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    Structure, Pharmacology and Roles in Physiology of the P2Y12 Receptor.
    Adv Exp Med Biol 2017 Sep 12. Epub 2017 Sep 12.
    Department of Pharmacology and Toxicology, Pharma Center, University of Bonn, D-53127, Bonn, Germany.
    P2Y receptors are G-protein-coupled receptors (GPCRs) for extracellular nucleotides. The platelet ADP-receptor which has been denominated P2Y12 receptor is an important target in pharmacotherapy. The receptor couples to Gαi2 mediating an inhibition of cyclic AMP accumulation and additional downstream events including the activation of phosphatidylinositol-3-kinase and Rap1b proteins. Read More

    Very Early Systemic Sclerosis and Pre-systemic Sclerosis: Definition, Recognition, Clinical Relevance and Future Directions.
    Curr Rheumatol Rep 2017 Sep 18;19(10):65. Epub 2017 Sep 18.
    Department of Experimental and Clinical Medicine, University of Florence, V. Pieraccini 18, 50139, Florence, Italy.
    Purpose Of Review: The approach to systemic sclerosis (SSc) has changed over the years with an increasing focus on the very early diagnosis of the disease. The terminology identifying patients in the early phase of SSc has been significantly confusing in the last three decades. The purpose of this article is to analyze how the concept of "very early SSc" has evolved over the years, which is the role of an early diagnosis and how early treat patients. Read More

    Serum-Mediated Oxidative Stress from Systemic Sclerosis Patients Affects Mesenchymal Stem Cell Function.
    Front Immunol 2017 1;8:988. Epub 2017 Sep 1.
    IRMB, INSERM, Montpellier University, Montpellier, France.
    Objectives: Properties of mesenchymal stromal/stem cells (MSCs) from systemic sclerosis (SSc) patients have been reported to be altered. MSC-based therapy may therefore rely on the use of allogeneic MSCs from healthy subjects. Here, we investigated whether heterologous MSCs could exhibit altered properties following exposure to oxidative environment of SSc sera. Read More

    Is there a role for IL-17 in the pathogenesis of systemic sclerosis?
    Immunol Lett 2017 Sep 14. Epub 2017 Sep 14.
    Pathology and Immunology, University Hospital and School of Medicine, Geneva, Switzerland; Dermatology, University Hospital and School of Medicine, Geneva, Switzerland. Electronic address:
    In systemic sclerosis (SSc) immuno-inflammatory events are central to disease development. Amongst other mediators of inflammation, interleukin 17 (IL-17) and Th17 cells have been reported to be increased in the peripheral blood and target organs including involved skin in SSc. They participate and amplify inflammatory responses by inducing the production of cytokines such as IL-6, chemokines such as CCL2 and CXCL8 (IL-8), matrix metalloproteinases-1, -2, -9 and the expression of adhesion molecules in stromal cells including fibroblasts and endothelial cells. Read More

    [Cancers associated with systemic sclerosis involving anti-RNA polymerase III antibodies].
    Ann Dermatol Venereol 2017 Sep 13. Epub 2017 Sep 13.
    Service de dermatologie, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France.
    Background: The incidence of cancer is increased in patients with systemic sclerosis (SSc). Further, recent studies have also shown that the presence of anti-RNA polymerase III antibodies is associated with a higher incidence of cancer in this population.

    Patients And Methods: Herein we present the cases of two men aged 56 and 23 years presenting SSc without anti-Scl70 or anti-centromere antibodies but with anti-RNA polymerase III antibodies. Read More

    Reuma.pt contribution to the knowledge of immune-mediated systemic rheumatic diseases.
    Acta Reumatol Port 2017 Sep 14. Epub 2017 Sep 14.
    Centro Hospitalar Baixo Vouga.
    Patient registries are key instruments aimed at a better understanding of the natural history of diseases, at assessing the effectiveness of therapeutic interventions, as well as identifying rare events or outcomes that are not captured in clinical trials. However, the potential of registries goes far beyond these aspects. For example, registries promote the standardization of clinical practice, can also provide information on domains that are not routinely collected in clinical practice and can support decision-making. Read More

