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    MicroRNA-21 in Skin Fibrosis: Potential for Diagnosis and Treatment.
    Mol Diagn Ther 2017 Jul 19. Epub 2017 Jul 19.
    Department of Physiology, Kunming Medical University, 1168 West Chunrong Road, Yuhua Avenue, Chenggong, Kunming, 650500, Yunnan, China.
    Skin fibrosis is a common pathological process characterized by fibroblast proliferation and excessive deposition of extracellular matrix. However, the pathogenesis of the disease is still not clear. Previous studies have shown that microRNA-21 may play pivotal roles in the regulation of a variety of skin fibrosis, including keloid, scleroderma, and hypertrophic scar. Read More

    Are there risk factors for scleroderma-related calcinosis?
    Mod Rheumatol 2017 Jul 19:1-5. Epub 2017 Jul 19.
    b Department of Medicine, Division of Rheumatology and Connective Tissue Research , Rutgers-Robert Wood Johnson Medical School , New Brunswick , NJ , USA.
    Objectives: Pathogenesis and risk factors of scleroderma associated calcinosis is poorly understood and there is no effective treatment. This study was performed to better understand the prevalence and clinical features associated with calcinosis in a cohort of SSc outpatients.

    Methods: In this cross-sectional study, we compared clinical characteristics of SSc patients with (SSc-calcinosis) and without calcinosis (SSc-control) seen in the outpatient Rutgers-RWJ Scleroderma Program between 2012 and 2015. Read More

    Intestinal microbiome in scleroderma: recent progress.
    Curr Opin Rheumatol 2017 Jul 15. Epub 2017 Jul 15.
    Department of Medicine, University of California, David Geffen School of Medicine, California, Los Angeles, USA.
    Purpose Of Review: Our evolving understanding of how gut microbiota affects immune function and homeostasis has led many investigators to explore the potentially pathologic role of gut microbiota in autoimmune diseases. This review will discuss the rapidly advancing field of microbiome research in systemic sclerosis (SSc), an incurable autoimmune disease with significant gastrointestinal morbidity and mortality.

    Recent Findings: Recent reports have identified common perturbations in gut microbiota across different SSc cohorts. Read More

    Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.
    Clin Rev Allergy Immunol 2017 Jul 16. Epub 2017 Jul 16.
    Section of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
    Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. Read More

    Assessment of English-French differential item functioning of the Satisfaction with Appearance Scale (SWAP) in systemic sclerosis.
    Body Image 2017 Jul 13;22:97-102. Epub 2017 Jul 13.
    Departments of Educational and Counselling Psychology; Psychiatry; Medicine; Epidemiology, Biostatistics, and Occupational Health; and Psychology, McGill University and Lady Davis Institute for Medical Research, Jewish General Hospital, Montréal, Québec, Canada.
    The Satisfaction with Appearance Scale (SWAP) has been used to assess body image distress among people with the rare and disfiguring disease systemic sclerosis (SSc); however, it has not been validated across different languages groups. The objective was to examine differential item functioning of the SWAP among 856 Canadian English- or French-speaking SSc patients. Confirmatory factor analysis was used to evaluate the SWAP two-factor structure (Dissatisfaction with Appearance and Social Discomfort). Read More

    Outcomes of sympathectomy and vascular bypass for digital ischaemia in connective tissue disorders.
    J Hand Surg Eur Vol 2017 Jul 1:1753193417718784. Epub 2017 Jul 1.
    1 Division of Plastic, Maxillofacial & Oral Surgery, Duke University Health System, Durham, NC, USA.
    All patients (36 hands) with connective tissue disorders who underwent periarterial sympathectomy of the hand alone or in conjunction with vascular bypass at our institution between 1995-2013 were reviewed. The durable resolution of ulcers was significantly higher in patients treated by periarterial sympathectomy and bypass than in patients treated by periarterial sympathectomy alone. Although there were more digital amputations in patients treated by periarterial sympathectomy alone, the difference was not statistically significant. Read More

    Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study.
    Eur Respir J 2017 Jul 13;50(1). Epub 2017 Jul 13.
    Dept of Internal Medicine, Division of Pulmonology, Medical University of Graz, Graz, Austria.
    Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. Read More

