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    HGF-Met Pathway in Regeneration and Drug Discovery.
    Biomedicines 2014 Oct 31;2(4):275-300. Epub 2014 Oct 31.
    Division of Tumor Dynamics and Regulation, Cancer Research Institute, Kanazawa University, Kanazawa 920-1192, Japan.
    Hepatocyte growth factor (HGF) is composed of an α-chain and a β-chain, and these chains contain four kringle domains and a serine protease-like structure, respectively. Activation of the HGF-Met pathway evokes dynamic biological responses that support morphogenesis (e.g. Read More

    Lung Density and Pulmonary Artery Diameter are Predictors of Pulmonary Hypertension in Systemic Sclerosis.
    J Thorac Imaging 2017 May 25. Epub 2017 May 25.
    Departments of *Radiology, Division of Image Processing †Pulmonology ‡Radiology §Rheumatology ¶Cardiology, Leiden University Medical Center, Leiden ∥Haga Teaching Hospital, The Hague, The Netherlands.
    Purpose: The aim was to evaluate computed tomography (CT)-measured pulmonary artery diameter (PAD) and lung density as predictors of pulmonary hypertension (PH) in subjects with systemic sclerosis (SSc). We compared these PAD values with normal values and between SSc subgroups with PH and/or interstitial lung disease (ILD). We investigated whether PAD predicts PH and whether lung densitometry, by using the 85th percentile density value (Perc85) as a measure for ILD, can predict PH. Read More

    Haematological Malignancies in Systemic Sclerosis Patients: Case Reports and Review of the World Literature.
    Case Rep Rheumatol 2017 4;2017:6230138. Epub 2017 May 4.
    Rheumatology Unit, Azienda Ospedaliero-Universitaria Policlinico di Modena, University of Modena and Reggio Emilia, Modena, Italy.
    Background. The association of systemic sclerosis (SSc) and haematological cancers was reported in a large number of case reports and cohort studies, describing SSc patients with highly heterogeneous clinical pictures. Objective. Read More

    Cardiovascular magnetic resonance imaging: clinical implications in the evaluation of connective tissue diseases.
    J Inflamm Res 2017 11;10:55-61. Epub 2017 May 11.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Cardiovascular magnetic resonance imaging is a recently developed noninvasive, nonradiating, operator-independent technique that has been successfully used for the evaluation of congenital heart disease, valvular and pericardial diseases, iron overload, cardiomyopathies, great and coronary vessel diseases, cardiac inflammation, stress-rest myocardial perfusion, and fibrosis. Rheumatoid arthritis and other spondyloarthropathies, systemic lupus erythematosus, inflammatory myopathies, mixed connective tissue diseases (CTDs), systemic sclerosis, vasculitis, and sarcoidosis are among CTDs with serious cardiovascular involvement; this is due to multiple causative factors such as myopericarditis, micro/macrovascular disease, coronary artery disease, myocardial fibrosis, pulmonary hypertension, and finally heart failure. The complicated pathophysiology and the high cardiovascular morbidity and mortality of CTDs demand a versatile, noninvasive, nonradiative diagnostic tool for early cardiovascular diagnosis, risk stratification, and treatment follow-up. Read More

    [Screening for pulmonary arterial hypertension in patients with systemic sclerosis: Comparison of DETECT algorithm to decisions of a multidisciplinary team, in a competence centre].
    Rev Med Interne 2017 May 22. Epub 2017 May 22.
    Service de médecine interne, hôpital Sud, CHU de Rennes, BP 90347, 16, boulevard de Bulgarie, 35203 Rennes cedex 2, France; UMR Inserm U1085, institut de recherche sur la santé, l'environnement et le travail (IRSET), université de Rennes 1, 35203 Rennes, France.
    Introduction: Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis and detecting PAH efficiently remains challenging. The DETECT study has offered in 2013 a composite screening tool for PAH. The objective of our study was to compare the indication of right heart catheterisation (RHC) as suggested by the DETECT algorithm with the decisions of a multidisciplinary team. Read More

    Tie2 as a novel key factor of microangiopathy in systemic sclerosis.
    Arthritis Res Ther 2017 May 25;19(1):105. Epub 2017 May 25.
    Department of Rheumatology, University Hospital Zurich, Gloriastrasse 25, 8091, Zurich, Switzerland.
    Background: The angiopoietin(Ang)/Tie2 system is a key regulator of vascular biology. The expression of membrane bound (mb) Tie2 and Ang-1 ensures vessel stability, whereas Ang-2, inducible by vascular endothelial growth factor (VEGF), hypoxia, and inflammation, acts as an antagonist. Tie2 signalling is also attenuated by soluble Tie2 (sTie2), the extracellular domain of the receptor, which is shed upon stimulation with VEGF. Read More

