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    Assessment of treatment effects on digital ulcer and blood perfusion by laser speckle contrast analysis in a patient affected by systemic sclerosis.
    Reumatismo 2017 Sep 21;69(3):134-136. Epub 2017 Sep 21.
    Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa.
    Laser speckle contrast analysis (LASCA) is a good tool to evaluate the variation in peripheral blood perfusion during long-term follow-up and is able to safely monitor digital ulcer evolution in scleroderma patients. It evaluates blood perfusion in different areas within the skin lesions and surrounding them during standard treatment. Read More

    Systematic review and meta-analysis of the epidemiology of polyautoimmunity in Sjögren's syndrome (secondary Sjögren's syndrome) focusing on autoimmune rheumatic diseases.
    Scand J Rheumatol 2017 Sep 20:1-14. Epub 2017 Sep 20.
    c Department of Rheumatology , University College London , London , UK.
    Objective: The epidemiology of polyautoimmunity in Sjögren's syndrome (secondary Sjögren's syndrome - sSS) is not well defined and has not been investigated before using a systematic approach. We conducted a systematic review of the epidemiology of sSS associated with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), scleroderma, and myositis, assessing the prevalence rates (PRs) and clinical and serological features of sSS.

    Method: A systematic literature search of PubMed and Embase databases (updated to March 2016) was performed to identify all published data on PR, demographic profile, clinical manifestations, laboratory features, and causes of death associated with sSS. Read More

    Interstitial lung disease in the connective tissue diseases; a paradigm shift in diagnosis and treatment.
    Clin Immunol 2017 Sep 15. Epub 2017 Sep 15.
    Division of Rheumatology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, United States. Electronic address:
    Interstitial lung disease (ILD) in the connective tissue diseases (CTD) is amongst the most challenging aspect of care of patients with rheumatic diseases and is the source of significant morbidity and mortality. While there has been progress in our understanding of the natural history of these complications, we still suffer from a limited reservoir of data to confidently determine which patients are at highest risk for disease and those who are at highest risk for disease progression. Treatment options until recently have been limited to anti-inflammatory therapies but with the emerging availability of anti-fibrotic therapies, a shift in strategy is emerging to target therapies based on the specific radiographic, histopathologic features and biomarker profiles that are unique to patients with rheumatic diseases and ILD. 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

    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

    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

    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

    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

    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

    Use of silver diamine fluoride for the maintenance of dental prostheses in a high caries-risk patient: A medical management approach.
    J Prosthet Dent 2017 Sep 6. Epub 2017 Sep 6.
    Graduate Research Assistant, University of San Francisco, San Francisco, Calif.
    A technique for using silver diamine fluoride (SDF) as part of a regimen to help maintain dental prostheses in a patient with scleroderma and at high risk of caries is presented. Medically compromised, xerostomic, or elderly patients generally face greater risk of caries and specifically with prosthetic retainer teeth. SDF is a minimally invasive solution to this problem. Read More

    Periostin in inflammation and allergy.
    Cell Mol Life Sci 2017 Sep 8. Epub 2017 Sep 8.
    Division of Medical Biochemistry, Department of Biomolecular Sciences, Saga Medical School, 5-1-1, Nabeshima, Saga, 849-8501, Japan.
    We found for the first time that IL-4 and IL-13, signature type 2 cytokines, are able to induce periostin expression. We and others have subsequently shown that periostin is highly expressed in chronic inflammatory diseases-asthma, atopic dermatitis, eosinophilc chronic sinusitis/chronic rhinosinusitis with nasal polyp, and allergic conjunctivitis-and that periostin plays important roles in the pathogenesis of these diseases. The epithelial/mesenchymal interaction via periostin is important for the onset of allergic inflammation, in which periostin derived from fibroblasts acts on epithelial cells or fibroblasts, activating their NF-κB. Read More

    Hyperspectral imaging as a novel diagnostic tool in microcirculation of wounds.
    Clin Hemorheol Microcirc 2017 Sep 4. Epub 2017 Sep 4.
    Department of Dermatology of the University Medicine Greifswald, Sauerbruchstrasse, Greifswald, Germany.
    Monitoring of perfusion is a cornerstone in surgery, phlebology and basic science to proof wound healing by interventions. In chronic wound management it is of utmost importance to realize and parametrize wound bed perfusion to verify actual, and plan further treatment by noninvasive diagnostics. Up to now monitoring is based on visual inspection of wounds as conventionally practiced over more than decades. Read More