    International consensus: what else can we do to improve diagnosis and therapeutic strategies in patients affected by autoimmune rheumatic diseases (rheumatoid arthritis, spondyloarthritides, systemic sclerosis, systemic lupus erythematosus, antiphospholipid syndrome and Sjogren's syndrome)?: The unmet needs and the clinical grey zone in autoimmune disease management.
    Autoimmun Rev 2017 Jul 10. Epub 2017 Jul 10.
    Department of Pathophysiology, University of Athens, Athens, Greece.
    Autoimmune diseases are a complex set of diseases characterized by immune system activation and, although many progresses have been done in the last 15 years, several unmet needs in the management of these patients may be still identified. Recently, a panel of international Experts, divided in different working groups according to their clinical and scientific expertise, were asked to identify, debate and formulate a list of key unmet needs within the field of rheumatology, serving as a roadmap for research as well as support for clinicians. After a systematic review of the literature, the results and the discussions from each working group were summarised in different statements. Read More

    [Systemic sclerosis and occupational difficulties: Results of a prospective study].
    Rev Med Interne 2017 Jul 10. Epub 2017 Jul 10.
    Service de pathologies professionnelles et maintien en emploi, CHRU de Lille, Lille, France; Laboratoire de recherche, EA4487 axe droit/santé-travail, Lille, France. Electronic address:
    Between 9000 and 14000 people have systemic sclerosis (ScS) in France. The work is often affected. Our study aims to assess the frequency of professional difficulties (DP) of scleroderma patients, identify these DP, the symptoms involved and the solutions used. Read More

    Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist's point of view.
    Korean J Intern Med 2017 Jul 30;32(4):600-610. Epub 2017 Jun 30.
    Division of Allergy and Respiratory Medicine, Department of Internal Medicine, Soon Chun Hyang University Seoul Hospital, Seoul, Korea.
    Interstitial lung disease (ILD) occurs in 15% of patients with collagen vascular disease (CVD), referred to as connective tissue disease (CTD). Despite advances in management strategies, ILD continues to be a significant cause of mortality in patients with CVD-associated ILD (CTD-ILD). There is a lack of randomized, clinical trials assessing pharmacological agents for CTD-ILD, except in cases of ILD-associated systemic sclerosis (SSc). Read More

    Phototherapy in systemic sclerosis: review.
    Photodermatol Photoimmunol Photomed 2017 Jul 13. Epub 2017 Jul 13.
    Division of Dermatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand.
    Systemic scleroderma-also known as systemic sclerosis (SSc)-is a chronic systemic connective tissue disease characterized by collagen deposition in cutaneous and internal organs, leading to skin sclerosis and multiple organ fibrosis. The pathogenesis is complex and remains poorly understood. Treatment is based on organ involvement and requires a multidisciplinary approach. Read More

    Parry Romberg Syndrome and linear facial scleroderma: management in pediatric population.
    J Biol Regul Homeost Agents 2017 Apr-Jun;31(2 Suppl 1):131-138
    Maxillofacial and Dental Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy.
    Among all different types of cutaneous scleroderma, Parry Romberg syndrome and linear scleroderma “en coup de sabre” typically involve the visage. Gradual degeneration of the tissues, from the skin up to the bone, is the stigmata of the diseases and the range of clinical manifestations is wide. They typically start during childhood and slowly progress before stabilizing. Read More

    Autoamputation and Polyneuropathy in Mixed Connective Tissue Disorder: A Case Report.
    Cureus 2017 Jun 5;9(6):e1313. Epub 2017 Jun 5.
    Department of Medicine, Jinnah Postgraduate Medical Center Karachi Pakistan.
    Mixed connective tissue disorder (MCTD) is a multisystem disease with overlapping features of other autoimmune diseases, such as systemic lupus erythematosus (SLE), myositis, rheumatoid arthritis, and scleroderma. MCTD presents with a distinctive antibody in serum known as U1-ribonucleoprotein (RNP). MCTD is quite rare as compared to other connective tissue disorders like SLE, systemic sclerosis, dermatomyositis, and polymyositis. Read More

    Caballeronia mineralivorans sp. nov., isolated from oak-Scleroderma citrinum mycorrhizosphere.
    Syst Appl Microbiol 2017 Jun 7. Epub 2017 Jun 7.
    Université de Lyon, INSA de Lyon, CNRS UMR 5240, Lyon, France.
    Six bacterial strains were isolated from the oak-Scleroderma citrinum ectomycorrhizosphere in acidic and nutrient-poor forest soil for their high efficacy to weather minerals. Four of the six isolates, PML1(12)(T) and PML1(4), PML1(14) and PML1(16), were further characterized extensively. They were Gram negative, obligate aerobic, motile, non spore forming and rod-shaped. Read More