    Neuro-ophthalmic sarcoidosis.
    Eye Brain 2012 13;4:13-25. Epub 2012 Mar 13.
    Department of Ophthalmology, University of Cincinnati Medical Center, Cincinnati, Ohio, USA.
    Neuro-ophthalmic disease occurs in about a third of patients with neurosarcoidosis. Optic nerve involvement is the most common manifestation, but other cranial nerves and the optic chiasm can be involved. However, there are several other common diseases that cause optic neuropathy, including multiple sclerosis. Read More

    Fibrosing myopathy in systemic sclerosis associates with higher mortality.
    Arthritis Care Res (Hoboken) 2017 May 23. Epub 2017 May 23.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
    Objective: To determine if a unique subtype of scleroderma muscle disease exists by comparing the clinical features of systemic sclerosis (SSc) patients with predominant fibrosis on muscle biopsy to those with inflammatory muscle histopathology.

    Methods: This retrospective, cross-sectional study included SSc patients with muscle weakness and an available muscle biopsy. Biopsies with fibrosis but without inflammation/necrosis were designated as "fibrosing myopathy" and those with inflammation and/or necrosis were assigned a category of "inflammatory myopathy". Read More

    Performance of Anti-nuclear Antibodies for Classifying Systemic Lupus Erythematosus: a Systematic Literature Review and Meta-regression of Diagnostic Data.
    Arthritis Care Res (Hoboken) 2017 May 23. Epub 2017 May 23.
    Rheumatology, Clinical Immunology and Allergy, University of Crete Medical School, Heraklion, Greece.
    Objective: To review the published literature on the performance of indirect immunofluorescence (IIF)-HEp-2 ANA testing for classification of SLE.

    Methods: A systematic literature search was conducted in the MEDLINE, EMBASE and COCHRANE databases for articles published between January 1990 and October 2015. The research question was structured according to PICO (Population, Intervention, Comparator, Outcome) format rules, and PRISMA recommendations were followed where appropriate. Read More

    Efficacy of mycophenolate mofetil and oral cyclophosphamide on skin thickness: Post-hoc analyses from the Scleroderma Lung Study I and II.
    Arthritis Care Res (Hoboken) 2017 May 23. Epub 2017 May 23.
    Division of Rheumatology, University of Michigan Scleroderma Program, Ann Arbor, MI, USA.
    Objectives: To assess the efficacy of mycophenolate mofetil (MMF) and cyclophosphamide (CYC) on the modified Rodnan skin score (mRSS) in participants enrolled in the Scleroderma Lung Study (SLS)-I and II.

    Methods: SLS-I participants received daily oral CYC or matching placebo for one year, whereas SLS-II participants received daily MMF for 2 years or daily oral CYC for 1 year followed by placebo for second year. We assessed the impact of MMF and CYC on the mRSS in SLS-II over 24-month period. Read More

    Cough is less common and less severe in systemic sclerosis-associated interstitial lung disease compared to other fibrotic interstitial lung diseases.
    Respirology 2017 May 23. Epub 2017 May 23.
    Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
    Background And Objective: The objectives of this study were to determine the prevalence and characteristics of cough in idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis (HP) and systemic sclerosis-associated interstitial lung disease (SSc-ILD).

    Methods: Cough severity was measured in consecutive patients with IPF (n = 77), HP (n = 32) and SSc-ILD (n = 67) using a 10-cm visual analogue scale (VAS). Dyspnoea and quality of life were measured using established questionnaires. Read More

    Circadian clustering of spontaneous epileptic seizures emerges after pilocarpine-induced status epilepticus.
    Epilepsia 2017 May 24. Epub 2017 May 24.
    Unit of Epileptology and Experimental Neurophysiology, Fondazione Istituto Neurologico Carlo Besta, Milan, Italy.
    Objective: Seizures in mesial temporal lobe epilepsy (MTLE) associated with hippocampal sclerosis are thought to develop with various latency intervals after an initial transient brain insult. To study seizure dynamics after an initial transient precipitating insult in a systematic fashion, we utilized continuous video-electroencephalography (EEG) monitoring after the induction of status epilepticus (SE) in a mouse MTLE model.