    Chronic exposure of Interleukin-13 Suppress the Induction of Matrix Metalloproteinase-1 by Tumor Necrosis Factor α in Normal and Scleroderma Dermal Fibroblasts through Protein Kinase B/Akt.
    Clin Exp Immunol 2017 Sep 7. Epub 2017 Sep 7.
    Division of Connective Tissue Diseases, University of Tennessee Health Science Center, Memphis, Tennessee.
    Objective: Peripheral blood mononuclear cells taken from patients with scleroderma express increased levels of IL-13. Moreover, the expression of MMP-1 from involved scleroderma skin fibroblasts is refractory to stimulation by tumor necrosis factor alpha. To elucidate the mechanism(s) involved, we examined the effect of IL-13 on TNF-α-induced matrix metalloproteinase-1 expression in normal and scleroderma human dermal fibroblast lines and studied the involvement of serine/threonine kinase B/Akt in this response. 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

    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

    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

    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

    Basidiospores attach to the seed of Shorea leprosula in lowland tropical dipterocarp forest and form functional ectomycorrhiza on seed germination.
    Mycorrhiza 2017 Sep 2. Epub 2017 Sep 2.
    Department of Biology, Faculty of Mathematics and Natural Sciences, Bogor Agricultural University, Darmaga Campus, Bogor, West Java, 16880, Indonesia.
    This research aimed to study the ectomycorrhiza formed by basidiospores attached to the outer surface of Shorea leprosula (Dipterocarpaceae) seed collected from a lowland tropical dipterocarp forest. Two groups of seeds were collected: control seeds collected from plastic net hanging 2 m above the ground and forest floor seeds collected on the forest floor. Before planting, 15 seeds from each group were observed for basidiospores attached to the seed. Read More

    Disintegration of collagen fibrils by Glucono-δ-lactone: An implied lead for disintegration of fibrosis.
    Int J Biol Macromol 2017 Sep 1. Epub 2017 Sep 1.
    Bioorganic Chemistry Laboratory, Council of Scientific and Industrial Research (CSIR) - Central Leather Research Institute (CLRI), Adyar, Chennai 600020, India; Academy of Scientific and Innovative Research (AcSIR), CSIR-CLRI Campus, Chennai 600020, India. Electronic address:
    Excess accumulation of collagen (fibrosis) undergoes self-aggregation, which leads to fibrillar collagen, on the extracellular matrix is the hallmark of a number of diseases such as keloids, hypertrophic scars, and systemic scleroderma. Direct inhibition or disintegration of collagen fibrils by small molecules offer a therapeutic approach to prevent or treat the diseases related to fibrosis. Herein, the anti-fibrotic property of Glucono-δ-lactone (GdL), known as acidifier, on the fibrillation and its disintegration of collagen was investigated. Read More

    Genome-wide DNA methylation analysis in blood cells from patients with Werner syndrome.
    Clin Epigenetics 2017 30;9:92. Epub 2017 Aug 30.
    CRIIS (Interdisciplinary, Interdepartmental and Specialistic Reference Center for Early Diagnosis of Scleroderma, Treatment of Sclerodermic Ulcers and Videocapillaroscopy), Sandro Pertini Hospital, Rome, Italy.
    Background: Werner syndrome is a progeroid disorder characterized by premature age-related phenotypes. Although it is well established that autosomal recessive mutations in the WRN gene is responsible for Werner syndrome, the molecular alterations that lead to disease phenotype remain still unidentified.

    Results: To address whether epigenetic changes can be associated with Werner syndrome phenotype, we analysed genome-wide DNA methylation profile using the Infinium MethylationEPIC BeadChip in the whole blood from three patients affected by Werner syndrome compared with three age- and sex-matched healthy controls. Read More

    Clinical Trials in Raynaud Phenomenon: A spoonful of sugar (pill) makes the medicine go down (in flames).
    Arthritis Rheumatol 2017 Aug 31. Epub 2017 Aug 31.
    Johns Hopkins University School of Medicine, Baltimore, Maryland, 21224.
    The peripheral vascular disease in systemic sclerosis (scleroderma) is a challenging problem because it presents with both vasospasm and an underlying structural vascular disease that leads not only to severe Raynaud phenomenon but often critical digital ischemia with progressive tissue damage or acute ulceration. While current therapy with a calcium channel blocker alone or combined with another vasodilating agent provides some modest benefit, it does not provide adequate control of Raynaud's events in many patients with scleroderma. Vasodilating therapy alone also does not address the underlying biological process causing progressive vascular disease. Read More