    Periostin in the pathogenesis of skin diseases.
    Cell Mol Life Sci 2017 Sep 15. Epub 2017 Sep 15.
    Department of Dermatology, Course of Integrated Medicine, Graduate School of Medicine, Osaka University, 2-2 Yamadaoka, Suita, Osaka, 5650871, Japan.
    Skin is an organ that is susceptible to damage by external injury, chronic inflammation, and autoimmunity. Tissue damage causes alterations in both the configuration and type of cells in lesional skin. This phenomenon, called tissue remodeling, is a universal biological response elicited by programmed cell death, inflammation, immune disorders, and tumorigenic, tumor proliferative, and cytoreductive activity. Read More

    Automated Measurement of Microvascular Function Reveals Dysfunction in Systemic Sclerosis: A Cross-sectional Study.
    J Rheumatol 2017 Sep 15. Epub 2017 Sep 15.
    From the University of Utah, Department of Internal Medicine, and the Department of Exercise and Sport Science, and the Department of Biochemistry; VA Salt Lake City, Geriatric Research, Education, and Clinical Center (GRECC), Salt Lake City, Utah, USA; Maastricht University, Department of Physiology, Maastricht, the Netherlands; MicroVascular Health Solutions LLC, Alpine, Utah, USA. Supported by the US National Institutes of Health (R01 AG040297 to AJD, K23 AR067889 to TMF, and K02 AG045339 to AJD); and the US Department of Veterans Affairs (I01 CX001183 to TMF). The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. HV is chief scientific officer at MicroVascular Health Solutions LLC and reports personal fees from GlycoCheck BV and MicroVascular Health Solutions LLC outside the submitted work. In addition, HV has a patent (WO2009068685): Diagnostic and Therapeutic Tools for Diseases Altering Vascular Function. D.R. Machin, PhD, University of Utah, Department of Internal Medicine, and VA Salt Lake City, GRECC; P.E. Gates, PhD, University of Utah, Department of Internal Medicine; H. Vink, PhD, Maastricht University, Department of Physiology, and MicroVascular Health Solutions LLC; T.M. Frech, MD, University of Utah, Department of Internal Medicine; A.J. Donato, PhD, University of Utah, Department of Internal Medicine, Department of Exercise and Sport Science, Department of Biochemistry, and VA Salt Lake City, GRECC. Address correspondence to A.J. Donato, VA Medical Center, SLC, 500 Foothill Drive, GRECC, Bldg 2, Rm 2D15A, Salt Lake City, Utah 84148, USA. E-mail: Accepted for publication June 21, 2017.
    Objective: The aim of our study was to determine whether an automated capture and analysis system could detect differences in structure and function of sublingual microvessels in patients with systemic sclerosis (SSc) compared to healthy controls.

    Methods: Intravital microscopy of the sublingual microcirculation was automatically captured and analyzed in 40 patients with SSc and 10 age-matched healthy controls.

    Results: Total and perfused microvascular density were lower in patients with SSc compared with controls (total microvascular density: 2471 ± 134 μm/mm(2) vs 3067 ± 197 μm/mm(2), p = 0. Read More

    Hepcidin and metallothioneins as molecular base for sex-dependent differences in clinical course of experimental autoimmune encephalomyelitis in chronic iron overload.
    Med Hypotheses 2017 Sep 20;107:51-54. Epub 2017 Jul 20.
    Department of Physiology and Immunology, Medical Faculty, University of Rijeka, B. Branchetta 20, 51 000 Rijeka, Croatia.
    Multiple sclerosis is a chronic demyelinating disease of the central nervous system characterised by inflammatory and degenerative changes. It is considered that disease arises from the influence of environmental factors on genetically susceptible individuals. Recent researches, using magnetic resonance imaging, connected iron deposits in different brain regions with demyelinating process in multiple sclerosis patients. Read More

    The role of gut microbiota in the pathogenesis of rheumatic diseases.
    Clin Rheumatol 2017 Sep 15. Epub 2017 Sep 15.
    Department of Rheumatology, Peking Union Medical College, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Beijing, 100730, China.
    Rheumatic diseases refer to many diseases with a loss of immune self-tolerance, leading to a chronic inflammation, degeneration, or metabolic derangement in multiple organs or tissues. The cause of rheumatic diseases remains to be elucidated, though both environmental and genetic factors are required for the development of rheumatic diseases. Over the past decades, emerging studies suggested that alteration of intestinal microbiota, known as gut dysbiosis, contributed to the occurrence or development of a range of rheumatic diseases, including rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis, systemic sclerosis, and Sjogren's syndrome, through profoundly affecting the balance between pro- and anti-inflammatory immune responses. Read More