    Methods: Continuous 24/7 video/telemetric hippocampal EEG recordings in the systemic pilocarpine MTLE mouse model. Read More

    Lung or Heart-Lung Transplant in Pulmonary Arterial Hypertension: What Is the Impact of Systemic Sclerosis?
    Exp Clin Transplant 2017 May 22. Epub 2017 May 22.
    From the Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, Ohio, USA.
    Objectives: Little is known about recipient characteristics and outcomes of patients with pulmonary arterial hypertension undergoing lung transplant, particularly those with systemic sclerosis-associated disease. Here, we describe the characteristics and outcomes of patients with pulmonary arterial hypertension undergoing lung transplant, focusing on systemic sclerosis-associated disease.

    Materials And Methods: This retrospective study included pulmonary arterial hypertension patients undergoing lung or heart-lung transplant between July 1992 and August 2013 at a single center. Read More

    FGF-23, Klotho and Vitamin D Levels in Scleroderma.
    Iran J Public Health 2017 Apr;46(4):530-536
    Dept. of Clinical Biochemistry and Laboratory Medicine, Tabriz University of Medical Sciences, Tabriz, Iran.
    Background: Scleroderma is a chronic connective tissue disease of unknown etiology. Vitamin D and parathyroid hormone (PTH) that play particular functions in calcium and phosphate homeostasis may be involved in the etiology of this disorder. Klotho, the co-receptor of the fibroblast growth factor 23 (FGF-23), can interfere with calcium and phosphate metabolism. Read More

    Optic neuritis as an early sign of multiple sclerosis.
    Eye Brain 2016 26;8:195-202. Epub 2016 Oct 26.
    Department of Neurology, Bakirkoy Prof Dr Mazhar Osman Training and Research Hospital, Istanbul, Turkey.
    Optic neuritis (ON) is an acute inflammatory demyelinating disorder of the optic nerve. The general characteristics of isolated ON include unilateral, subacute, and painful visual loss without systemic or other neurological symptoms. The etiology for ON varies including demyelinating disorders or infections, inflammation, toxic reasons, and genetic disorders. Read More

    Coexistence of diabetes mellitus type 1 with diffuse systemic sclerosis - case report and literature review.
    Reumatologia 2017 28;55(2):104-107. Epub 2017 Apr 28.
    Department of Rheumatology and Connective Tissue Diseases, Medical University, Lublin, Poland.
    Diabetic sclerodactyly is a frequently recognized skin finding that may occur in patients with diabetes mellitus but coexistence of diabetes and systemic sclerosis is rare. We describe a case of coexistence of type 1 diabetes mellitus and systemic sclerosis in 42-year-old man with the history of Raynaud's phenomenon, progressive diffuse hardening of the skin and sclerodactyly, slowly worsening with time. The medical history included type 1 diabetes since childhood with microvascular complications. Read More

    Stubborn rectal prolapse in systemic sclerosis.
    Reumatologia 2017 28;55(2):100-103. Epub 2017 Apr 28.
    Department of Dermatology and Allergology Hospital Dresden-Friedrichstadt, Dresden, Germany.
    Systemic sclerosis (SSc) is an autoimmune connective tissue disorder. Anorectal involvement might typically cause fecal incontinence and rarely rectal prolapse. Here we report three female patients, who were admitted with a mean history of 10 years suffering from SSc. Read More

    Functionalisation of Polyvinylpyrrolidone on Gold Nanoparticles Enhances Its Anti-Amyloidogenic Propensity towards Hen Egg White Lysozyme.
    Biomedicines 2017 May 3;5(2). Epub 2017 May 3.
    Department of Biotechnology and Medical Engineering, National Institute of Technology Rourkela, Odisha 769008, India.
    Protein amyloids are characterized by aggregates that usually consist of fibres containing misfolded proteins and having a cross β-sheet conformation. These aggregates can eventually lead to several degenerative diseases like Alzheimer's disease, amyotrophic lateral sclerosis (ALS), Huntington's disease and Parkinson's disease. The present study describes the effect of chemically synthesized polyvinylpyrrolidone (PVP)-conjugated gold nanoparticles (PVP-AuNps) on hen egg white lysozyme (HEWL) amyloids. Read More