    Diagnosing and managing scleroderma-related pulmonary arterial hypertension.
    JAAPA 2017 Sep;30(9):11-18
    Katherine Alexis Athanasiou practices rheumatology at the Arthritis Institute of Long Island in Hicksville, N.Y. At Northwell Health System in New Hyde Park, N.Y., Sonu Sahni is a clinical research physician, Amrinder Rana is a research volunteer, and Arunabh Talwar is a physician in the Department of Pulmonary, Critical Care, and Sleep Medicine and director of the Advanced Lung Disease Center. The authors have disclosed no potential conflicts of interest, financial or otherwise.
    Scleroderma is an uncommon autoimmune disease of unknown cause that may affect any organ system in the body. Patients with scleroderma are prone to developing pulmonary complications, including pulmonary arterial hypertension (PAH), that are the leading cause of death in this population. This article describes scleroderma-related PAH and its diagnosis and management. Read More

    EQ-5D studies in musculoskeletal and connective tissue diseases in eight Central and Eastern European countries: a systematic literature review and meta-analysis.
    Rheumatol Int 2017 Aug 28. Epub 2017 Aug 28.
    Department of Health Economics, Corvinus University of Budapest, Fővám tér 8., 1093, Budapest, Hungary.
    EQ-5D is becoming the preferred instrument to measure health-state utilities involved in health technology assessment. The objective of this study is to assess the state of EQ-5D research in musculoskeletal disorders in 8 Central and Eastern European countries and to provide a meta-analysis of EQ-5D index scores. Original research articles published in any language between Jan 2000 and Sept 2016 were included, if they reported any EQ-5D outcome from at least two musculoskeletal patients from Austria, Bulgaria, the Czech Republic, Hungary, Poland, Romania, Slovakia, or Slovenia. Read More

    The Role of NT-proBNP in the Diagnosis of Ventricular Arrhythmias in Patients with Systemic Sclerosis.
    Iran J Public Health 2017 Jul;46(7):906-916
    Rheumatology Clinic, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
    Background: In patients with systemic sclerosis, NT-proBNP is a useful diagnostic marker for pulmonary hypertension and ventricular dysfunction, with important prognostic significance. The aim of this study was to assess the relationship between the NT-proBNP levels and the presence and severity of ventricular arrhythmias in patients with scleroderma.

    Methods: Forty consecutive patients with a diagnostic of systemic sclerosis according to the EULAR criteria admitted at the Rheumatology Clinic of Cluj-Napoca, Romania, from Jan 2014 to Apr 2014 were enrolled. Read More

    Innate lymphoid cells and fibrotic regulation.
    Immunol Lett 2017 Aug 24. Epub 2017 Aug 24.
    Department of Applied Sciences, Faculty of Health and Life Sciences, Northumbria University, Newcastle upon Tyne, UK. Electronic address:
    Innate lymphoid cells (ILCs) are innate immune cells that do not possess B or T cell receptors but belong to the lymphoid lineage. While these cells have not yet been extensively investigated since their classification as a homogenous group, emerging evidence suggests that they exert significant regulatory roles in both tissue remodelling and inflammation, and are therefore, also involved in fibrotic regulation. The following review will serve to outline the transcription factors, surface markers, and cytokines that define each subgroup, and the process by which these cells differentiate. Read More

    Metabolic control of immune system activation in rheumatic diseases.
    Arthritis Rheumatol 2017 Aug 25. Epub 2017 Aug 25.
    Division of Rheumatology, Departments of Medicine, Microbiology and Immunology, Biochemistry and Molecular Biology, State University of New York, Upstate Medical University, College of Medicine, Syracuse, New York, 13210.
    Metabolic pathways exert profound influence over the development of unicellular and multi-cellular organisms. Engagement of antigen receptors and co-stimulatory molecules, growth factors, hormones, cytokines, environmental factors, and other regulatory cues shape the development of the immune system by reprogramming of metabolic gene expression in a cell type-specific manner. In fact, the heterogeneity of cells both within the innate and adaptive immune systems depends on the supply of metabolites that allow for lineage-specific differentiation. Read More

    Update upon efficacy and safety of etanercept for the treatment of spondyloarthritis and juvenile idiopathic arthritis.
    Mod Rheumatol 2017 Aug 24:1-15. Epub 2017 Aug 24.
    a Department of Internal Medicine, Scleroderma Unit, Clinical Immunology Unit , University of Genova and IRCCS-Azienda Ospedaliera Universitaria San Martino , Genova , Italy.
    TNF-α inhibitors have demonstrated efficacy both as monotherapy and in combination with disease-modifying anti-rheumatic drugs (DMARDs) in the treatment of chronic inflammatory immune-mediated diseases such as rheumatoid arthritis, Crohn's disease, ulcerative colitis, ankylosing spondylitis (AS), psoriasis (Ps) and/or psoriatic arthritis (PsA) and may be administered off-label to treat disseminated granuloma annulare, systemic lupus erythematosus and systemic sclerosis. There are several TNF-α inhibitors available for clinical use including infliximab, adalimumab, golimumab, certolizumab pegol and etanercept. In this article, we discuss the efficacy and safety of etanercept in the treatment of spondyloarthritis and juvenile idiopathic arthritis (JIA). Read More