    Emergency Neurologic Life Support: Spinal Cord Compression.
    Neurocrit Care 2017 Sep 14. Epub 2017 Sep 14.
    Department of Neurology, University of Miami, Miami, FL, USA.
    There are many causes of acute myelopathy including multiple sclerosis, systemic disease, and acute spinal cord compression (SCC). SCC should be among the first potential causes considered given the significant permanent loss of neurologic function commonly associated with SCC. This impairment can occur over a short period of time, and may be avoided through rapid and acute surgical intervention. Read More

    New Insights into Cardiac Involvement in Juvenile Scleroderma: A Three-Dimensional Echocardiographic Assessment Unveils Subclinical Ventricle Dysfunction.
    Pediatr Cardiol 2017 Sep 14. Epub 2017 Sep 14.
    Department of Pediatric Rheumatology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
    Cardiac manifestations in juvenile scleroderma or systemic sclerosis (JSSc) have poor prognosis, begin in early stages of the disease, and remain clinically asymptomatic. New echocardiography modalities, such as 2D/3D speckle tracking (STE, strain analysis for regional and global ventricular functions), can detect cardiac involvement in early stages. We assessed 21 JSSc patients and 19 controls using 2D/3D STE. Read More

    Measurement of Serum Klotho in Systemic Sclerosis.
    Dis Markers 2017 22;2017:9545930. Epub 2017 Aug 22.
    Department of Rheumatology, University Hospital "Luigi Sacco", Milan, Italy.
    Background: The aim of our study was to evaluate the serum concentration of klotho in a cohort of systemic sclerosis (SSc) patients compared to that of healthy controls and to correlate its levels with the degree and the kind of organ involvement.

    Methods: Blood samples obtained from both patients and controls were collected and analysed by an ELISA test for the determination of human soluble klotho. Scleroderma patients were evaluated for disease activity through clinical, laboratory, and instrumental assessment. Read More

    Hormonal contraception and the development of autoimmunity: A review of the literature.
    Linacre Q 2017 Aug 18;84(3):275-295. Epub 2017 Aug 18.
    BriaCell Therapeutics Corporation, Havertown, PA, USA.
    Estrogens and progestins are known to have profound effects on the immune system and may modulate the susceptibility to autoimmune diseases. A comprehensive literature search was carried out using PubMed for any of 153 autoimmune disease terms and the terms contraception, contraceptive, or their chemical components with limits of Humans + Title or Abstract. Over 1,800 titles were returned and scanned, 352 papers retrieved and reviewed in depth and an additional 70 papers retrieved from the bibliographies. Read More

    Efficacy and safety of autologous haematopoietic stem cell transplantation in systemic sclerosis: A systematic review of literature.
    Br J Dermatol 2017 Sep 14. Epub 2017 Sep 14.
    Department of Rheumatology, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France.
    We aimed at assessing the efficacy of autologous haematopoietic stem cell transplantation (HSCT) for skin sclerosis and lung function in systemic sclerosis (SSc). We performed a systematic literature review in the Pubmed and Scopus databases from the earliest records to March 2016. We assessed quality using the Cochrane tool for randomized studies, Newcastle-Ottawa scale for controlled-cohort studies and an 18-item quality appraisal checklist for case series. Read More

    Effects of immunomodulators on the response induced by vaccines against autoimmune diseases.
    Autoimmunity 2017 Sep 14:1-10. Epub 2017 Sep 14.
    a Qantu Therapeutics, Inc , Lewisville , TX , USA.
    A promising treatment for T-cell-mediated autoimmune diseases is the induction of immune tolerance by modulating the immune response against self-antigens, an objective that may be achieved by vaccination. There are two main types of vaccines currently under development. The tolerogenic vaccines, composed of proteins formed by a cytokine fused to a self-antigen, which usually induce tolerance by eliminating the T-cells that are immune reactive against the self-antigen. Read More