    Association between demyelinating disease and autoimmune rheumatic disease in a pediatric population.
    Rev Bras Reumatol Engl Ed 2017 May - Jun;57(3):224-228. Epub 2016 Oct 25.
    Universidade Federal de São Paulo (Unifesp), Departamento de Pediatria, Setor de Reumatologia Pediátrica, São Paulo, SP, Brazil. Electronic address:
    Introduction: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. Read More

    Genotype and haplotype analysis of ABCB1 at 1236, 2677 and 3435 among systemic sclerosis patients.
    Autoimmunity 2017 May 23:1-6. Epub 2017 May 23.
    b Department of Dermatology and Venereology, Chair of Dermatology and Venereology , Medical University of Lodz , Łódź, Poland.
    Systemic sclerosis (SSc) belongs to the group of systemic diseases of the connective tissue, which are characterized by a chronic autoimmune inflammatory process. P-glycoprotein, initially associated with the drug resistance in patients with cancer, becomes more and more often a subject of considerations in terms of its significance in the development of illnesses, including autoimmune diseases. The aim of the study was an attempt to answer the question whether there was a relationship between ABCB1 polymorphisms and morbidity of systemic sclerosis in a Polish population. Read More

    Serum prolidase activity in systemic sclerosis.
    Clin Rheumatol 2017 May 22. Epub 2017 May 22.
    Medical Faculty, Department of Medical Biochemistry, Sutcu Imam University, Kahramanmaras, Turkey.
    Systemic sclerosis, also known as scleroderma, is a complex systemic inflammatory autoimmune disease that targets the vasculature and connective tissue-producing cells and components of the innate and adaptive immune systems. The disease is characterized by a hardening of the skin and an increased synthesis of collagen . Prolidase is a specific imidodipeptidase involved in collagen degradation. Read More

    Echocardiographic Predictors for Worsening of Six-Minute Walk Distances in Patients With Systemic Sclerosis (Scleroderma).
    Am J Cardiol 2017 Apr 26. Epub 2017 Apr 26.
    Department of Cardiovascular Medicine, Tokushima University Hospital, Tokushima, Japan.
    Change in 6-minute walk distance (6MWD) has been used as a clinical marker in pulmonary hypertension. Determinants and worsening of 6MWD remain a matter of debate because nonpulmonary factors have an impact on the 6MWD. We hypothesized that future reduction of 6MWD in patients with systemic sclerosis (SSc) was more closely associated with cardiac dysfunction. Read More

    Sirt1 ameliorates systemic sclerosis by targeting the mTOR pathway.
    J Dermatol Sci 2017 May 3. Epub 2017 May 3.
    Division of Rheumatology, Huashan Hospital, Fudan University, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, China. Electronic address:
    Background: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by inflammation and fibrosis. Our previous research has indicated that Sirtuin1 (Sirt1) plays a role in the regulation of TNF-α-induced inflammation; however, whether Sirt1 may inhibit the progress of SSc by blocking inflammation remains unknown.

    Objective: We aimed to investigate the function of Sirt1 in SSc. Read More

    Fetal and neonatal involvement in maternal rheumatologic disease.
    J Matern Fetal Neonatal Med 2017 May 22:1-22. Epub 2017 May 22.
    a Department of Pediatrics , Hamamatsu University School of Medicine , Shizuoka , Japan.
    A pregnancy complicated with rheumatologic diseases can have various influences on the fetus and/or neonate. Maternal systemic lupus erythematosus (SLE) may cause preterm and/or small for gestational age (SGA) delivery and neonatal lupus (NL). Some neonates with NL have congenital heart block (CHB) with increased morbidity and mortality, even requiring pacemakers. Read More

    Cardiopulmonary assessment of patients with systemic sclerosis for hematopoietic stem cell transplantation: recommendations from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party and collaborating partners.
    Bone Marrow Transplant 2017 May 22. Epub 2017 May 22.
    Department of Haematology, Sheffield Teaching Hospitals NHS Foundation Trust, University of Sheffield, Sheffield, UK.
    Systemic sclerosis (SSc) is a rare disabling autoimmune disease with a similar mortality to many cancers. Two randomized controlled trials of autologous hematopoietic stem cell transplantation (AHSCT) for SSc have shown significant improvement in organ function, quality of life and long-term survival compared to standard therapy. However, transplant-related mortality (TRM) ranged from 3-10% in patients undergoing HSCT. Read More