    Intradermal Injections of Bleomycin to Model Skin Fibrosis.
    Methods Mol Biol 2017 ;1627:43-47
    Department of Dermatology, Fukushima Medical University, Fukushima, Japan.
    Systemic sclerosis (scleroderma) is a fibrotic condition characterized by immunologic abnormalities, vascular injury, and increased accumulation of extracellular matrix proteins in the affected organs. Although the etiology of scleroderma has not yet been fully elucidated, a growing body of evidence suggests that extracellular matrix overproduction by activated fibroblasts results from a complex interaction among endothelial cells, immunocytes, and fibroblasts, involving a number of mediators such as cytokines, chemokines, growth factors, and their receptors. For a better understanding of the pathophysiology of scleroderma, animal models are important tools. Read More

    Mapping and predicting mortality from systemic sclerosis.
    Ann Rheum Dis 2017 08 23. Epub 2017 Aug 23.
    Rheumatology A department, Paris Descartes University, INSERM U1016, Sorbonne Paris Cité, Cochin Hospital, Paris, France.
    Objectives: To determine the causes of death and risk factors in systemic sclerosis (SSc).

    Methods: Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Read More

    Endothelin-1, α-Klotho, 25(OH) Vit D levels and severity of disease in scleroderma patients.
    Rheumatol Int 2017 Aug 22. Epub 2017 Aug 22.
    Connective Tissue Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.
    Considering the role of endothelin-1 (ET-1) in tissue remodeling and fibrosis during the development of scleroderma as well as the effect of α-Klotho in pathogenesis of calcinosis and/or endothelial cell injury and its correlation with severity of disease, this study aimed to evaluate serum ET-1, α-Klotho and 25(OH) vitamin D levels in patients with limited and diffuse scleroderma compared to healthy subjects. In this cross-sectional study, 60 scleroderma patients according to the ACR/EULAR 2013 criteria and 60 age- and sex-matched healthy controls were included. In patients, clinical examination was performed and Medsger severity scale was assessed. Read More

    Juvenile systemic sclerosis: experience from a tertiary care center from India.
    Rheumatol Int 2017 Aug 22. Epub 2017 Aug 22.
    Division of Pediatric Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, AIIMS, New Delhi, India.
    Juvenile systemic sclerosis (JSSc) is a rare disorder with paucity of information on its treatment and longterm  outcome. Herein, we are sharing our experience with this rare entity. Case records of children, diagnosed to have systemic sclerosis attending Pediatric Rheumatology Clinic at All India Institute of Medical Sciences, New Delhi from January 1998 to June 2016 were reviewed. Read More

    [Evaluation of the toxicity of Basidiomycota fungi on the hatching of Artemia franciscana cysts].
    Rev Iberoam Micol 2017 Aug 19. Epub 2017 Aug 19.
    Laboratorio de Acuicultura Experimental, Departamento de Ciencias Biológicas, Centro Universitario de la Costa, Universidad de Guadalajara, Puerto Vallarta, Jalisco, México. Electronic address:
    Background: The consumption of wild mushrooms has increased in recent years. However, not all mushrooms are edible and some of them may cause poisoning. Therefore, their toxicity needs to be studied. Read More

    Survey of False-positive Reactivity of Latex Agglutination Test for Kala-azar (Katex) without Urine Sample Boiling Process in Autoimmune Patients.
    Iran J Public Health 2017 Jun;46(6):804-810
    Leishmaniasis Research Center, Kerman University of Medical Sciences, Kerman, Iran.
    Background: Latex agglutination test for Kala-azar (KAtex) is an easy, inexpensive, and field-applicable antigen detection test. However, the main drawback of this method is the boiling step applied to remove false positivity of the test. This study was conducted to survey false positivity results of latex agglutination test for KAtex without boiling process in urine of some autoimmune patients. Read More

    Mortality causes and outcomes in Indigenous populations of Canada, the United States, and Australia with rheumatic disease: A systematic review.
    Semin Arthritis Rheum 2017 Jul 25. Epub 2017 Jul 25.
    Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada; Department of Community Health Sciences, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada. Electronic address:
    Background: Indigenous populations of Canada, America, Australia, and New Zealand have increased rates and severity of rheumatic disease. Our objective was to summarize mortality outcomes and explore disease and social factors related to mortality.