    Position paper: Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis.
    Nat Rev Rheumatol 2017 Sep 14. Epub 2017 Sep 14.
    Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Centre Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, Eugenstrasse 3, 73230 Kirchheim unter Teck, Germany.
    Anti-neutrophil cytoplasmic antibodies (ANCAs) are valuable laboratory markers used for the diagnosis of well-defined types of small-vessel vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). According to the 1999 international consensus on ANCA testing, indirect immunofluorescence (IIF) should be used to screen for ANCAs, and samples containing ANCAs should then be tested by immunoassays for proteinase 3 (PR3)-ANCAs and myeloperoxidase (MPO)-ANCAs. The distinction between PR3-ANCAs and MPO-ANCAs has important clinical and pathogenic implications. Read More

    Expressions of p53 and PUMA in fibroblasts of systemic sclerosis patients are normal at transcription level.
    J Cosmet Dermatol 2017 Sep 14. Epub 2017 Sep 14.
    Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran.
    Background: Systemic sclerosis (SSc) fibroblasts show resistance apoptosis mechanisms, which enhances the fibrosis stage of the disease. Impaired function of p53 upregulated modulator of apoptosis (PUMA) has been related to deficits in p53-dependant apoptosis pathway. This study aimed to evaluate the transcriptional levels of p53 and PUMA mRNAs in fibroblasts from SSc patients and compare it with healthy individuals. Read More

    A modified graft-versus-host-induced model for systemic sclerosis, with pulmonary fibrosis in Rag2-deficient mice.
    FEBS Open Bio 2017 Sep 16;7(9):1316-1327. Epub 2017 Aug 16.
    Division of RheumatologyHuashan HospitalFudan UniversityShanghaiChina.
    Systemic sclerosis (SSc) is a connective tissue disease that results in fibrosis in multiple organs. Various animal models for this disease have been developed, both genetic and induced. One of the induced models, sclerodermatous graft-versus-host disease (scl-GvHD), exhibits the main characteristics of SSc, but involves lethal γ-irradiation of recipients. Read More

    Humanistic and cost burden of systemic sclerosis: A review of the literature.
    Autoimmun Rev 2017 Sep 9. Epub 2017 Sep 9.
    Boehringer Ingelheim GmbH, Ingelheim, Germany. Electronic address:
    Background: Systemic sclerosis (SSc), or systemic scleroderma, is a chronic multisystem autoimmune disease characterised by widespread vascular injury and progressive fibrosis of the skin and internal organs. Patients with SSc have decreased survival, with pulmonary involvement as the main cause of death. Current treatments for SSc manage a range of symptoms but not the cause of the disease. Read More

    Impact of combined sodium chloride and saturated long-chain fatty acid challenge on the differentiation of T helper cells in neuroinflammation.
    J Neuroinflammation 2017 Sep 12;14(1):184. Epub 2017 Sep 12.
    Department of Neurology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg, Schwabachanlage 6, 91054, Erlangen, Germany.
    Background: There has been a marked increase in the incidence of autoimmune diseases like multiple sclerosis (MS) in the last decades which is most likely driven by a change in environmental factors. Here, growing evidence suggests that ingredients of a Western diet like high intake of sodium chloride (NaCl) or saturated fatty acids may impact systemic immune responses, thus increasing disease susceptibility. Recently, we have shown that high dietary salt or long-chain fatty acid (LCFA) intake indeed aggravates T helper (Th) cell responses and neuroinflammation. Read More

    Combination of capillaroscopic and ultrasonographic evaluations in systemic sclerosis: Results of a cross-sectional study.
    Arthritis Care Res (Hoboken) 2017 Sep 12. Epub 2017 Sep 12.
    Department of Internal Medicine, CHU Rennes, University of Rennes 1 all in Rennes, France.
    Objectives: To compare microvascular damages on nailfold capillaroscopy (NC) with macrovascular manifestations evaluated by hand power doppler ultrasonography (PDUS) in Systemic Sclerosis (SSc) patients, and to assess the associations of these damages with the main digital manifestations of the disease: digital ulcers (DU), acro-osteolysis and Calcinosis.