    Systemic sclerosis dermal fibroblasts suppress Th1 cytokine production via galectin-9 overproduction due to Fli1 deficiency.
    J Invest Dermatol 2017 May 18. Epub 2017 May 18.
    Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan.
    Dermal fibroblasts promote skin-localized transdifferentiation of Tregs to Th2-like cells in systemic sclerosis (SSc). However, the entire effect of SSc dermal fibroblasts on immune cells still remains unknown. Since galectin-9 induces Th2 cytokine-predominant immune imbalance by negatively regulating Th1/Th17 cells in inflammatory diseases, we investigated the contribution of galectin-9 to Th immune balance in SSc lesional skin. Read More

    Myeloid Populations in Systemic Autoimmune Diseases.
    Clin Rev Allergy Immunol 2017 May 20. Epub 2017 May 20.
    Genomic Medicine Department, GENYO, Centre for Genomics and Oncological Research: Pfizer / University of Granada / Andalusian Regional Government, PTS Granada, Avda de la Ilustración 114, 18016, Granada, Spain.
    Systemic autoimmune diseases (SADs) encompass a wide spectrum of clinical signs as a reflection of their complex physiopathology. A variety of mechanisms related with the innate immune system are in the origin of the loss of self-tolerance in these diseases, and for most of them, the myeloid leukocytes are key actors. Monocytes, macrophages, dendritic cells, and neutrophils are first-line immune effectors located in the interface between innate and adaptive immunity. Read More

    Increased proportions of functionally impaired regulatory T cell subsets in systemic sclerosis.
    Clin Immunol 2017 May 15. Epub 2017 May 15.
    Department of Immunology and Biotechnology, University of Pécs, Clinical Center, Szigeti út 12, 7624 Pécs, Hungary. Electronic address:
    Treg abnormalities have been implicated in the pathogenesis of systemic sclerosis (SSc). Treg subpopulations and their cytokines, IL-10 and TGF-β in the peripheral blood of early stage SSc patients were investigated. We hypothesized that epigenetically regulated methylation of the FOXP3 promoter and enhancer regions are altered in Tregs of SSc patients, which might be involved in the T cell imbalance. Read More

    Cardiovascular magnetic resonance in systemic sclerosis: "Pearls and pitfalls".
    Semin Arthritis Rheum 2017 Mar 31. Epub 2017 Mar 31.
    Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; Department of Geriatric Medicine, AOUC, Florence, Italy.
    Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular dysfunction and excessive fibrosis, involving internal organs including the heart. The estimated prevalence of cardiac involvement in SSc is high and remains subclinical until the late stages. It is either primary, related to myocardial inflammation and fibrosis, or secondary, due to pulmonary arterial hypertension (SSc-PAH) or systemic hypertension, in those patients with renal involvement. Read More

    Patient participation in patient-reported outcome instrument development in systemic sclerosis.
    Clin Exp Rheumatol 2017 May 2. Epub 2017 May 2.
    Jewish General Hospital, Lady Davis Institute and McGill University, Montreal, Canada.
    Objectives: The patient perspective captured using Patient-Reported Outcome (PRO) instruments provide insight into the patient condition not always captured by physician-derived assessment tools. Target patient population involvement is an essential component of PRO instrument development. We have reviewed the level of patient involvement in the development of PRO instruments used in the assessment of systemic sclerosis (SSc). Read More

    Validity of the Workers Productivity and Activity Impairment Questionnaire: Specific Health Problem (WPAI:SHP) in patients with systemic sclerosis.
    Clin Exp Rheumatol 2017 May 8. Epub 2017 May 8.
    Department of Medicine, The University of Melbourne at St. Vincent's Hospital; and Department of Rheumatology, St. Vincent's Hospital, Melbourne, Australia.
    Objectives: To evaluate the construct validity of the Workers Productivity and Impairment Activity Index: Specific Health Problem (WPAI:SHP) in Australian systemic sclerosis (SSc) patients.

