27,787 results match your criteria Systemic Sclerosis


Maxillofacial Implications of Scleroderma and Systemic Sclerosis: A Case Report and Literature Review.

J Oral Maxillofac Surg 2019 Jan 28. Epub 2019 Jan 28.

Assistant Professor and Associate Program Director, Department of Oral and Maxillofacial Surgery, University of Rochester Medical Center, Eastman Institute for Oral Health, Rochester, NY.

Scleroderma and more generally systemic sclerosis comprise a group of autoimmune diseases characterized by increased collagen synthesis and deposition within connective tissue. The Scleroderma Foundation estimates that as many as 300,000 persons in the United States have scleroderma. Although the etiology of the disease is unknown, it is thought to be related to chronic inflammation secondary to autoimmune tissue damage. Read More

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http://dx.doi.org/10.1016/j.joms.2019.01.027DOI Listing
January 2019

Histone modifications underlie monocyte dysregulation in patients with systemic sclerosis, underlining the treatment potential of epigenetic targeting.

Ann Rheum Dis 2019 Feb 6. Epub 2019 Feb 6.

Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.

Background And Objective: Systemic sclerosis (SSc) is a severe autoimmune disease, in which the pathogenesis is dependent on both genetic and epigenetic factors. Altered gene expression in SSc monocytes, particularly of interferon (IFN)-responsive genes, suggests their involvement in SSc development. We investigated the correlation between epigenetic histone marks and gene expression in SSc monocytes. Read More

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http://dx.doi.org/10.1136/annrheumdis-2018-214295DOI Listing
February 2019

Perspectives on the interlinked nature of systemic sclerosis and reflux disease.

Expert Rev Gastroenterol Hepatol 2019 Mar 4;13(3):213-227. Epub 2019 Jan 4.

b Department of Rheumatology and Internal Medicine , Medical University of Bialystok , Bialystok , Poland.

Introduction: Systemic sclerosis (SSc) is a multisystem connective tissue disease, characterized by chronic inflammation and vascular changes that result in esophageal smooth muscle atrophy and fibrosis. Subsequent progressive loss of peristalsis in the distal esophagus and loss of lower esophageal sphincter function lead to problems with the protective barrier and exposure of sensitive tissues to the gastroduodenal contents, a disorder called reflux disease. Areas covered: Depending on the range, nature and symptoms of the disease, the term 'reflux disease' may refer to gastroesophageal reflux, laryngopharyngeal reflux, microaspiration into the airways and silent reflux. Read More

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http://dx.doi.org/10.1080/17474124.2019.1561274DOI Listing

GAS5 genomic variants and risk of multiple sclerosis.

Neurosci Lett 2019 Feb 18. Epub 2019 Feb 18.

Phytochemistry Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Electronic address:

The lncRNA Growth arrest-specific 5 (GAS5) has crucial roles in the apoptosis, suppression of cell growth and regulation of response to glucocorticoids. Previous studies have demonstrated its role in the pathogenesis of some immune-related disorders such as systemic lupus erythematosus and multiple sclerosis (MS). In the current study, we genotyped two possibly functional GAS5 polymorphisms (rs2067079 and rs6790) in 810 individuals including 410 MS patients and 400 age and sex-matched healthy subjects. Read More

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http://dx.doi.org/10.1016/j.neulet.2019.02.028DOI Listing
February 2019

Support Groups in Scleroderma.

Curr Rheumatol Rep 2019 Feb 21;21(4). Epub 2019 Feb 21.

Lady Davis Institute of the Jewish General Hospital, 4333 Côte-Sainte-Catherine Road,, Montréal, Québec, H3T 1E4, Canada.

Purpose Of Review: This review presents evidence on support group effectiveness in common diseases, research on support groups in systemic sclerosis (SSc), and work underway by the Scleroderma Patient-centered Intervention Network in collaboration with patient organizations to train SSc support group leaders in order to improve support group access and the experiences of support groups for members and leaders.

Recent Findings: Giving and receiving emotional and practical support from others with SSc is an important reason that individuals with SSc attend support groups, but many patients cannot access support groups. SSc support group leaders report confidence in their ability to facilitate groups, but are less confident in tasks such as managing group dynamics and sustaining the group. Read More

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http://dx.doi.org/10.1007/s11926-019-0808-yDOI Listing
February 2019

Association of TNFSF4 (OX40L) polymorphisms with systemic sclerosis-related calcinosis.

Rheumatology (Oxford) 2019 Feb 20. Epub 2019 Feb 20.

Institute of Parasitology and Biomedicine López-Neyra, IPBLN-CSIC, PTS Granada, Granada, Spain.

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http://dx.doi.org/10.1093/rheumatology/kez015DOI Listing
February 2019

Arrhythmia in systemic sclerosis: catheter ablation or not?