    Methods: NC, hand X-Rays and PDUS were systematically performed in 64 unselected SSc patients. PDUS evaluation with assessment of ulnar artery occlusion (UAO) and finger pulp blood flow (FPBF) was performed blinded for the results of X-Rays and NC. Read More

    ESVM guidelines - the diagnosis and management of Raynaud's phenomenon.
    Vasa 2017 Sep 12:1-11. Epub 2017 Sep 12.
    25 Department of Clinical Cardiology and Angiology, Hospital Bulovka, Prague, Czech Republic.
    Regarding the clinical diagnosis of Raynaud's phenomenon and its associated conditions, investigations and treatment are substantial, and yet no international consensus has been published regarding the medical management of patients presenting with this condition. Most knowledge on this topic derives from epidemiological surveys and observational studies; few randomized studies are available, almost all relating to drug treatment, and thus these guidelines were developed as an expert consensus document to aid in the diagnosis and management of Raynaud's phenomenon. This consensus document starts with a clarification about the definition and terminology of Raynaud's phenomenon and covers the differential and aetiological diagnoses as well as the symptomatic treatment. Read More

    An international SUrvey on non-iNvaSive tecHniques to assess the mIcrocirculation in patients with RayNaud's phEnomenon (SUNSHINE survey).
    Rheumatol Int 2017 Sep 11. Epub 2017 Sep 11.
    Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
    To canvas opinion concerning the role of non-invasive techniques in the assessment of patients with Raynaud's phenomenon (Rp) in clinical and research settings: four nailfold capillaroscopy methods [videocapillaroscopy (NVC), dermoscopy, stereomicroscopy, digital USB microscopy], four laser Doppler methods (laser Doppler flowmetry, imaging, anemometry/velocimetry, laser speckle contrast analysis), thermographic imaging, and upper limb arterial Doppler ultrasound. Emails with a link to the survey were sent to physicians from the European Scleroderma Trials and Research group (EUSTAR), the EULAR Study Group on Microcirculation in Rheumatic Diseases (SG_MC/RD) and members of the pediatric rheumatology Email board. The main descriptive analysis related to physicians looking after adult patients, with some analysis also of opinions from paediatric rheumatologists. Read More

    Two years follow-up of an open-label pilot study of treatment with rituximab in patients with early diffuse cutaneous systemic sclerosis.
    Acta Clin Belg 2017 Sep 11:1-7. Epub 2017 Sep 11.
    a Department of Rheumatology , Universitair Ziekenhuis Gent , Ghent , Belgium.
    Objectives: Following results in open-label studies of rituximab in patients with systemic sclerosis, a Belgian three-centre initiative was launched to explore safety and efficacy of rituximab in early, diffuse cutaneous systemic sclerosis (dcSSc).

    Methods: Open-label study of 17 patients with early dcSSc, treated with two courses of rituximab, at month 0 and 6. Clinical examination, lung function testing, echocardiography, disease activity score (DAS) and functional status were performed at baseline and over 24 months of follow-up. Read More

    Treg/Th17 cell balance and phytohaemagglutinin activation of T lymphocytes in peripheral blood of systemic sclerosis patients.
    World J Exp Med 2017 Aug 20;7(3):84-96. Epub 2017 Aug 20.
    Ekaterina Krasimirova, Tsvetelina Velikova, Ekaterina Ivanova-Todorova, Kalina Tumangelova-Yuzeir, Dobroslav Kyurkchiev, Laboratory of Clinical Immunology, University Hospital "St. Ivan Rilski", Department of Clinical Laboratory and Clinical Immunology, Medical University of Sofia, 1431 Sofia, Bulgaria.
    Aim: To investigate T-cell activation, the percentage of peripheral T regulatory cells (Tregs), Th17 cells and the circulating cytokine profile in systemic sclerosis (SSc).

    Methods: We enrolled a total of 24 SSc patients and 16 healthy controls in the study and divided the patients as having diffuse cutaneous SSc (dcSSc, n = 13) or limited cutaneous SSc (lcSSc, n = 11). We performed a further subdivision of the patients regarding the stage of the disease - early, intermediate or late. Read More

    Toll Like Receptors in systemic sclerosis: An emerging target.
    Immunol Lett 2017 Sep 6. Epub 2017 Sep 6.
    Faculty of Health and Life Sciences, Northumbria University, Ellison Building, Newcastle Upon Tyne, United Kingdom. Electronic address:
    Pattern Recognition Receptors are critical receptors that elicit an immune response upon their activation that culminates in activation of NF-KB and cytokine secretion. Key among these receptors are the Toll-Like Receptors (TLRs). These evolutionary conserved receptors form a key part in the defence against various pathogens and comprise a key part of the innate immune system. Read More