    The "myth" of loss of angiogenesis in systemic sclerosis: a pivotal early pathogenetic process or just a late unavoidable event?
    Arthritis Res Ther 2017 Jul 6;19(1):162. Epub 2017 Jul 6.
    Department of Experimental and Clinical Medicine, Division of Rheumatology and Scleroderma Unit, Azienda Ospedaliera Universitaria Careggi, University of Florence, Viale Pieraccini 18, 50139, Florence, Italy.
    Systemic sclerosis is considered a disease dominated by a "loss of angiogenesis", although in its early phases evidence indicates a disturbed angiogenic response only. In fact, microvascular changes are primarily due to endothelial cell injury, triggering downstream significant enlargement of the capillary in an inflammatory environment, followed by capillary rupture (microhemorrhages). Subsequent pro-angiogenic efforts lead to an aberrant angiogenesis and, eventually, to a total loss of vessel repair and regeneration (loss of angiogenesis). Read More

    Limited cutaneous systemic sclerosis skin demonstrates distinct molecular subsets separated by a cardiovascular development gene expression signature.
    Arthritis Res Ther 2017 Jul 4;19(1):156. Epub 2017 Jul 4.
    Centre for Rheumatology and Connective Tissue Diseases, University College London, London, UK.
    Background: Systemic sclerosis (SSc; scleroderma) is an uncommon autoimmune rheumatic disease characterised by autoimmunity, vasculopathy and fibrosis. Gene expression profiling distinguishes scleroderma from normal skin, and can detect different subsets of disease, with potential to identify prognostic biomarkers of organ involvement or response to therapy. We have performed gene expression profiling in skin samples from patients with limited cutaneous SSc (lcSSc). Read More

    An MIF Promoter Polymorphism Is Associated with Susceptibility to Pulmonary Arterial Hypertension in Diffuse Cutaneous Systemic Sclerosis.
    J Rheumatol 2017 Jul 1. Epub 2017 Jul 1.
    From the Instituto de Parasitología y Biomedicina López-Neyra, IPBLN-CSIC; Unidad de Enfermedades Sistémicas Autoinmunes, Department of Internal Medicine, Hospital Clínico Universitario San Cecilio, Granada; Department of Internal Medicine, Hospital Valle de Hebron; Department of Rheumatology, Hospital de la Santa Creu i de Sant Pau; Department of Internal Medicine, Hospital Universitario de Bellvitge, Barcelona; Department of Rheumatology, Hospital Universitario 12 de Octubre; Department of Rheumatology, Hospital Clínico San Carlos, Madrid; Department of Rheumatology, Hospital Carlos Haya, Málaga; Servicio de Medicina Interna, Hospital Virgen del Rocio, Seville; Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain; Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan, Milan; Department of Medicine, Università degli Studi di Verona, Verona; Servizio di Reumatologia ed Immunologia Clinica Spedali Civili, Brescia, Italy; Department of Rheumatology, Rikshospitalet, Oslo University Hospital, Oslo, Norway; Department of Dermatology, Josefs-Hospital, Ruhr University Bochum, Bochum; Clinic of Rheumatology, University of Lübeck, Lübeck; Klinik für Immunologie und Rheumatologie, Hannover Medical School, Hannover; Department of Dermatology, University of Cologne, Cologne, Germany; Department of Rheumatology, Radboud University Nijmegen Medical Centre, Nijmegen; VU University Medical Center, Amsterdam; Department of Rheumatology, Leiden University Medical Center, Leiden; Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, the Netherlands; Glasgow Biomedical Research Centre, University of Glasgow, Glasgow; Arthritis Research UK Epidemiology Unit, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. Supported by the following grants: JM was funded by SAF2015-66761-P from the Spanish Ministry of Economy and Competitiveness. This study was also funded by PI-0590-2010, from Consejería de Salud y Bienestar Social, Junta de Andalucía, Spain. ELI was supported by Ministerio de Educación, Cultura y Deporte through the program FPU. L. Bossini-Castillo, PhD, Instituto de Parasitología y Biomedicina López-Neyra, IPBLN-CSIC; D. Campillo-Davó, PhD, Instituto de Parasitología y Biomedicina López-Neyra, IPBLN-CSIC; E. López-Isac, PhD, Instituto de Parasitología y Biomedicina López-Neyra, IPBLN-CSIC; F.D. Carmona, PhD, Instituto de Parasitología y Biomedicina López-Neyra, IPBLN-CSIC; C.P. Simeon, MD, Department of Internal Medicine, Hospital Valle de Hebron; P. Carreira, MD, Department of Rheumatology, Hospital Universitario 12 de Octubre; J.L. Callejas-Rubio, MD, Unidad de Enfermedades Sistémicas Autoinmunes, Department of Internal Medicine, Hospital Clínico Universitario San Cecilio; I. Castellví, MD, Department of Rheumatology, Hospital de la Santa Creu i de Sant Pau; A. Fernández-Nebro, MD, Department of Rheumatology, Hospital Carlos Haya; L. Rodríguez-Rodríguez, MD, Department of Rheumatology, Hospital Clínico San Carlos; M. Rubio-Rivas, MD, Department of Internal Medicine, Hospital Universitario de Bellvitge; F.J. García-Hernández, MD, Servicio de Medicina Interna, Hospital Virgen del Rocio; A.B. Madroñero, MD, Department of Internal Medicine, Hospital General San Jorge; L. Beretta, MD, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan; A. Santaniello, MD, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan; C. Lunardi, MD, Department of Medicine, Università degli Studi di Verona; P. Airó, MD, Servizio di Reumatologia ed Immunologia Clinica Spedali Civili; A.M. Hoffmann-Vold, MD, Department of Rheumatology, Rikshospitalet, Oslo University Hospital; A. Kreuter, MD, Department of Dermatology, Josefs-Hospital, Ruhr University Bochum; G. Riemekasten, MD, Clinic of Rheumatology, University of Lübeck; T. Witte, MD, Klinik für Immunologie und Rheumatologie, Hannover Medical School; N. Hunzelmann, MD, Department of Dermatology, University of Cologne; M.C. Vonk, MD, Department of Rheumatology, Radboud University Nijmegen Medical Centre; A.E. Voskuyl, MD, VU University Medical Center; J. de Vries-Bouwstra, MD, Department of Rheumatology, Leiden University Medical Center; P. Shiels, MD, Glasgow Biomedical Research Centre, University of Glasgow; A. Herrick, MD, Arthritis Research UK Epidemiology Unit, The University of Manchester, Manchester Academic Health Science Centre; J. Worthington, PhD, Arthritis Research UK Epidemiology Unit, The University of Manchester, Manchester Academic Health Science Centre; T.R. Radstake, MD, Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht; J. Martin, MD, PhD, Instituto de Parasitología y Biomedicina López-Neyra, IPBLN-CSIC. L. Bossini-Castillo, D. Campillo-Davó, and E. López-Isac contributed equally to this study. Address correspondence to E. López-Isac, Instituto de Parasitología y Biomedicina López-Neyra, IPBLN-CSIC, Parque Tecnológico Ciencias de la Salud, Avenida del Conocimiento s/n 18016-Armilla (Granada), Spain. E-mail: Accepted for publication May 5, 2017.
    Objective: Systemic sclerosis (SSc) is a fibrotic immune-mediated disease of unknown etiology. Among its clinical manifestations, pulmonary involvement is the leading cause of mortality in patients with SSc. However, the genetic factors involved in lung complication are not well defined. Read More