Scand J Rheumatol 2019 Feb 21:1-2. Epub 2019 Feb 21.

a Department of Cardiology , Peking Union Medical College Hospital, Chinese Academy of Medical Sciences , Beijing , China.

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http://dx.doi.org/10.1080/03009742.2018.1555281DOI Listing
February 2019

Dasatinib for chronic myelogenous leukemia improves skin symptoms of systemic sclerosis.

Int J Hematol 2019 Feb 20. Epub 2019 Feb 20.

Department of Hematology, Ome Municipal General Hospital, 4-16-5, Higashiome, Ome, Tokyo, Japan.

A 64-year-old man was diagnosed with limited cutaneous systemic sclerosis 5 years prior to this report. His sclerotic skin symptoms did not respond to oral low-dose prednisone (5-10 mg/day). Five years after the diagnosis, the patient presented with leukocytosis 3. Read More

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http://dx.doi.org/10.1007/s12185-019-02618-wDOI Listing
February 2019

Adipokine expression in systemic sclerosis lung and gastrointestinal organ involvement.

Cytokine 2019 Feb 19;117:41-49. Epub 2019 Feb 19.

Dept of Rheumatology and Clinical Immunology, Campus Kerckhoff, Justus-Liebig-University, Giessen, Germany.

Objectives: The immunomodulatory properties of adipokines have previously been reported in autoimmune disorders. Less is known about the role of adipokines in systemic sclerosis (SSc). Lung and gastrointestinal tract are frequently involved in SSc; therefore, these organs were analyzed for adipokine expression as well as pulmonary samples of patients suffering from idiopathic pulmonary fibrosis (IPF) as comparison. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10434666183042
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http://dx.doi.org/10.1016/j.cyto.2018.11.013DOI Listing
February 2019
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Do thermographic parameters help to classify patients with early systemic sclerosis?

Rheumatology (Oxford) 2019 Feb 19. Epub 2019 Feb 19.

Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.

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http://dx.doi.org/10.1093/rheumatology/kez018DOI Listing
February 2019

Primary ocular presentation of tuberous sclerosis - A case report.

Indian J Ophthalmol 2019 Mar;67(3):433-435

Department of Ophthalmology, Christian Medical College, Vellore, Tamil Nadu, India.

A 25-year-old man presented with decreased vision in the left eye with hypopigmented elevated subretinal lesion over the optic disk with abnormal vasculature, subretinal and retinal hemorrhages, and fluid in the macula. An area of high spike over the disk with corresponding orbital shadowing was seen on B scan ultrasonography. Fundus fluorescein angiography revealed abnormal vasculature. Read More

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http://dx.doi.org/10.4103/ijo.IJO_925_18DOI Listing

Sjögren's syndrome and systemic lupus erythematosus: links and risks.

Open Access Rheumatol 2019 29;11:33-45. Epub 2019 Jan 29.

Rheumatology Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Sao Paulo, Brazil,

Systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS) may coexist, and they are chronic complex disorders, with an autoimmune background, multifactorial etiology, multiple circulating autoantibodies, and variable prognosis. The prominent feature of SS is the impairment of the lacrimal and salivary glands leading to sicca symptoms. This disease may be classified as primary Sjögren's syndrome (pSS), or secondary Sjögren's syndrome (sSS) since it is often associated to other autoimmune disorders, principally SLE, rheumatoid arthritis, and systemic sclerosis. Read More

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http://dx.doi.org/10.2147/OARRR.S167783DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6357904PMC
January 2019

Oral rehabilitation in a patient with sclerotic-phenotype chronic graft versus host disease: a case report.

Quintessence Int 2019 ;50(3):208-213

Acute myeloid leukemia is a bone marrow malignancy in which blasts count increases by more than 20% in the bone marrow. Allogeneic hematopoietic stem cell transplantation (alloHCT) is a treatment option for these patients with high risk of graft versus host disease (GVHD) development. Chronic GVHD (cGVHD) often mimics a variety of autoimmune conditions such as systemic lupus erythematous or systemic sclerosis. Read More

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http://dx.doi.org/10.3290/j.qi.a41973DOI Listing
January 2019

Juvenile polyautoimmunity in a rheumatology setting.

Autoimmun Rev 2019 Feb 14. Epub 2019 Feb 14.

Center for Autoimmune Diseases Research (CREA), School of Medicine and Health Sciences, Universidad del Rosario, Bogota, Colombia.. Electronic address:

Overt polyautoimmunity (PolyA) corresponds to the presence of more than one well-defined autoimmune disease (AD) manifested clinically in a single patient. The current study aimed to describe the main characteristics of juvenile PolyA in a pediatric rheumatology setting and analyze the chronological aspects, index cases, familial autoimmunity, and clustering pattern. This was a cross-sectional and multicenter study in which 313 children with overt PolyA were included. Read More

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http://dx.doi.org/10.1016/j.autrev.2018.11.006DOI Listing
February 2019
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Elevated kynurenine levels in diffuse cutaneous and anti-RNA polymerase III positive systemic sclerosis.