    Association between objective signs and subjective symptoms of dry eye disease in patients with systemic sclerosis.
    Rheumatol Int 2017 Sep 7. Epub 2017 Sep 7.
    Department of Ophthalmology, Faculty of Medicine, University of Debrecen, Nagyerdei krt. 98, 4032, Debrecen, Hungary.
    The aim of this study was to evaluate the association between clinical signs and symptoms of dry eye disease (DED) in patients with systemic sclerosis (SSc). This cross-sectional observational study included 19 SSc patients and 19 normal subjects with no ocular symptoms or ocular surface disorders. Clinical parameters included tear film break-up time (tBUT), Schirmer I, lissamine green (LG) dye, and tear film osmolarity tests, tear production, and tear secretion flow. Read More

    Spectrum of esophageal dysmotility in systemic sclerosis on high-resolution esophageal manometry as defined by Chicago classification.
    Dis Esophagus 2017 Dec;30(12):1-6
    Departments of Gastroenterology and Hepatology.
    The classic manometric findings in systemic sclerosis are aperistalsis of the esophageal body with hypotensive lower esophageal sphincter. These changes contribute to gastroesophageal reflux disease in these patients. With widespread use of high-resolution esophageal manometry, diverse abnormalities are seen. Read More

    Pedal Cutaneous Manifestations of Tuberous Sclerosis.
    J Am Podiatr Med Assoc 2017 Jul;107(4):324-328
    This case report concerns a patient with a painful soft-tissue mass on his fourth toe. He was evaluated for this soft-tissue mass and was diagnosed as having tuberous sclerosis. The podiatric physician should be able to evaluate a patient with a lower-extremity complaint and relate whether this complaint may correlate with a systemic disorder. Read More

    Rituximab in early systemic sclerosis.
    RMD Open 2017 28;3(2):e000384. Epub 2017 Jul 28.
    Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands.
    Objectives: (1) Hypothesis testing of the potency of rituximab (RTX) in preventing fibrotic complications and (2) assessing acceptability and feasibility of RTX in early systemic sclerosis (SSc).

    Methods: A small, 24-month, randomised, double-blind, placebo-controlled, single-centre trial in patients with SSc diagnosed <2 years was conducted. Patients received RTX or placebo infusions at t=0, t=15 days and t=6 months. Read More

    Agranulocytosis Induced by Sinomenine Hydrochloride.
    Am J Case Rep 2017 Sep 6;18:959-962. Epub 2017 Sep 6.
    Department of Rheumatology, Southwest Hospital, Third Military Medical University, Chongqing, China (mainland).
    BACKGROUND Sinomenine hydrochloride is an alkaloid that is extracted from the Chinese herbal plant Sinomenium acutum, and is used as a herbal medicine in the treatment or rheumatic disease. This report is the first to describe a case of sinomenine hydrochloride-induced agranulocytosis. CASE REPORT A 44-year-old woman with systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) was treated with sinomenine hydrochloride and developed agranulocytosis with a neutrophil count of 0. Read More

    Growth Differentiation Factor-15 (GDF-15) Level and Relation to Clinical Manifestations in Egyptian Systemic Sclerosis patients: Preliminary Data.
    Immunol Invest 2017 Oct;46(7):703-713
    a Rheumatology and Rehabilitation Department , Cairo University Hospital , Cairo , Egypt.
    Aim Of The Work: This study aims to assess Growth differentiation factor-15 (GDF-15) level in Scleroderma patients and its relation to disease manifestations.