    The impact of high-dose narrowband ultraviolet A1 on dermal thickness, collagen and matrix-metalloproteinases in animal model of scleroderma.
    J Photochem Photobiol B 2017 Jun 21;173:448-455. Epub 2017 Jun 21.
    State Research Institute Centre for Innovative Medicine, Santariskiu St. 5, LT-08406 Vilnius, Lithuania; Vilnius University, Faculty of Medicine, Center of Rheumatology, Santariskiu St. 2, LT-08661 Vilnius, Lithuania. Electronic address:
    Objective: The main purpose of the present study was to define the impact of high-dose of 365±5nm ultraviolet A1 (UVA1) on dermal fibrosis in the pre-established, bleomycin-induced mouse model of scleroderma.

    Methods: DBA/2 strain mice with the pre-established, bleomycin-induced scleroderma were irradiated with cumulative UVA1 dose of 1200J/cm(2) and in parallel were challenged with prolonged administration of bleomycin. Non-treated groups served as the control. Read More

    Adiponectin is an endogenous anti-fibrotic mediator and therapeutic target.
    Sci Rep 2017 Jun 30;7(1):4397. Epub 2017 Jun 30.
    Northwestern Scleroderma Program, Feinberg School of Medicine, Chicago, IL, 60611, USA.
    Skin fibrosis in systemic sclerosis (SSc) is accompanied by attrition of dermal white adipose tissue (dWAT) and reduced levels of circulating adiponectin. Since adiponectin has potent regulatory effects on fibroblasts, we sought to assess adiponectin signaling in SSc skin biopsies, and evaluate fibrosis in mice with adiponectin gain- and loss-of-function mutations. Furthermore, we investigated the effects and mechanism of action of agonist peptides targeting adiponectin receptors in vitro and in vivo. Read More

    Clinical determinants of elevated systolic pulmonary artery pressure measured by transthoracic Doppler echocardiography in early systemic sclerosis.
    Clin Exp Rheumatol 2017 Jun 20. Epub 2017 Jun 20.
    Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Florence, Italy.
    Objectives: To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Doppler-echocardiography (TTE) in patients with early systemic sclerosis (SSc).