Clin Immunol 2019 Feb 14. Epub 2019 Feb 14.

Royal Free Hospital, Centre for Rheumatology and Connective Tissue Diseases, UCL, London, UK. Electronic address:

Systemic sclerosis (SSc) is a systemic disease characterized by vasculopathy, progressive fibrosis and autoimmune activation. Tryptophan (Trp) metabolism has been linked to altered immune cell function and to malignancy. We have investigated the role of Trp metabolic pathway in SSc measuring serum Trp, Kynurenine (Kyn) and Trp/Kyn ratio in a cohort of 97 SSc patients and 10 healthy controls. Read More

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http://dx.doi.org/10.1016/j.clim.2018.12.009DOI Listing
February 2019
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Health-related quality of life in systemic sclerosis compared with other rheumatic diseases: a cross-sectional study.

Arthritis Res Ther 2019 Feb 15;21(1):61. Epub 2019 Feb 15.

Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, 03080, Republic of Korea.

Background: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis of the skin and the involvement of multiple internal organs. Previous studies reported poorer health-related quality of life (HRQoL) in patients with SSc compared with the general population. However, very little is known about how HRQoL in SSc patients compares with that in patients with other systemic autoimmune diseases, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and Sjogren's syndrome (SjS). Read More

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http://dx.doi.org/10.1186/s13075-019-1842-xDOI Listing
February 2019
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Lysyl oxidase-a possible role in systemic sclerosis-associated pulmonary hypertension: a multicentre study.

Rheumatology (Oxford) 2019 Feb 15. Epub 2019 Feb 15.

Rheumatology Unit, Bnai-Zion Medical Center, Haifa, Israel.

Objective: Lysyl oxidase (LOX) is an extracellular enzyme that cross-links collagen fibrils. LOX was found to be increased in serum of SSc patients and was suggested to be related to skin fibrosis, yet a vascular source of LOX has been demonstrated in idiopathic pulmonary arterial hypertension (iPAH). We aimed to validate elevated LOX serum levels in SSc and to study its correlation with clinical characteristics and investigate its main source at the tissue level. Read More

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http://dx.doi.org/10.1093/rheumatology/kez035DOI Listing
February 2019
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Cyclophosphamide for Systemic Sclerosis-related Interstitial Lung Disease: A Comparison of Scleroderma Lung Study I and II.

J Rheumatol 2019 Feb 15. Epub 2019 Feb 15.

From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: Accepted for publication October 18, 2018.

Objective: To compare safety and efficacy outcomes between the cyclophosphamide (CYC) arms of Scleroderma Lung Study (SLS) I and II.

Methods: Participants enrolled in the CYC arms of SLS I (n = 79) and II (n = 69) were included. SLS I and II randomized participants to oral CYC for 1 year and followed patients for an additional year off therapy (in SLS II, patients received placebo in Year 2). Read More

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http://dx.doi.org/10.3899/jrheum.180441DOI Listing
February 2019
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Economic Burden among Commercially Insured Patients with Systemic Sclerosis in the United States.

J Rheumatol 2019 Feb 15. Epub 2019 Feb 15.

From the Analysis Group Inc., Boston, Massachusetts; Boehringer Ingelheim Pharmaceuticals Inc., Ridgefield, Connecticut; University of Colorado School of Medicine, Denver, Colorado, USA. This study was funded by Boehringer Ingelheim Pharmaceuticals Inc. Y. Fan is a salaried employee of Boehringer Ingelheim Pharmaceuticals Inc. Z. Zhou, W. Tang, X. Liu, D. Thomason, Z.Y. Zhou, and D. Macaulay are employees of Analysis Group Inc., which has received consultancy fees from Boehringer Ingelheim Pharmaceuticals Inc. Z. Zhou, MS, Analysis Group Inc.; Y. Fan, ScD, Boehringer Ingelheim Pharmaceuticals Inc.; W. Tang, MS, Analysis Group Inc.; X. Liu, MS, Analysis Group Inc.; D. Thomason, MBA, Analysis Group Inc.; Z.Y. Zhou, PhD, Analysis Group Inc.; D. Macaulay, PhD, Analysis Group Inc.; A. Fischer, MD, University of Colorado School of Medicine. Address corresponding to Z. Zhou, 111 Huntington Ave., Boston, Massachusetts 02199-7668, USA. E-mail: Full Release Article. For details see Reprints and Permissions at jrheum.org. Accepted for publication October 16, 2018.

Objective: To quantify healthcare resource utilization (HRU), work loss, and annual direct and indirect healthcare costs among patients with systemic sclerosis (SSc) compared to matched controls in the United States.