    Methods: SSc patients, identified through the Australian Scleroderma Cohort Study database, completed the WPAI:SHP and a quality of life instrument (PROMIS-29) cross-sectionally. The construct validity of the WPAI:SHP was assessed by the correlations between the WPAI:SHP and a range of SSc health states. Read More

    Reply to: Moyamoya disease and systemic sclerosis (MoSys syndrome): a combination of two rare entities: comment to the authors.
    Clin Exp Rheumatol 2017 Apr 26. Epub 2017 Apr 26.
    Department of Internal Medicine and Rheumatology, Justus-Liebig-University, Giessen, and Kerckhoff-Klinik, Bad Nauheim, Germany.
    ****************************************************************************. Read More

    A comprehensive guide to telocytes and their great potential in cardiovascular system.
    Bratisl Lek Listy 2017 ;118(5):302-309
    Telocytes, a recently discovered type of interstitial cells, have a very distinctive morphology - the small cell body with long extensions, named telopodes. In our review, apart from introducing general aspects of telocytes, we focus on properties, functions and future potential of those cells in cardiovascular system. However, physiological functions of telocytes in cardiovascular system are still regarded as quite enigmatic. Read More

    A Comparison of Neuroimaging Abnormalities in Multiple Sclerosis, Major Depression and Chronic Fatigue Syndrome (Myalgic Encephalomyelitis): is There a Common Cause?
    Mol Neurobiol 2017 May 17. Epub 2017 May 17.
    Department of Medicine, Imperial College London, Hammersmith Hospital, London, England, W12 0HS, UK.
    There is copious evidence of abnormalities in resting-state functional network connectivity states, grey and white matter pathology and impaired cerebral perfusion in patients afforded a diagnosis of multiple sclerosis, major depression or chronic fatigue syndrome (CFS) (myalgic encephalomyelitis). Systemic inflammation may well be a major element explaining such findings. Inter-patient and inter-illness variations in neuroimaging findings may arise at least in part from regional genetic, epigenetic and environmental variations in the functions of microglia and astrocytes. Read More

    [Skin diseases associated with environmental factors].
    Bundesgesundheitsblatt Gesundheitsforschung Gesundheitsschutz 2017 May 17. Epub 2017 May 17.
    Abteilung Allergologie, Paul-Ehrlich-Institut, Paul-Ehrlich-Str. 51-59, 63225, Langen, Deutschland.
    Background: Multiple environmental exposures may derange the regulatory and repair mechanisms of the skin and lead to dermatological disease.

    Objectives: Provide an overview of non-allergic skin diseases associated with environmental factors.

    Materials And Methods: Review of current scientific evidence for associations of non-allergic skin diseases with environmental exposures: irritation, chemicals, infection, UV-radiation, temperature. Read More

    Standardization of the modified Rodnan skin score for use in clinical trials of systemic sclerosis.
    J Scleroderma Relat Disord 2017 Jan-Apr;2(1):11-18
    Centre for Rheumatology, Royal Free and University College London Medical School, London, UK.
    The modified Rodnan skin score (mRSS) is a measure of skin thickness and is used as a primary or secondary outcome measure in clinical trials of systemic sclerosis (scleroderma). This state-of-art review provides a historical perspective of the development of the mRSS, summarizes the performance of mRSS as an outcome measure, provides guidance on assessing mRSS, and makes recommendations for incorporation of the mRSS into clinical trials. Read More

    CXCL4 Exposure Potentiates TLR-Driven Polarization of Human Monocyte-Derived Dendritic Cells and Increases Stimulation of T Cells.
    J Immunol 2017 May 17. Epub 2017 May 17.
    Laboratory of Translational Immunology, University Medical Center Utrecht, 3584 EA Utrecht, the Netherlands;
    Chemokines have been shown to play immune-modulatory functions unrelated to steering cell migration. CXCL4 is a chemokine abundantly produced by activated platelets and immune cells. Increased levels of circulating CXCL4 are associated with immune-mediated conditions, including systemic sclerosis. Read More

    A systematic review of overlapping microRNA patterns in systemic sclerosis and idiopathic pulmonary fibrosis.
    Eur Respir Rev 2017 Jun 17;26(144). Epub 2017 May 17.
    Division of Allergy and Clinical Immunology, Dept of Clinical and Experimental Medicine, University of Messina, Messina, Italy.
    Lung fibrosis can be observed in systemic sclerosis and in idiopathic pulmonary fibrosis, two disorders where lung involvement carries a poor prognosis. Although much has been learned about the pathogenesis of these conditions, interventions capable of reversing or, at the very least, halting disease progression are not available. Recent studies point to the potential role of micro messenger RNAs (microRNAs) in cancer and tissue fibrogenesis. Read More

