morbidity and mortality. We studied 28 patients with systemic sclerosis at Gandhi Medical College and Hospital, Hyderabad, over a period of two years for cardiopulmonary involvement. Read More

    Head and Neck Lymphoma in an Iranian Population.
    Iran J Otorhinolaryngol 2017 Sep;29(94):261-267
    Department of Oral and Maxillofacial Medicine, School of Dentistry, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
    Introduction: This study is aimed to assess the prevalence and characteristics of head and neck lymphoma in a defined group of an Iranian population.

    Materials And Methods: In this retrospective study, 126,450 biopsy reports from two referral Pathology Departments, (Tehran, the capital of Iran) were evaluated. In cases with head and neck lymphoma, other variables such as age, sex, specific location of lesions, and histopathological findings were recorded. Read More

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