    Methods: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research (EUSTAR) database was performed. SSc patients with <3 years from the first non-Raynaud's phenomenon (RP) symptom at baseline EUSTAR visit, were selected. Read More

    Incidence and prevalence of Systemic Sclerosis (SSc) in Poland - differences between rural and urban regions.
    Ann Agric Environ Med 2017 May;24(2):240-244
    Institute of Rural Health, Lublin, Poland.
    [b] Abstract Introduction.[/b] Systemic sclerosis (SSc) is a rare and potentially severe connective tissue disease, characterized by skin fibrosis and involvement of internal organs. Because of its rarity and heterogeneous clinical presentation, reliable epidemiological studies on SSc have been particularly difficult to carry out. Read More


    Deep Proteome Profiling Reveals Common Prevalence of MZB1-positive Plasma B Cells in Human Lung and Skin Fibrosis.
    Am J Respir Crit Care Med 2017 Jun 27. Epub 2017 Jun 27.
    Univerity of Munich-Comprehensive Pneumology Center, Helmholtz Zentrum München, Munich/Neuherberg, Germany ;
    Rationale: Analyzing the molecular heterogeneity of different forms of organ fibrosis may reveal common and specific factors and thus identify potential future therapeutic targets.

    Objectives: We sought to use proteome-wide profiling of human tissue fibrosis to (1) identify common and specific signatures across endstage interstitial lung disease (ILD) cases, (2) characterize ILD subgroups in an unbiased fashion, and (3) identify common and specific features of lung and skin fibrosis.

    Methods: We collected samples of ILD tissue (n=45) and healthy donor controls (n=10), as well as fibrotic skin lesions from localized scleroderma and uninvolved skin (n=6). Read More

    Photodynamic therapy in dermatology beyond non-melanoma cancer: an update.
    Photodiagnosis Photodyn Ther 2017 Jun 21. Epub 2017 Jun 21.
    Wellman Center for Photomedicine, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Dermatology, Harvard Medical School, Boston, MA 02115, USA; Harvard-MIT Division of Health Sciences and Technology, Cambridge, MA 02139, USA. Electronic address:
    Photodynamic therapy (PDT) employs a photosensitizer (PS) and visible light in the presence of oxygen, leading to production of cytotoxic reactive oxygen species, which can damage the cellular organelles and cause cell death. In dermatology PDT has usually taken the form of topical application of a precursor in the heme biosynthesis pathway, called 5-aminolevulinic acid (or its methyl ester), so that an active PS, protoporphyrin IX accumulates in the skin. As PDT enhances dermal remodeling and resolves chronic inflamation, it has been used to treat cutaneous disorders include actinic keratoses, acne, viral warts, skin rejuvenation, psoriasis, localized scleroderma, some non-melanoma skin cancers and port-wine stains. Read More

    Sex-biased eicosanoid biology: Impact for sex differences in inflammation and consequences for pharmacotherapy.
    Biochem Pharmacol 2017 Jun 22. Epub 2017 Jun 22.
    Institute of Pharmacy, Friedrich-Schiller-University Jena, Philosophenweg 14, D-07743 Jena, Germany. Electronic address:
    The incidence, severity and progression of autoimmune diseases (e.g. scleroderma, multiple sclerosis, rheumatoid arthritis) and certain inflammatory diseases (e. Read More

    Six-Minute Walk Test as a Predictor of Diagnosis, Disease Severity, and Clinical Outcomes in Scleroderma-Associated Pulmonary Hypertension: The DIBOSA Study.
    Lung 2017 Jun 23. Epub 2017 Jun 23.
    Respiratory Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, A90, Cleveland, OH, 44195, USA.
    Background: Pulmonary hypertension (PH) is a common complication of scleroderma (SSc) and is a leading cause of morbidity and mortality.

    Objectives: To explore the utility of the 6MWT in the prediction of SSc-PH and to assess its prognostic implications.

