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    [RHEOLOGICAL PROPERTIES OF BLOOD SERUM AT SYSTEMIC SCLEROSIS].
    Georgian Med News 2018 Mar(276):92-97
    Donetsk National Medical University, Lyman, Ukraine.
    Systemic sclerosis (SSc) is accompanied by severe disorders of serum component of blood rheological properties (BRP), the study indicators of which performed as the monitoring of the therapeutic interventions effectiveness. BRP changes have not been established at different forms of SSc, the connection between physicochemical serum rheological properties and morphological signs of vascular skin lesions and extracardiac implications of the disease remains unknown. The purpose and objectives of the study - to evaluate the changes of BRP serum component with clinical and morphological variants of limited and diffuse form of SSc. Read More

    Training and support needs of scleroderma support group facilitators: the North American Scleroderma Support Group Facilitators Survey.
    Disabil Rehabil 2018 Apr 26:1-6. Epub 2018 Apr 26.
    a Jewish General Hospital , Lady Davis Institute for Medical Research , Montréal , Canada.
    Purpose: Peer-facilitated support groups are an important resource for people with scleroderma, but little is known about challenges faced by support group facilitators. The objective was to identify training and support needs of scleroderma support group facilitators to inform the development of an educational training program.

    Methods: A 32-item survey assessed confidence of support group facilitators to execute tasks necessary for successfully facilitating support groups. Read More

    Comparison of individually tailored versus fixed-schedule rituximab regimen to maintain ANCA-associated vasculitis remission: results of a multicentre, randomised controlled, phase III trial (MAINRITSAN2).
    Ann Rheum Dis 2018 Apr 25. Epub 2018 Apr 25.
    Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases: Vasculitis and Scleroderma, Cochin Hospital, Paris Descartes University, Paris, France.
    Objective: To compare individually tailored, based on trimestrial biological parameter monitoring, to fixed-schedule rituximab reinfusion for remission maintenance of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAVs).

    Methods: Patients with newly diagnosed or relapsing granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) in complete remission after induction therapy were included in an open-label, multicentre, randomised controlled trial. All tailored-arm patients received a 500 mg rituximab infusion at randomisation, with rituximab reinfusion only when CD19+B lymphocytes or ANCA had reappeared or ANCA titre rose markedly based on trimestrial testing until month 18. Read More

    NADPH oxidase, oxidative stress and fibrosis in Systemic Sclerosis.
    Free Radic Biol Med 2018 Apr 22. Epub 2018 Apr 22.
    Dipartimento di Scienze Cliniche e Molecolari, Clinica Medica, Università Politecnica delle Marche. Electronic address:
    Systemic sclerosis (SSc) is an autoimmune disease characterized by damage of small vessels, immune abnormalities and exaggerated production of extracellular matrix. The etiology of the disease is unknown and the pathogenesis ill defined. However, there is consistent evidence that oxidative stress contributes to the establishment and progression of the disease. Read More

    Isoprostane in systemic sclerosis: a systematic review and meta-analysis.
    Mod Rheumatol 2018 Apr 25:1-14. Epub 2018 Apr 25.
    d Department of Clinical Biochemistry , Urmia University of Medical Sciences , West Azerbaijan Province 24 , Urmia , Iran.
    Objectives: to further the knowledge of oxidative stress in systemic sclerosis (SSc) we performed a systematic review and meta-analysis on studies measuring isoprostane, a vasoactive agent deriving from arachidonic acid and implicated in the vasculopathy of SSc.

    Methods: systematic search following the PRISMA guidelines in PubMed and EMBASE between January-1990/December-2017 using the terms: oxidative stress, isoprostane, systemic sclerosis, scleroderma.

    Results: After the screening process 8 studies including 240 SSc patients and 192 controls were included in the systematic review and meta-analysis, 6 investigating urinary and 2 serum isoprostane: random effect meta-analysis revealed isoprostane overgeneration in SSc (p < 0. Read More

    The Convergence of Vasculopathy and Vasculitis: Computer Mapping Analysis of 2 Renal Biopsies in a Patient with both Systemic Sclerosis and ANCA-Related Vasculitis.
    Case Rep Nephrol Dial 2018 Jan-Apr;8(1):35-44. Epub 2018 Mar 2.
    Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA.
    Scleroderma vasculopathy and ANCA (antineutrophil cytoplasmic antibodies)-associated glomerulonephritis have rarely been reported to occur simultaneously in one patient. Herein, we report a patient who presented with a classic constellation of clinical and laboratory findings of systemic scleroderma and was subsequently found to be positive for p-ANCA. Two renal biopsies, performed 5 months apart, demonstrated typical changes of the two entities in both acute and "healed" phases, which were analyzed by computer mapping techniques. Read More

    Gauzoma in A Scleroderma Patient following Open Heart Surgery: A Case Report.
    Curr Rheumatol Rev 2018 Apr 22. Epub 2018 Apr 22.
    Shiraz University of Medical Sciences - Shiraz Geriatric Research Center Shiraz. Iran.
    Introduction: Gauzoma is an iatrogenic complication, which occurs rarely due to surgical team negligence. Depending on the sterility of the retained tissue, it can lead to life threatening surgical complications or may remain asymptomatic for many years and be detected incidentally in imaging studies. It may be mistaken as tumors or aneurysms. Read More