    Patients And Methods: This study included 55 scleroderma patients and 40 age and sex matched healthy volunteers. All patients were subjected to full history taking, thorough clinical examination, and laboratory investigations. Read More

    [Manifestations of the connective tissue associated interstitial lung disease under high resolution computed tomography].
    Zhong Nan Da Xue Xue Bao Yi Xue Ban 2017 Aug;42(8):934-939
    Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, China.
    Objective: To analyze the features of the connective tissue associated interstitial lung disease (CTD-ILD) by high resolution computed tomography (HRCT).
 Methods: A total of 127 patients with CTD-ILD, who were diagnosed by clinic laboratory examination and pathology in Xiangya Hospital of Central South University form September 2013 to September 2015, were enrolled for this study. Their lung features of HRCT imaging were retrospectively analyzed. Read More

    Mortality trend of inpatients with connective tissue diseases: 2005-2014.
    Zhong Nan Da Xue Xue Bao Yi Xue Ban 2017 Aug;42(8):927-933
    Department of Rheumatology and Immunology, Xiangya Hospital, Central South University; Institute of Rheumatology and Immunology, Central South University, Changsha 410008, China.
    Objective: To analyze the trend relevant factors leading to death and their patterns over a 10-year period in inpatients with connective tissue diseases (CTDs).
 Methods: All clinical data about death in inpatients with CTDs were retrospectively reviewed between 2005 and 2014 at the Department of Rheumatology and Immunology in Xiangya Hospital of Central South University.
 Results: In the 10-year time period, the overall hospital mortality was 15. Read More

    Therapeutic targeting of TAZ and YAP by dimethyl fumarate in systemic sclerosis fibrosis.
    J Invest Dermatol 2017 Sep 1. Epub 2017 Sep 1.
    Arthritis Center. Electronic address:
    Systemic sclerosis (scleroderma, SSc) is a devastating fibrotic disease with few treatment options. Fumaric acid esters, including dimethyl fumarate (DMF, Tecfidera®) have shown therapeutic effects in several disease models, prompting us to determine whether DMF is effective as a treatment for SSc dermal fibrosis. We found that DMF blocks the pro-fibrotic effects of TGFβ in SSc skin fibroblasts. Read More

    Esophageal baseline impedance levels allow the identification of esophageal involvement in patients with systemic sclerosis.
    Semin Arthritis Rheum 2017 Aug 9. Epub 2017 Aug 9.
    Division of Gastroenterology, Departement of Surgery, Oncology and Gastroenterology, Gastroenterology Unit, University of Padua, Via Giustiniani 2, 35128 Padova, Italy. Electronic address:
    Introduction: Distal esophageal baseline impedance (BI) levels reflect the esophageal mucosal integrity in reflux disease. Systemic sclerosis (SSc) could potentially affect the integrity of esophageal mucosa and consequently impair distal and proximal BI levels, but data in this regard are lacking.

    Aim And Methods: We aimed to prospectively investigate and compare BI levels among non-erosive reflux disease (NERD), SSc patients, and healthy controls (HCs). Read More

    Acute and fatal cardiotoxicity following high-dose cyclophosphamide in a patient undergoing autologous stem cell transplantation for systemic sclerosis despite satisfactory cardiopulmonary screening.
    Bone Marrow Transplant 2017 Sep 4. Epub 2017 Sep 4.
    Department of Clinical Immunology, National Referral Center for Autoimmune Diseases, Strasbourg University Hospital, Strasbourg, France.

    Efficacy of intensive hand physical therapy in patients with systemic sclerosis.
    Clin Exp Rheumatol 2017 Aug 30. Epub 2017 Aug 30.
    Department of Rheumatology and Immunology of Medical Centre, University of Pécs, Hungary.
    Objectives: To evaluate the efficacy of a three-week period of complex and intensive hand physical therapy on hand function in patients with systemic sclerosis (SSc).

    Methods: Thirty-one patients with SSc were treated. Hand stretching exercises, ergotherapy supplemented with thermal and mud baths, whirlpool therapy and soft tissue massage were daily used during a three-week period. Read More

    Autologous stem cell transplantation in systemic sclerosis: a systematic review.
    Clin Exp Rheumatol 2017 Aug 30. Epub 2017 Aug 30.
    The Department of Rheumatology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia.
    Objectives: Haematopoetic autologous stem cell transplantation (ASCT) has emerged as a treatment option for patients with refractory, severe autoimmune disease. This is a systematic review of the current literature on ASCT in adult patients with systemic sclerosis (SSc).