    Malignancy as a comorbidity in rheumatic diseases: a retrospective hospital-based study.
    Clin Rheumatol 2017 May 16. Epub 2017 May 16.
    Division of Oncology, Guang An Men Hospital affiliated to China Academy of Traditional Chinese Medicine, Beijing, 100053, China.
    Patients with Rheumatic diseases (RDs) are at an increased risk of malignancies compared with the general population. The aim of this study was to examine the relative frequency of several cancers in a single homogeneous cohort of patients with different RDs. Patients diagnosed with rheumatoid arthritis (RA), Ankylosing spondylitis (AS), Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermatomyositis (DM), or polymyositis were included. Read More

    Fli1 Deficiency Induces CXCL6 Expression in Dermal Fibroblasts and Endothelial Cells, Contributing to the Development of Fibrosis and Vasculopathy in Systemic Sclerosis.
    J Rheumatol 2017 May 15. Epub 2017 May 15.
    From the Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. Supported by a grant for Research on Intractable Diseases from the Ministry of Health, Labor, and Welfare of Japan. T. Taniguchi, MD, PhD, Assistant Professor, Department of Dermatology, University of Tokyo Graduate School of Medicine; Y. Asano, MD, PhD, Associate Professor, Department of Dermatology, University of Tokyo Graduate School of Medicine; K. Nakamura, MD, Graduate Student, Department of Dermatology, University of Tokyo Graduate School of Medicine; T. Yamashita, MD, Graduate Student, Department of Dermatology, University of Tokyo Graduate School of Medicine; R. Saigusa, MD, Graduate Student, Department of Dermatology, University of Tokyo Graduate School of Medicine; Y. Ichimura, MD, PhD, Assistant Professor, Department of Dermatology, University of Tokyo Graduate School of Medicine; T. Takahashi, MD, PhD, Assistant Professor, Department of Dermatology, University of Tokyo Graduate School of Medicine; T. Toyama, MD, PhD, Assistant Professor, Department of Dermatology, University of Tokyo Graduate School of Medicine; A. Yoshizaki, MD, PhD, Lecturer, Department of Dermatology, University of Tokyo Graduate School of Medicine; S. Sato, MD, PhD, Professor, Department of Dermatology, University of Tokyo Graduate School of Medicine. Address correspondence to Dr. Y. Asano, Department of Dermatology, University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. E-mail: Full Release Article. For details see Reprints and Permissions at Accepted for publication April 17, 2017.
    Objective: CXCL6, a chemokine with proangiogenic property, is reported to be involved in vasculopathy associated with systemic sclerosis (SSc). We investigated the contribution of CXCL6 to SSc development by focusing on the association of friend leukemia virus integration 1 (Fli1) deficiency, a potential predisposing factor of SSc, with CXCL6 expression and clinical correlation of serum CXCL6 levels.

    Methods: mRNA levels of target genes and the binding of Fli1 to the CXCL6 promoter were evaluated by quantitative reverse transcription-PCR and chromatin immunoprecipitation, respectively. Read More

    Evidence for a Derangement of the Microvascular System in Patients with a Very Early Diagnosis of Systemic Sclerosis.
    J Rheumatol 2017 May 15. Epub 2017 May 15.
    From the Department of Internal Medicine, São João Hospital Center, Al Prof Hernâni Monteiro; I3S, Instituto de Investigação e Inovação em Saúde, University of Porto; Departamento de Biomedicina, Unidade de Bioquímica, Faculty of Medicine, University of Porto; Nobre Laboratory, Faculty of Medicine, University of Porto, Porto, Portugal; Department of Experimental and Clinical Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence; Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, Florence; Department of Translational Medical Sciences, Centre for Basic and Clinical Immunology Research (CISI), University of Naples Federico II, Naples, Italy. IC was supported by a research grant from the Foundation for the Development of Internal Medicine in Europe. The study was partially funded by the Portuguese National Funding Agency (FCT: UID/BIM/04293/2013). I. Chora, MD, Department of Internal Medicine, São João Hospital Center, Al Prof Hernâni Monteiro, and I3S, University of Porto; E. Romano, PhD, Department of Experimental and Clinical Medicine, Division of Rheumatology, AOUC, University of Florence; M. Manetti, PhD, Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence; C. Mazzotta, PhD, Department of Experimental and Clinical Medicine, Division of Rheumatology, AOUC, University of Florence; R. Costa, MSc, I3S, University of Porto, and Departamento de Biomedicina, Unidade de Bioquímica, Faculty of Medicine, University of Porto; V. Machado, PhD, I3S, University of Porto, and Departamento de Biomedicina, Unidade de Bioquímica, Faculty of Medicine, University of Porto; A. Cortez, MSc, Nobre Laboratory, Faculty of Medicine, University of Porto; C. Bruni, MD, Department of Experimental and Clinical Medicine, Division of Rheumatology, AOUC, University of Florence; G. Lepri, MD, Department of Experimental and Clinical Medicine, Division of Rheumatology, AOUC, University of Florence; S. Guiducci, MD, PhD, Department of Experimental and Clinical Medicine, Division of Rheumatology, AOUC, University of Florence; A. De Paulis, MD, Department of Translational Medical Sciences, CISI, University of Naples Federico II; R. Soares, PhD, I3S, University of Porto, and Departamento de Biomedicina, Unidade de Bioquímica, Faculty of Medicine, University of Porto; M. Matucci- Cerinic, MD, PhD, Department of Experimental and Clinical Medicine, Division of Rheumatology, AOUC, University of Florence. Address correspondence to Dr. I. Chora, Rua Faria de Guimarães, 649, 5º andar, 4200-291 Porto, Portugal. E-mail: Accepted for publication April 11, 2017.
    Objective: To investigate whether patients with a very early diagnosis of systemic sclerosis (VEDOSS) may already present circulating markers and in vitro signs of microvascular dysfunction.