    Methods: A systematic search was performed in medical (Medline, EMBASE, and CINAHL), Indigenous and conference abstract databases (to June 2015) combining search terms for Indigenous populations and rheumatic diseases. Read More

    [Advances in studies on autoimmune diseases treated by halofuginone].
    Zhongguo Zhong Yao Za Zhi 2017 Jun;42(11):2042-2045
    The State Key Laboratory of Pharmaceutical Biotechnology, Medical School, Nanjing University, Nanjing 210093, China.
    Halofuginone (HF) is a derivative of dichroine which is the extract of traditional Chinese medicine. It is widely used as an efficient anticoccidial drug. Recent studies have found that HF has unique biological activities, showing great potential capacities in the treatment of autoimmune diseases. Read More

    Validation of the ACR/EULAR classification criteria for systemic sclerosis in patients with early scleroderma.
    Rheumatol Int 2017 Aug 17. Epub 2017 Aug 17.
    Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, Rua Botucatu 740, 3º andar, São Paulo, SP, 04023-062, Brazil.
    The aim of this study was to validate the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in patients with SSc, including patients with early SSc. Fifty-six consecutive patients with early SSc (2001 LeRoy and Medsger criteria), 122 patients with established SSc (1980 ACR classification criteria), and 141 patients with SSc-like disorders were included in this cross-sectional study. The diagnostic performance of the 2013 ACR/EULAR criteria was compared with the 1980 ACR criteria in several subsets of patients. Read More

    Autoimmune potential of perchloroethylene: Role of lipid-derived aldehydes.
    Toxicol Appl Pharmacol 2017 Oct 14;333:76-83. Epub 2017 Aug 14.
    Department of Pathology, University of Texas Medical Branch, Galveston, TX 77555, USA. Electronic address:
    Tetrachloroethene (perchloroethylene, PCE), an ubiquitous environmental contaminant, has been implicated in inducing autoimmunity/autoimmune diseases (ADs), including systemic lupus erythematosus (SLE) and scleroderma in humans. However, experimental evidence suggesting the potential of PCE in mediating autoimmunity is lacking. This study was, therefore, undertaken to explore PCE's potential in inducing/exacerbating an autoimmune response. Read More

    Understanding coping strategies among people living with scleroderma: a focus group study.
    Disabil Rehabil 2017 Aug 17:1-10. Epub 2017 Aug 17.
    a Lady Davis Institute for Medical Research , Jewish General Hospital , Montréal , Canada.
    Purpose: Systemic sclerosis or scleroderma is a chronic, rare connective tissue disease with negative physical and psychological implications. Coping strategies used by scleroderma patients have not been studied in-depth. The objective of the present study was to gain a greater understanding of the coping strategies employed by people living with scleroderma. Read More

    Nintedanib inhibits macrophage activation and ameliorates vascular and fibrotic manifestations in the Fra2 mouse model of systemic sclerosis.
    Ann Rheum Dis 2017 Aug 16. Epub 2017 Aug 16.
    Department of Internal Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany.
    Background: Nintedanib is an inhibitor targeting platelet-derived growth factor receptor, fibroblast growth factor receptor and vascular endothelial growth factor receptor tyrosine kinases that has recently been approved for the treatment of idiopathic pulmonary fibrosis. The aim of this study was to analyse the effects of nintedanib in the fos-related antigen-2 (Fra2) mouse model of systemic sclerosis (SSc).

    Methods: The effects of nintedanib on pulmonary arterial hypertension with proliferation of pulmonary vascular smooth muscle cells (PVSMCs) and luminal occlusion, on microvascular disease with apoptosis of microvascular endothelial cells (MVECs) and on fibroblast activation with myofibroblast differentiation and accumulation of extracellular matrix were analysed. Read More

    Regulatory T cell deficient scurfy mice exhibit a Th2/M2-like inflammatory response in the skin.
    J Dermatol Sci 2017 Sep 13;87(3):285-291. Epub 2017 Jul 13.
    Department of Dermatology, University of Heidelberg, Im Neuenheimer Feld 440, 69120 Heidelberg, Germany. Electronic address:
    Background: Scurfy mice have a functional defect in regulatory T cells (Treg), which leads to lethal multi-organ inflammation. The missing Treg function results in uncontrolled autoimmune cellular and humoral inflammatory responses. We and others have previously shown that during the course of disease scurfy mice develop severe skin inflammation and autoantibodies including anti-nuclear autoantibodies (ANA). Read More

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