    JUVENILE SCLERODERMA-what has changed in the meantime?
    Curr Rheumatol Rev 2018 Apr 22. Epub 2018 Apr 22.
    Istanbul University, Cerrahpasa Medical School - Department of Pediatric Rheumatology Istanbul. Turkey.
    Background: Juvenile scleroderma is a rarely seen chronic connective tissue disorder characterized by stiffening of the skin. The frequency of the disease was reported as one per million. According to organ involvement, the disease is divided into two main forms: systemic and localized scleroderma. Read More

    Neuropathic pain: is it an underestimated symptom in systemic sclerosis?
    Clin Rheumatol 2018 Apr 24. Epub 2018 Apr 24.
    Department of Biostatistics, Cukurova University School of Medicine, Adana, Turkey.
    Pain is one of the most common symptoms in systemic sclerosis (SSc) patients, yet not considered in the assessment of disease severity. This study aimed to investigate the frequency of neuropathic pain (NP) and to evaluate its interference with the quality of life (QoL) in SSc patients. Diffuse and Limited SSc patients diagnosed by American College of Rheumatology 2013 criteria were included in the study. Read More

    Increased serum levels of adhesion molecules ICAM-1 and VCAM-1 in systemic sclerosis are not specific for pulmonary manifestations.
    Clin Rheumatol 2018 Apr 23. Epub 2018 Apr 23.
    Department of Rheumatology, St Vincent's Hospital (Melbourne), 41 Parade, Fitzroy, VIC, 3065, Australia.
    Studies suggest elevated serum intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) levels may be markers of pulmonary arterial hypertension in systemic sclerosis (SSc-PAH). We sought to evaluate whether ICAM-1 and VCAM-1 levels are useful screening biomarkers for incident SSc-PAH. In this cross-sectional study, four groups were selected from the Australian Scleroderma Cohort Study: group 1 (n = 15) had definite PAH; group 2 (n = 19) had interstitial lung disease (ILD); group 3 (n = 30) were SSc-controls; and group 4 (n = 34) were healthy controls. Read More

    Clinical pattern of systemic sclerosis in Central Ukraine. Association between clinical manifestations of systemic sclerosis and hypertension.
    Reumatologia 2018 28;56(1):24-30. Epub 2018 Feb 28.
    Rheumatology Department, Municipal institution "Mechnikov Dnipropetrovsk regional clinic", Dnipro, Ukraine.
    Objectives: Systemic sclerosis (SSc) is a rare disease of connective tissue, manifestations of which may vary in different geographical areas. We aimed to describe the clinical portrait of patients with SSc in Dnipropetrovsk region and to investigate how initial clinical and laboratory characteristics are connected with the presence of hypertension in SSc onset.

    Material And Methods: Patients were enrolled to this study from the registry of SSc patients, established in the Rheumatology Department, Mechnikov Dnipropetrovsk Regional Clinic, Dnipro. Read More

    Patient acceptable symptom state and minimal clinically important difference for patient-reported outcomes in systemic sclerosis: A secondary analysis of a randomized controlled trial comparing personalized physical therapy to usual care.
    Semin Arthritis Rheum 2018 Mar 27. Epub 2018 Mar 27.
    AP-HP, Service de Rééducation et de Réadaptation de l'Appareil Locomoteur et des Pathologies du Rachis, Hôpitaux Universitaires Paris Centre-Groupe Hospitalier Cochin, Paris, France; Sorbonne Paris Cité, Université Paris Descartes, Faculté de Médecine, Paris, France; INSERM UMR 1124, Laboratoire de Pharmacologie, Toxicologie et Signalisation Cellulaire, Faculté des Sciences Fondamentales et Biomédicales, Centre Universitaire des Saints-Pères, Paris, France. Electronic address:
    Background: To estimate patient acceptable symptom state (PASS) and minimal clinically important difference (MCID) for patient-reported outcomes in systemic sclerosis (SSc).

    Methods: We conducted a secondary analysis of the SCLEREDUC trial, a 12-month randomized controlled trial comparing the efficacy of physical therapy to usual care in 220 SSc patients followed-up from September 2005 to October 2010. Self-rated state and change in patient health at 12 months were assessed by using 2 external anchors extracted from the Medical Outcomes Study 36-Item Short-Form. Read More

    Progress in understanding the pathophysiology of multiple sclerosis.
    Rev Neurol (Paris) 2018 Apr 18. Epub 2018 Apr 18.
    Pôle des Neurosciences et de l'Appareil Locomoteur, CHRU de Lille, LIRIC, U995, équipe 3, Université de Lille, 59037 Lille Cedex, France. Electronic address:
    Multiple sclerosis (MS) arises in people who have a genetic susceptibility to environmental factors and events, which ultimately trigger the disease. It is thought that peripheral immune cells are mobilized and enter the CNS through the impaired blood-brain barrier in the subarachnoid space, as acute lesions show large numbers of macrophages and CD8+ T cells and, to a lesser extent, CD4+ T cells, B cells and plasma cells. Demyelination is mostly localized to focal lesions in early relapsing-remitting (RR) MS, whereas other areas of white matter appear normal. Read More