Methods: Data were obtained from a large US commercial claims database. Patients were ≥ 18 years old at the index date (first SSc diagnosis) and had ≥ 1 SSc diagnosis in the inpatient (IP) or emergency room (ER) setting, or ≥ 2 SSc diagnoses on 2 different dates in the outpatient (OP) setting between January 1, 2005, and March 31, 2015; continuous enrollment was required during the followup period (12 months after the index date). Read More

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http://dx.doi.org/10.3899/jrheum.180445DOI Listing
February 2019

Long-term Efficacy and Safety of Monotherapy Versus Combination Therapy in Systemic Sclerosis-associated Pulmonary Arterial Hypertension: A Retrospective Cohort Study from the Nationwide Spanish Scleroderma Registry (RESCLE).

J Rheumatol 2019 Feb 15. Epub 2019 Feb 15.

From the Autoimmune Diseases Unit. Department of Internal Medicine. Bellvitge University Hospital. L'Hospitalet de Llobregat, Barcelona, Spain; Department of Internal Medicine. Corporación Sanitaria Universitaria Parc Taulí. Sabadell, Barcelona, Spain; Unit of Autoimmune Diseases, Department of Internal Medicine. Hospital Universitario Vall d'Hebron. Barcelona, Spain; Unit of Systemic Autoimmune Diseases and Thrombosis. Department of Internal Medicine. Complejo Hospitalario Universitario de Vigo. Vigo, Pontevedra, Spain; Department of Internal Medicine. Hospital Universitario Virgen de las Nieves. Granada, Spain; Department of Internal Medicine. Hospital Universitario y Politécnico La Fe. Valencia, Spain; Department of Internal Medicine. Hospital Universitario Mútua Terrassa. Terrassa, Barcelona, Spain; Unit of Autoimmune Diseases, Department of Internal Medicine. Hospital Clínico Universitario Lozano Blesa. Zaragoza, Spain; Department of Systemic Autoimmune Diseases, Clinical Institute of Medicine and Dermatology. Hospital Universitario Clinic. Barcelona, Spain; Department of Internal Medicine. Hospital Universitario Central de Asturias. Oviedo, Asturias, Spain; Unit of Systemic Autoimmune Diseases, Department of Internal Medicine. Hospital Campus de la Salud, Complejo Universitario de Granada. Granada, Spain; Unit of Systemic Autoimmune Diseases, Department of Internal Medicine. Hospital de Cabueñes. Gijón, Asturias, Spain; Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Spain; Unit of Systemic Autoimmune Diseases, Department of Internal Medicine. Consorci Hospitalari de Vic. Vic, Barcelona, Spain; Department of Internal Medicine. Hospital Universitario Rey Juan Carlos. Móstoles, Madrid, Spain; Faculty of Medicine and Health Sciences. Universitat Internacional de Catalunya,Barcelona,Spain. Address correspondence to: Melani Pestaña-Fernández MD; Bellvitge University Hospital, Department of Internal Medicine, Feixa Llarga s/n 08907 L'Hospitalet de Llobregat Barcelona (Spain). Email:

Objective: Monotherapy is an option as first line therapy for pulmonary arterial hypertension (PAH). However, combination therapy is a beneficial alternative. Our objective was to evaluate efficacy of monotherapy vs. Read More

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http://www.jrheum.org/lookup/doi/10.3899/jrheum.180595
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http://dx.doi.org/10.3899/jrheum.180595DOI Listing
February 2019
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Clinical features of systemic sclerosis patients with anti-RNA polymerase III antibody in a single centre in Spain.

Clin Exp Rheumatol 2019 Feb 11. Epub 2019 Feb 11.

Unit of Systemic Autoimmune Diseases, Institut de Recerca Vall d'Hebron, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain.

Objectives: To evaluate the clinical features and survival of patients with positive anti-RNA polymerase III (anti-RNAP III) in a Spanish single centre.

Methods: We analysed 221 patients with SSc according to LeRoy and Medsger criteria. Twenty-six patients with positivity for anti-RNAP III antibodies were compared with 195 negative patients. Read More

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February 2019
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The Clinical Pathophysiology of Chronic Systemic Sclerosis.

Fed Pract 2018 May;35(5):36-43

is a Medical Student, and is an Assistant Professor of Clinical Medicine, both at David Geffen School of Medicine at University of California, Los Angeles. Dr. Hashemi is a Primary Care Attending Physician and the Ambulatory Care Clerkship Director at the VA Greater Los Angeles Healthcare System, West Los Angeles campus.

Primary care providers should monitor disease progression in the skin and in the pulmonary, renal, cardiac, and gastrointestinal systems in patients with systemic sclerosis, a rare autoimmune and connective tissue disease. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367998PMC

Significance of anti-neutrophil cytoplasmic antibodies in systemic sclerosis.

Arthritis Res Ther 2019 Feb 14;21(1):57. Epub 2019 Feb 14.