    Methods: A retrospective review of SSc patients from 2003 to 2013, with 6MWT and echocardiogram (n = 286), was conducted. Read More

    Case report of robotic dor fundoplication for scleroderma esophagus with aperistalsis on manometry.
    Int J Surg Case Rep 2017 Jun 16;37:69-71. Epub 2017 Jun 16.
    Department of Surgery, Texas Tech University Health Sciences Center, El Paso, TX, United States.
    Scleroderma is a systemic disease of collagen deposition resulting in fibrosis of small arteries and arterioles. It commonly affects the skin, lungs, and gastrointestinal tract. The most common site of GI tract involvement is the esophagus. Read More

    Capillaroscopy In Routine Diagnostics: Potentials And Limitations.
    Curr Rheumatol Rev 2017 06 14. Epub 2017 Jun 14.
    Research Laboratory and Academic Division of Clinical Rheumatology, University of Genova, Genova. Italy.
    Nailfold capillaroscopy is a safe and useful investigational tool that allows an early detection and a qualitative description of the microvascular abnormalities in patients with Raynaud's phenomenon secondary to scleroderma-spectrum disorders. Nowadays, the role of capillaroscopy in the diagnosis of systemic sclerosis is well known. Capillaroscopy has been included in the new 2013 classification criteria for systemic sclerosis and it is considered a key investigation in the very early phases of the disease. Read More

    Nailfold Capillaroscopy Within and Beyond the Scope of Connective Tissue Diseases.
    Curr Rheumatol Rev 2017 Jun 14. Epub 2017 Jun 14.
    Justus-Liebig University Gießen, Kerckhoff Clinic, Bad Nauheim. Germany.
    Nailfold capillaroscopy is a noninvasive instrumental method for morphological analysis of the nutritive capillaries in the nailfold area. In rheumatology, it is a method of choice among instrumental modalities for differentiation of primary and secondary Raynaud's phenomenon (RP) in rheumatic diseases. RP is a common diagnostic problem in rheumatology. Read More

    Improvement of peripheral artery disease with Sildenafil and Bosentan combined therapy in a patient with limited cutaneous systemic sclerosis: A case report.
    Medicine (Baltimore) 2017 Jun;96(25):e6988
    aUnité de Médecine Vasculaire, INSERM CIC 1414, Pôle Imagerie Médicale et Explorations Fonctionnelles, CHU de Rennes, 35033 Rennes Cedex, France. bMitoVasc Institute, UMR CNRS 6015-INSERM U1083, Angers University Hospital, Angers Cedex cUnité de Médecine Vasculaire, Plateau des Consultations Médicales, Cholet Cedex France.
    Rationale: Sildenafil, a phosphodiesterase-5-inhibitor and Bosentan, an endothelin-1-receptor antagonist combined therapy could have beneficial effect in systemic sclerosis (SSc) patients with peripheral artery disease.

    Patient Concerns: We report a case of a 48-year-old Black woman, who developed severe left limb claudication and walking limitation following a left femoropopliteal bypass occlusion in 2014. She was a heavy smoker and had a history of right middle cerebral artery ischemic stroke and bilateral Raynaud phenomenon. Read More

    Preliminary safety and efficacy profile of prucalopride in the treatment of systemic sclerosis (SSc)-related intestinal involvement: results from the open label cross-over PROGASS study.
    Arthritis Res Ther 2017 Jun 20;19(1):145. Epub 2017 Jun 20.
    Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Via Pace 9, 20122, Milano, Italy.
    Background: Prokinetics are used to treat enteric dismotility symptoms in systemic sclerosis (SSc) patients, but they often lack adequate efficacy. The most effective prokinetics belonging to the serotonin (5-HT4) receptor agonists class were withdrawn due to cardiac toxicity in relation to modest 5-HT4 receptor affinity. Prucalopride is a high-affinity 5-HT4 receptor agonist with no major cardiac issues, for which the efficacy in SSc has not yet been assessed. Read More

    [Chronic stress and mental disorders in patients with systemic scleroderma: Results of an interdisciplinary study].
    Ter Arkh 2017 ;89(5):26-32
    Moscow Research Institute of Psychiatry, Branch, V.P. Serbsky Federal Medical Research Center of Psychiatry and Narcology, Ministry of Health of Russia, Moscow, Russia.
    Aim: To analyze of the prevalence of stressful factors and mental disorders (MDs), as well as their clinical psychopathological and clinical psychological characteristics to improve the comprehensive diagnosis and treatment of systemic scleroderma (SSD).