    Methods: Serum samples were obtained from 55 patients with systemic sclerosis (SSc), 25 patients with VEDOSS, and 55 matched healthy controls (HC). Serum levels of pan-vascular endothelial growth factor (VEGF) and soluble neuropilin-1 (sNRP-1) were measured by ELISA. Read More

    RXRB is a MHC-encoded susceptibility gene associated with anti-topoisomerase I antibody-positive systemic sclerosis.
    J Invest Dermatol 2017 May 12. Epub 2017 May 12.
    Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.
    Systemic sclerosis (SSc) is a systemic autoimmune and connective tissue disorder associated with the human leukocyte antigen (HLA) locus. However, the functional relationship between HLA gene(s) and disease development remains unknown. To elucidate major histocompatibility complex (MHC)-linked SSc genetics, we performed genotyping of MHC-borne microsatellites and HLA-DPB1 alleles using DNA obtained from 318 anti-topoisomerase I antibody-positive patients and 561 healthy controls, all of Japanese descent. Read More

    Circulating Anti-Nuclear Antibodies in Systemic Sclerosis: Utility in Diagnosis and Disease Subsetting.
    J Nippon Med Sch 2017 ;84(2):56-63
    Department of Allergy and Rheumatology, Nippon Medical School.
    The presence of circulating anti-nuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). Currently, a variety of SSc-specific ANAs, including anticentromere, anti-topoisomerase I, and anti-RNA polymerase III antibodies, have been well characterized, and their commercial kits are available worldwide. Since these autoantibodies are specifically detected in SSc patients and are associated with unique sets of disease manifestations, they are widely used in routine clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvements and prognosis. Read More

    IL-10-producing regulatory B cells (B10 cells), IL-17+ T cells and autoantibodies in systemic sclerosis.
    Clin Immunol 2017 May 11. Epub 2017 May 11.
    Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa 40500, Greece. Electronic address:
    We aimed to analyze IL-10+ Breg (B10) cells, found to be reduced in systemic sclerosis (SSc), in relation to SSc-specific autoAbs and IL-17+ and IFNγ+ T cells in SSc. Peripheral blood B10 cells from 26 patients with SSc positive for anti-Topo I or anti-Cen autoAbs, and 12 healthy controls (HC) were studied by flow cytometry. IL-17+ and IFNγ+ T cells were also studied. Read More

    The fact not to ignore: Mean blood pressure is the main predictor of increased arterial stiffness in patients with systemic rheumatic diseases.
    Adv Med Sci 2017 May 10;62(2):223-229. Epub 2017 May 10.
    Medical Faculty, Vilnius University, Vilnius, Lithuania; State Research Institute for Innovative Medicine, Vilnius, Lithuania.
    Purpose: We aimed to evaluate the association between carotid-radial pulse wave velocity (PWV), augmentation index (AIx), and flow-mediated dilatation (FMD) of the brachial artery and factors potentially influencing them in patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and systemic sclerosis (SSc).

    Material And Methods: 316 patients diagnosed with RA (32%), SLE (20%), SSc (16%) and 156 controls (32%) were included in the study. Parameters of arterial stiffness AIx and PWV were obtained using applanation tonometry. Read More

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