The University of Melbourne, 41 Victoria Parade Fitzroy, Melbourne, Victoria, 3065, Australia.

Background: Up to 12% of patients with systemic sclerosis (SSc) have anti-neutrophil cytoplasmic antibodies (ANCA). However, the majority of these patients do not manifest ANCA-associated vasculitis (AAV) and the significance of ANCA in these patients is unclear. The aim of this study is to determine the prevalence of ANCA in a well-characterised SSc cohort and to examine the association between ANCA and SSc clinical characteristics, other autoantibodies, treatments and mortality. Read More

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https://arthritis-research.biomedcentral.com/articles/10.118
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http://dx.doi.org/10.1186/s13075-019-1839-5DOI Listing
February 2019
2 Reads

Serum KL-6 levels reflect the severity of interstitial lung disease associated with connective tissue disease.

Arthritis Res Ther 2019 Feb 14;21(1):58. Epub 2019 Feb 14.

Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, 101, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.

Background: Biomarkers have been actively investigated to supplement functional and imaging modalities to predict the severity, therapeutic responsiveness, and progression of connective tissue disease-associated interstitial lung disease (CTD-ILD). This study aimed to evaluate Krebs von den Lungen 6 (KL-6) as a potential biomarker reflecting the severity of CTD-ILD as assessed through computed tomography (CT) and pulmonary function test (PFT) parameters.

Methods: This retrospective study included 549 Korean patients with rheumatoid arthritis, systemic sclerosis, inflammatory myositis, and other CTDs with or without concurrent ILD. Read More

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http://dx.doi.org/10.1186/s13075-019-1835-9DOI Listing
February 2019
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Tolerability, treatment satisfaction and quality of life outcomes in stable multiple sclerosis patients switched from injectable therapies to auto injected intramuscular interferon beta 1a: The SFERA study.

Mult Scler Relat Disord 2019 Feb 6;30:104-109. Epub 2019 Feb 6.

Neurocenter of Southern Switzerland, Ospedale Regionale di Lugano, Via Tesserete 46, 6903 Lugano, Switzerland.

Background: Interferon beta (IFNB) and Glatiramer acetate, long-term first line disease modifying treatments (DMTs) for multiple sclerosis (MS), have different injection frequencies crucial for injection site related side effects. We aimed at investigating whether switching to intramuscular IFNB-1a injected once/week with the Avonex®Pen™ device improves treatment tolerability and quality of life in stable MS patients.

Methods: Clinically stable MS patients, whom their treating neurologist switched from high frequency injectable DMTs to weekly intramuscular IFNB-1a because of bothersome injection site reactions, were included. Read More

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http://dx.doi.org/10.1016/j.msard.2019.02.010DOI Listing
February 2019
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Additional Rheumatologic Tests Critical in Patients with Systemic Sclerosis.

Authors:
Edward S Harris

Am Fam Physician 2019 Feb;99(4):221-222

Madison, WI, USA.

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February 2019

Prevalence, Treatment and Outcomes of Coexistent Pulmonary Hypertension and Interstitial Lung Disease in Systemic Sclerosis.

Arthritis Rheumatol 2019 Feb 14. Epub 2019 Feb 14.

Division of Rheumatology, Department of Internal Medicine, University of Michigan.

Objective: Systemic sclerosis (SSc) is associated with interstitial lung disease (ILD) and pulmonary hypertension (PH). We sought to determine prevalence, characteristics, treatment, and outcomes for subjects with PH in a SSc-associated ILD (SSc-ILD) cohort.

Methods: Subjects with SSc-ILD on high resolution computed tomography (HRCT) were included in a prospective observational cohort. Read More

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http://dx.doi.org/10.1002/art.40862DOI Listing
February 2019
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Scleroderma patient perspectives on social support from close social relationships.

Disabil Rehabil 2019 Feb 14:1-11. Epub 2019 Feb 14.

a Lady Davis Institute for Medical Research, Jewish General Hospital , Montreal , Canada.

Purpose: People with rare diseases must cope with many physical and psychological challenges; an endeavor that can be difficult to carry out without external support. The purpose of this study was to understand how patients with scleroderma perceive the social support they need and receive from close relationships to better manage their disease.

Method: Four focus groups with patients (N = 19) were conducted. Read More

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http://dx.doi.org/10.1080/09638288.2018.1531151DOI Listing
February 2019
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Overexpression of apoptosis-related protein, survivin, in fibroblasts from patients with systemic sclerosis.

Ir J Med Sci 2019 Feb 13. Epub 2019 Feb 13.

Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Background/objectives: Recent studies suggest that, in addition to activation and hypersecretion of matrix components, fibroblasts from patients with systemic sclerosis (SSc) are resistant to apoptosis. Previous studies have shown that survivin, a member of inhibition of apoptosis (IAP) family, plays an important role in apoptosis resistance. Accordingly, we decided to study the expression of the most important members of IAP family in SSc fibroblasts, which can block apoptosis either by binding and inhibiting caspases or through caspase-independent mechanisms. Read More

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http://dx.doi.org/10.1007/s11845-019-01978-wDOI Listing
February 2019

Circulating follicular helper T cells are increased in systemic sclerosis and promote plasmablast differentiation through the IL-21 pathway which can be inhibited by ruxolitinib.

Ann Rheum Dis 2019 Feb 13. Epub 2019 Feb 13.

Centre de Recherche Saint-Antoine (CRSA), Sorbonne Université, INSERM U938, Paris, France

Objectives: Systemic sclerosis (SSc) is an autoimmune disease characterised by widespread fibrosis, microangiopathy and autoantibodies. Follicular helper T (Tfh) cells CD4CXCR5PD-1 cooperate with B lymphocytes to induce the differentiation of plasmocytes secreting immunoglobulins (Ig). Circulating Tfh (cTfh) cells are increased in several autoimmune diseases. Read More

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http://dx.doi.org/10.1136/annrheumdis-2018-214382DOI Listing
February 2019

Systemic Sclerosis and Serum Content of Transforming Growth Factor.

Adv Exp Med Biol 2019 Feb 14. Epub 2019 Feb 14.

Department of Rheumatology and Internal Diseases, Poznan University of Medical Sciences, Poznan, Poland.

Systemic sclerosis is a connective tissue disease characterized by tissue fibrosis leading to interstitial lung disease. Transforming growth factor-β (TGF-β) has been of interest as a potential diagnostic marker and also as a drug target in systemic sclerosis. The aim of this study was to assess the serum content of TGF-β1 in patients with systemic sclerosis and to assess its potential role in tissue fibrosis. Read More

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http://link.springer.com/10.1007/5584_2019_341
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http://dx.doi.org/10.1007/5584_2019_341DOI Listing
February 2019
3 Reads

Surgical management of pulmonary aspergillosis in pediatric population.

J Pediatr Surg 2019 Jan 11. Epub 2019 Jan 11.

Introduction: Aspergillosis is an invasive infection that affects mainly immunocompromised patients, and the lung is the most common site of infection. We reviewed our experience with the surgical management of pulmonary aspergillosis (PA) in pediatric population at a tertiary care institution.

Methods: A retrospective chart review was conducted for pediatric patients (<16 years) who were managed for PA over the period from January 2001 to December 2016. Read More

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http://dx.doi.org/10.1016/j.jpedsurg.2019.01.003DOI Listing
January 2019
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Exocrine pancreatic function is preserved in systemic sclerosis.

Arthritis Res Ther 2019 Feb 12;21(1):52. Epub 2019 Feb 12.

Section of Rheumatology, Department of Clinical Sciences, Lund University, 221 85, Lund, Sweden.

Background: Systemic sclerosis (SSc) has been suggested to cause exocrine pancreatic dysfunction. However, a case-control-based autopsy study failed to associate systemic sclerosis with any pancreatic histopathology. The primary objective of this study was to examine the exocrine pancreatic function in consecutive SSc patients in relation to an age- and sex-matched control group. Read More

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https://arthritis-research.biomedcentral.com/articles/10.118
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http://dx.doi.org/10.1186/s13075-019-1840-zDOI Listing
February 2019
2 Reads

Second autologous haematopoietic stem cell transplantation in systemic sclerosis-a case report.

Rheumatology (Oxford) 2019 Feb 8. Epub 2019 Feb 8.

Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.

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http://dx.doi.org/10.1093/rheumatology/kez010DOI Listing
February 2019
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Microparticles in systemic sclerosis: Potential pro-inflammatory mediators and pulmonary hypertension biomarkers.

Respirology 2019 Feb 12. Epub 2019 Feb 12.

Stanley S. Scott Cancer Center, Louisiana State University Health Sciences Center, New Orleans, LA, USA.

Background And Objective: Endothelial microparticles (EMP) are submicron vesicles released from endothelial cells. We aimed to determine the utility of EMP as biomarkers of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) patients and the pathogenic role of microparticles (MP) in vascular inflammation.

Methods: Levels of EMP (CD144+, CD31+, CD62E+ and CD143+) were compared between three groups (10 SSc patients with PAH, 10 SSc patients without pulmonary hypertension (no-PH) and 10 healthy age- and sex-matched controls). Read More

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http://dx.doi.org/10.1111/resp.13500DOI Listing
February 2019
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Macrophage migration inhibitory factor polymorphisms are a potential susceptibility marker in systemic sclerosis from southern Mexican population: association with MIF mRNA expression and cytokine profile.

Clin Rheumatol 2019 Feb 12. Epub 2019 Feb 12.

Instituto de Investigación en Ciencias Biomédicas, Centro Universitario de Ciencias de la Salud, Universidad de Guadalajara, Guadalajara, Jalisco, Mexico.