    Subjects And Methods: Examinations were performed in 110 patients (predominantly women (n=97 (88.2%); mean age, 49. Read More

    Mortality Risk Prediction in Scleroderma-Related Interstitial Lung Disease: The SADL Model.
    Chest 2017 Jun 16. Epub 2017 Jun 16.
    Department of Medicine, University of California, San Francisco, San Francisco, CA; Department of Medicine, University of Colorado Denver, Denver, CO.
    Background: Interstitial lung disease (ILD) is an important cause of morbidity and mortality in patients with scleroderma (Scl). Risk prediction and prognostication in patients with Scl-ILD are challenging because of heterogeneity in the disease course.

    Methods: We aimed to develop a clinical mortality risk prediction model for Scl-ILD. Read More

    Evaluation of cancer-associated myositis and scleroderma autoantibodies in breast cancer patients without rheumatic disease.
    Clin Exp Rheumatol 2017 Jun 19. Epub 2017 Jun 19.
    Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore; and Division of Medical Oncology, Department of Medicine, Johns Hopkins University School of Medicine Baltimore MD, USA.
    Objectives: Systemic sclerosis (scleroderma) and dermatomyositis are two prototypic autoimmune diseases that are strongly associated with malignancy. While specific autoantibodies in these diseases are markers of an increased risk of cancer at scleroderma and dermatomyositis onset, it is not known whether these autoantibodies are biomarkers of cancer risk in patients without rheumatic disease.

    Methods: In a matched case-control study of women without rheumatic disease, identified from a familial breast cancer cohort, 50 breast cancer cases and 50 controls were assayed for 3 autoantibodies that are known markers of cancer-associated scleroderma and dermatomyositis: anti-RNA polymerase III, anti-NXP2, and anti-TIF1γ. Read More

    High-resolution manometry compared with the University of California, Los Angeles Scleroderma Clinical Trials Consortium GIT 2.0 in Systemic Sclerosis.
    Semin Arthritis Rheum 2017 May 19. Epub 2017 May 19.
    Department of Medicine, Division of Rheumatology, University of California in Los Angeles(emeritus), Los Angeles, California; Department of Rheumatology, Division of Rheumatology, University of Washington, Seattle Washington; Division of Rheumatology and Experimental Medicine, University of Florence, Florence, Italy.
    Objectives: To study esophageal high resolution manometry (HRM) in systemic sclerosis (SSc) patients and the correlation of findings to The University of California, Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA SCTC_GIT 2.0). Read More

    Evaluation and management approaches for scleroderma lung disease.
    Ther Adv Respir Dis 2017 Aug 16;11(8):327-340. Epub 2017 Jun 16.
    Division of Rheumatology, Massachusetts General Hospital, 55 Fruit Street, Yawkey 2C-2100, Boston, MA 02114, USA.
    Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are leading causes of morbidity and mortality in systemic sclerosis (SSc). As symptoms are often under-reported in SSc, early screening of ILD and PAH is of paramount importance, and early treatment may be associated with better clinical outcomes. Serologies are particularly helpful in identifying patients at risk for pulmonary involvement. Read More

    Rituximab versus cyclophosphamide for the treatment of connective tissue disease-associated interstitial lung disease (RECITAL): study protocol for a randomised controlled trial.
    Trials 2017 Jun 15;18(1):275. Epub 2017 Jun 15.
    NIHR Biomedical Research Unit, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK.
    Background: Interstitial lung disease (ILD) frequently complicates systemic autoimmune disorders resulting in considerable morbidity and mortality. The connective tissue diseases (CTDs) most frequently resulting in ILD include: systemic sclerosis, idiopathic inflammatory myositis (including dermatomyositis, polymyositis and anti-synthetase syndrome) and mixed connective tissue disease. Despite the development, over the last two decades, of a range of biological therapies which have resulted in significant improvements in the treatment of the systemic manifestations of CTD, the management of CTD-associated ILD has changed little. Read More

    The cumulative number of micro-haemorrhages and micro-thromboses in nailfold videocapillaroscopy is a good indicator of disease activity in systemic sclerosis: a validation study of the NEMO score.
    Arthritis Res Ther 2017 Jun 13;19(1):133. Epub 2017 Jun 13.
    UOC Day Hospital of Rheumatology, Department of Rheumatology, ASST G. Pini-CTO, Milan, Italy.
    Background: Some abnormalities in nailfold videocapillaroscopy (NVC), such as the presence of micro-haemorrhages (MHEs), micro-thromboses (MTs), giant capillaries (GCs) and reduction in the number of capillaries (nCs), suggest a disease activity (DA) phase in systemic sclerosis (SSc). In a previous paper, we showed that the number of micro-haemorrhages and micro-thromboses (the so-called NEMO score) was the NVC feature more closely associated with DA. The present study was aimed at validating the NEMO score as a measure of DA in patients with SSc. Read More