    Methods: Original articles published between 2005 and 2016 that evaluated the use of ASCT in patients with SSc were reviewed with respect to the primary outcomes of overall and transplant related mortality (TRM) rates, and secondary outcomes of changes in modified Rodnan Skin Score (mRSS), forced vital capacity (FVC), progression/event free survival (P/EFS) and quality of life measures. Read More

    A novel iloprost administration method with portable syringe pump for the treatment of acral ulcers and Raynaud's phenomenon in systemic sclerosis patients. A pilot study (ILOPORTA).
    Clin Exp Rheumatol 2017 Aug 31. Epub 2017 Aug 31.
    Clinica Medica, Department of Internal Medicine, Ospedali Riuniti University Hospital, Ancona; and Department of Clinical and Molecular Sciences, Section of Clinical Medicine, Università Politecnica delle Marche, School of Medicine, Ancona, Italy.
    Objectives: The objective of the study is to evaluate the feasibility, safety and efficacy of home infusion of iloprost with the new portable syringe pump Infonde®, for the treatment of scleroderma-related Raynaud's phenomenon and digital ulcers.

    Methods: 12 scleroderma patients were treated with iloprost at home, using the pump, with infusion cycles of 2 days per month (24 hours a day), for 6 months.

    Results: The home treatment proved feasible since ten patients (83%) completed the entire infusion cycle, thus satisfying the feasibility target imposed by the protocol (75%). Read More

    Severe oesophageal disease and its associations with systemic sclerosis.
    Clin Exp Rheumatol 2017 Aug 30. Epub 2017 Aug 30.
    Joint Academic Rheumatology Program, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
    Objectives: Severe oesophageal disease in patients with systemic sclerosis (SSc), referred as scleroderma oesophagus, is characterised by ineffective or absent peristalsis along with hypotensive oesophagogastric junction (hEGJ). The associations between scleroderma oesophagus and different clinical and laboratory manifestations of SSc is still controversial. In this study we aimed to assess associations between scleroderma oesophagus, diagnosed by high resolution manometry (HRM), and other manifestations of disease. Read More

    The presence of small joint contractures is a risk factor for survival in 439 patients with systemic sclerosis.
    Clin Exp Rheumatol 2017 Aug 31. Epub 2017 Aug 31.
    Department of Rheumatology and Immunology, University of Pécs, Hungary.
    Objectives: Analysis of risk factors and mortality of 439 patients with systemic sclerosis (SSc) in a tertiary care centre.

    Methods: The mean follow up time was 8.4±5. Read More

    Pathophysiological Mechanisms in Sclerosing Skin Diseases.
    Front Med (Lausanne) 2017 18;4:120. Epub 2017 Aug 18.
    Department of Dermatology, University of Cologne, Cologne, Germany.
    Sclerosing skin diseases represent a large number of distinct disease entities, which include systemic sclerosis, localized scleroderma, and scleredema adultorum. These pathologies have a common clinical appearance and share histological features. However, the specific interplay between cytokines and growth factors, which activate different mesenchymal cell populations and production of different extracellular matrix components, determines the biomechanical properties of the skin and the clinical features of each disease. Read More

    Unmet Needs in Systemic Sclerosis Understanding and Treatment: the Knowledge Gaps from a Scientist's, Clinician's, and Patient's Perspective.
    Clin Rev Allergy Immunol 2017 Sep 2. Epub 2017 Sep 2.
    Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
    Systemic sclerosis (SSc) is a highly heterogeneous disease caused by a complex molecular circuitry. For decades, clinical and molecular research focused on understanding the primary process of fibrosis. More recently, the inflammatory, immunological and vascular components that precede the actual onset of fibrosis, have become a matter of increasing scientific scrutiny. Read More

    Corticosteroid inhibits chemokines production in systemic sclerosis patients.
    Steroids 2017 Sep 1;127:24-30. Epub 2017 Sep 1.
    Laboratório de Imunomodulação e Novas Abordagens Terapêuticas, Núcleo de Pesquisa em Inovação Terapêutica (NUPIT), Universidade Federal de Pernambuco, Recife, PE, Brazil.
    In this study, we evaluated glucocorticoids (GC) effects on cytokine/chemokine levels in serum samples and peripheral blood mononuclear cell (PBMC) production from systemic sclerosis (SSc) patients. We evaluated cytokine and chemokine levels in serum samples from SSc patients taking or not taking systemic glucocorticoids. PBMCs response to methylprednisolone (MP) was examined from 15 SSc patients and 8 healthy control subjects following PBMC stimulation with anti-CD3/CD28. Read More

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