Introduction: Systemic sclerosis (SSc) is a complex autoimmune disease, characterized by microvascular lesions, autoimmunity, and fibrosis. It is suggested that MIF participates in the amplification of the proinflammatory process in SSc; moreover, the promoter polymorphisms - 794 CATT (rs5844572) and - 173G>C (rs755622) in the MIF gene have been associated with an increase in MIF serum levels in several autoimmune diseases. The aim of this study was to analyze the relationship of the - 794 CATT and - 173G>C MIF polymorphisms with mRNA expression, MIF serum levels, and the Th1/Th2/Th17 cytokine profile in SSc. Read More

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http://dx.doi.org/10.1007/s10067-019-04459-8DOI Listing
February 2019

Association of Optic Neuritis with Neuromyelitis Optica Spectrum Disorder and Multiple Sclerosis in Korea.

Korean J Ophthalmol 2019 Feb;33(1):82-90

Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Purpose: To describe the clinical characteristics and course of optic neuritis (ON) and its association with neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) in Korea.

Methods: In this retrospective case series, 125 eyes of 91 Korean patients with ON were included. The medical documents of adult patients with ON were retrospectively reviewed. Read More

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http://dx.doi.org/10.3341/kjo.2018.0050DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6372377PMC
February 2019

Efficacy of BAFF in Monitoring Treatment Response in Early Vietnamese Systemic Sclerosis Patients.

Open Access Maced J Med Sci 2019 Jan 28;7(2):264-268. Epub 2019 Jan 28.

University of Rome G. Marconi, Rome, Italy.

Background: B-cell activating factor (BAFF) is considered to have a role in the pathogenesis of systemic sclerosis (SSc).

Aim: We conducted a longitudinal study on early SSc patients to determine the change in BAFF serum level after treatment and its association with organ involvements.

Methods: A total of 46 patients (32 diffuse, 14 limited) were recruited, among which 35 patients (24 diffuse, 11 limited) completed 12-month follow-up. Read More

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http://dx.doi.org/10.3889/oamjms.2019.070DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364724PMC
January 2019

Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality.

Ann Thorac Med 2019 Jan-Mar;14(1):83-89

Department of Medicine, Division of Rheumatology, King Saud University, Riyadh, Saudi Arabia.

Background: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). The objective of this study is to describe the clinical characteristics, mortality, and predictors of SSc-PAH in Saudi patients.

Methods: Retrospective chart review study of SSc patients who were followed for at least 1 year in three tertiary care centers in Saudi Arabia was conducted. Read More

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http://dx.doi.org/10.4103/atm.ATM_33_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341865PMC
February 2019
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Damage indices in rheumatic diseases: A systematic review of the literature.

Semin Arthritis Rheum 2019 Jan 17. Epub 2019 Jan 17.

The University of Melbourne, St Vincent's Hospital, Melbourne, Australia; St Vincent's Hospital, Melbourne, Australia. Electronic address:

Objective: To review the current literature, and evaluate the psychometric properties of disease damage indices in rheumatic diseases.

Methods: A search of Medline, EMBASE, and Cochrane Library databases was performed to June 2018 to identify damage indices in all systemic rheumatic diseases. Articles were included in a systematic review if indices were composite (multi-organ) in nature and if adequate detail on methodology was described. Read More

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http://dx.doi.org/10.1016/j.semarthrit.2019.01.007DOI Listing
January 2019
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Topical use of mammalian target of rapamycin inhibitors in dermatology: A systematic review with meta-analysis.

J Am Acad Dermatol 2019 Mar;80(3):735-742

Universities of Tours and Nantes, INSERM 1246-SPHERE, Tours, France; Department of Dermatology and Reference Center for Rare Diseases and Vascular Malformations, CHRU Tours, Tours, France.

Background: Systemic mammalian target of rapamycin (mTOR) inhibitors are currently used in many dermatologic indications. Their topical use is recent and poorly codified.

Objective: To provide an overview of the topical use of mTOR inhibitors in dermatologic conditions and evaluate their efficacy and safety. Read More

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http://dx.doi.org/10.1016/j.jaad.2018.10.070DOI Listing

The -P1104A Autoimmune Protective Variant Limits Coordinate Signals Required to Generate Specialized T Cell Subsets.

Front Immunol 2019 25;10:44. Epub 2019 Jan 25.

Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Seattle, WA, United States.

TYK2 is a JAK family member that functions downstream of multiple cytokine receptors. Genome wide association studies have linked a SNP (rs34536443) within encoding a Proline to Alanine substitution at amino acid 1104, to protection from multiple autoimmune diseases including systemic lupus erythematosus (SLE) and multiple sclerosis (MS). The protective role of this SNP in autoimmune pathogenesis, however, remains incompletely understood. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00044DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6355696PMC
January 2019
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A potential contribution of decreased galectin-7 expression in stratified epithelia to the development of cutaneous and esophageal manifestations in systemic sclerosis.