    External Cervical Resorption: A New Oral Manifestation of Systemic Sclerosis.
    J Endod 2017 Jun 9. Epub 2017 Jun 9.
    Human Anatomy and Embryology Unit, Department of Experimental Pathology and Therapeutics, Faculty of Medicine and Health Sciences, University of Barcelona, Barcelona, Spain.
    Systemic sclerosis (SSc) is a complex, chronic, and progressive autoimmune disease. SSc causes bone resorption of mandible and distal phalanges of fingers through a known mechanism, and it has also been pointed out as a possible cause of apical root resorption of teeth, because tooth resorption is regulated by similar mechanisms to those controlling bone resorption. The objective of this article was to report the first case in the literature of external cervical resorption (ECR) as an oral manifestation of SSc. Read More

    Scleroderma-like Disorders.
    Curr Rheumatol Rev 2017 06 11. Epub 2017 Jun 11.
    Fortis Escorts Hospital, Jaipur, India
    Scleroderma is a term used to describe diseases that involve hardening and tightening of the skin and the underlying subcutaneous connective tissue. It could be localized to skin and subcutaneous tissue, or may involve the internal organs too in systemic sclerosis. There are disorders that can cause hardening and tightening of skin and mimic scleroderma but are rarely associated with Raynaud phenomenon, sclerodactyly, and autoantibodies in the serum, features specific to scleroderma/systemic sclerosis. Read More

    Angiogenic and angiostatic factors in renal scleroderma-associated vasculopathy.
    Microvasc Res 2017 Jun 8;114:41-45. Epub 2017 Jun 8.
    Department of Clinical Medicine, Sapienza University of Rome, Rome, Italy.
    Background: The angiogenesis in systemic sclerosis (SSc) is impaired. An imbalance of pro-angiogenic factors and angiogenesis inhibitors has been implicated in the progression of peripheral microvascular damage, defective vascular repair and fibrosis. Intrarenal resistance index are considered markers of renal vasculopathy. Read More

    Manufacture of Autologous CD34(+) Selected Grafts in the NIAID-Sponsored HALT-MS and SCOT Multicenter Clinical Trials for Autoimmune Diseases.
    Biol Blood Marrow Transplant 2017 Jun 30. Epub 2017 Jun 30.
    National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland. Electronic address:
    To ensure comparable grafts for autologous hematopoietic cell transplantation (HCT) in the National Institute of Allergy and Infectious Diseases-sponsored Investigational New Drug protocols for multiple sclerosis (HALT-MS) and systemic sclerosis (SCOT), a Drug Master File approach to control manufacture was implemented, including a common Master Production Batch Record and site-specific SOPs with "Critical Elements." We assessed comparability of flow cytometry and controlled rate cryopreservation among sites and stability of cryopreserved grafts using hematopoietic progenitor cells (HPCs) from healthy donors. Hematopoietic Progenitor Cells, Apheresis-CD34+ Enriched, for Autologous Use (Auto-CD34(+)HPC) graft specifications included ≥70% viable CD34(+) cells before cryopreservation. Read More

    The role of platelets in autoimmunity, vasculopathy, and fibrosis: Implications for systemic sclerosis.
    Semin Arthritis Rheum 2017 May 23. Epub 2017 May 23.
    Division of Rheumatology, Department of Internal Medicine, University of Patras Medical School, Patras University Hospital, 26504 Rion, Patras, Greece. Electronic address:
    Introduction: Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy, autoimmunity, and widespread dermal and visceral fibrosis. This article summarizes the current knowledge about the potential contribution of platelets in the disease process and the rationale of targeting platelets as an adjunct treatment for SSc.

    Methods: We performed an electronic search (Medline) using the keywords platelets, systemic sclerosis, autoimmunity, fibrosis, Raynaud, and pulmonary arterial hypertension. Read More

    Scleroderma Induced by Pembrolizumab: A Case Series.
    Mayo Clin Proc 2017 Jul 7;92(7):1158-1163. Epub 2017 Jun 7.
    Division of Rheumatology, Mayo Clinic, Rochester, MN. Electronic address:
    Immune checkpoint inhibitors are approved for select cancer treatment and have shown survival benefit in patients with advanced melanoma. Adverse events, including immune-related adverse events, are common and potentially life-threatening. We describe cases of 2 patients with scleroderma (patient 1 had diffuse scleroderma, and patient 2 had limited scleroderma) that developed while they were receiving pembrolizumab therapy for metastatic melanoma. Read More

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