Exp Dermatol 2019 Feb 9. Epub 2019 Feb 9.

Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, 113-8655, Japan.

Backgrounds: Stratified epithelia have caught much attention as potential contributors to the development of dermal and esophageal fibrosis in systemic sclerosis (SSc). Galectin-7 is a marker of all types of stratified epithelia, which is involved in the maintenance of epidermal homeostasis. So far, the role of galectin-7 has not been studied in SSc. Read More

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http://dx.doi.org/10.1111/exd.13900DOI Listing
February 2019
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Is retinal microvascular damage an early manifestation of systemic sclerosis?

Ann Rheum Dis 2019 Feb 9. Epub 2019 Feb 9.

School of Medicine, University of Bari, Bari, Italy.

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http://dx.doi.org/10.1136/annrheumdis-2019-215127DOI Listing
February 2019
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Implication of oxidative stress in the pathogenesis of systematic sclerosis via inflammation, autoimmunity and fibrosis.

Redox Biol 2019 Jan 25:101122. Epub 2019 Jan 25.

Inserm, U1016, Institut Cochin, Paris, France; Cnrs, UMR8104, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, France; Department of Immunology, Cochin Teaching Hospital, AP-HP, 27, rue du faubourg Saint-Jacques, F75014, Paris, France.

Systemic sclerosis is an autoimmune disorder characterized by inflammation and a progressive fibrosis affecting the skin and visceral organs. Over the last two decades, it became clear that oxidative stress plays a key role in its pathogenesis. In this review, we highlighted the role of ROS in the various pathological components of systemic sclerosis, namely the inflammatory, the autoimmune and the fibrotic processes. Read More

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http://dx.doi.org/10.1016/j.redox.2019.101122DOI Listing
January 2019

Reply to 'Moyamoya vasculopathy with anti-SCL-70 antibodies: A case report and review of the literature'.

J Clin Neurosci 2019 Feb 4. Epub 2019 Feb 4.

Goethe University, Division of Rheumatology, University Hospital Frankfurt, Frankfurt am Main, Germany.

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http://dx.doi.org/10.1016/j.jocn.2019.01.043DOI Listing
February 2019

Abnormal pulmonary artery systolic pressure response after exercise in systemic sclerosis patients: A PRISMA-compliant meta-analysis.

Medicine (Baltimore) 2019 Feb;98(6):e14342

Department of Respiratory Medicine, The Second XiangYa hospital, Central South University.

Background: Pulmonary artery systolic pressure (PASP) is an important parameter for detecting pulmonary arterial hypertension (PAH). The difference between rest PASP and post-exercise PASP (ΔPASP) may play a role in predicting and screening resting PAH. The aim of this study is to analyze ΔPASP in systemic sclerosis (SSc) patients with PAH or non-PAH and suggest a cutoff value of ΔPASP for detection of PAH. Read More

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http://dx.doi.org/10.1097/MD.0000000000014342DOI Listing
February 2019
1 Read
5.723 Impact Factor

Five-year outcome in the copaxone observatory: a nationwide cohort of patients with multiple sclerosis starting treatment with glatiramer acetate in France.

J Neurol 2019 Feb 7. Epub 2019 Feb 7.

Teva Santé, La Défense, France.

The benefits provided by disease-modifying treatments in multiple sclerosis have been demonstrated in clinical trials, but the extent to which they can be extrapolated to everyday care is less clear, as are the long-term benefits of treatment. The objective of this prospective observational cohort study performed in France was to evaluate the effectiveness and safety of glatiramer acetate in patients with relapsing-remitting multiple sclerosis over a 5-year period. All neurologists in France were invited to participate and enroll adult patients starting a first treatment with brand glatiramer acetate 20 mg. Read More

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http://dx.doi.org/10.1007/s00415-019-09211-5DOI Listing
February 2019

A low perfusion-metabolic mismatch in Tl and I-BMIPP scintigraphy predicts poor prognosis in systemic sclerosis patients with asymptomatic cardiac involvement.

Int J Rheum Dis 2019 Feb 6. Epub 2019 Feb 6.

Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan.

Aim: This study investigated the prognostic factors of cardiac death or cardiac failure using cardiac scintigraphy, echocardiography (UCG), and magnetic resonance imaging (MRI) in asymptomatic systemic sclerosis (SSc) patients.

Methods: We retrospectively evaluated SSc patients who had undergone cardiac scintigraphy using thallium ( Tl) and I-β-methyl-P-iodophenyl-pentadecanoic acid ( I-BMIPP), UCG, and cardiac MRI. We calculated the mismatch score in scintigraphy by subtracting the uptake of I-BMIPP from that of Tl. Read More

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http://dx.doi.org/10.1111/1756-185X.13508DOI Listing
February 2019
2 Reads