    Autoantibodies and scleroderma phenotype define subgroups at high-risk and low-risk for cancer.
    Ann Rheum Dis 2018 Apr 20. Epub 2018 Apr 20.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
    Objectives: Recent studies demonstrate autoantibodies are powerful tools to interrogate molecular events linking cancer and the development of autoimmunity in scleroderma. Investigating cancer risk in these biologically relevant subsets may provide an opportunity to develop personalised cancer screening guidelines. In this study, we examined cancer risk in distinct serologic and phenotypic scleroderma subsets and compared estimates with the general population. Read More

    Differences in clinical presentation and incidence of cardiopulmonary involvement in late-onset versus early-onset systemic sclerosis: inception cohort study.
    Int J Rheum Dis 2018 Apr 19. Epub 2018 Apr 19.
    Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
    Introduction: Data regarding the incidence rate (IR) of cardiopulmonary involvement in comparison between late-onset SSc and early-onset SSc are limited.

    Objective: To compare the prevalence of clinical manifestations and the IR of cardiopulmonary involvement compared between the two subgroups.

    Methods: An inception cohort of SSc patients seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, between January 2010 and June 2016, was used. Read More

    Systemic sclerosis and malignancy.
    Int J Rheum Dis 2018 Apr 19. Epub 2018 Apr 19.
    Department of Rheumatology, Adnan Menderes University, Aydin, Turkey.
    Background: Systemic sclerosis (SSc) has increased risk of morbidity and mortality due to pulmonary fibrosis, pulmonary arterial hypertension, renal crisis, infections and malignancies. Chemical exposure, smoking and cytotoxic drugs increase the malignancy risk in rheumatic diseases including SSc. We aim to evaluate characteristics, identify risk factors and mortality in SSc patients with malignancies. Read More

    Musculoskeletal Manifestations of Non-RA Connective Tissue Diseases: Scleroderma, Systemic Lupus Erythematosus, Still's Disease, Dermatomyositis/Polymyositis, Sjögren's Syndrome, and Mixed Connective Tissue Disease.
    Semin Musculoskelet Radiol 2018 Apr 19;22(2):166-179. Epub 2018 Apr 19.
    Department of Musculoskeletal Radiology, University Hospital of Lille, Lille Cedex, France.
    The most common systemic rheumatologic conditions are connective tissue diseases (including rheumatoid arthritis [RA]) followed by spondyloarthropathy. With the advent of biotherapies and imaging biomarkers, development in the imaging of RA and spondyloarthropathies has received substantial attention in the literature. This article details the various musculoskeletal imaging features of the other connective tissue diseases such as scleroderma and progressive systemic sclerosis, systemic lupus erythematosus, Still's disease, dermatomyositis and polymyositis, Sjögren's syndrome, and mixed connective tissue disease. Read More

    Imaging Features of the Juvenile Inflammatory Arthropathies.
    Semin Musculoskelet Radiol 2018 Apr 19;22(2):147-165. Epub 2018 Apr 19.
    Department of Paediatric Imaging, Hospital Jeanne de Flandre, CHU Lille, France.
    We discuss the imaging of several juvenile inflammatory arthropathies including juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, juvenile scleroderma, juvenile dermatomyositis, and chronic recurrent multifocal osteomyelitis. Juvenile idiopathic arthritis is the most common autoimmune chronic systemic disease of connective tissue in children. The remaining systemic juvenile connective tissue diseases are rare. Read More

    Characterization of inflammatory cell infiltrate of scleroderma skin: B cells and skin score progression.
    Arthritis Res Ther 2018 Apr 18;20(1):75. Epub 2018 Apr 18.
    Unità Operativa Complessa di Reumatologia, Istituto di Reumatologia e Scienze Affini, Università Cattolica del Sacro Cuore, Rome, Italy.
    Background: The purpose of this study was to investigate the frequency and the distribution of inflammatory cell infiltrate in two sets of cutaneous biopsies derived from clinically affected and unaffected skin in patients with systemic sclerosis (SSc) and to test correlation between the cell infiltrate and the progression of skin involvement.

    Methods: Skin was immunohistochemically assessed to identify CD68, CD3, CD20 and CD138-positive (+) cells in clinically affected and unaffected skin in 28 patients with SSc. Patients were followed for 6 months and the characteristics of the infiltrate were analyzed according to disease duration, clinical features and skin involvement progression. Read More

    From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution.
    Dermatol Res Pract 2018 30;2018:1284687. Epub 2018 Jan 30.
    Scleroderma Unit, Chair of Rheumatology, University of Modena and Reggio Emilia, Modena, Italy.
    Background: Systemic sclerosis (SSc) and localized scleroderma (LoS) are two different diseases that may share some features. We evaluated the relationship between SSc and LoS in our case series of SSc patients.

    Methods: We analysed the clinical records of 330 SSc patients, in order to find the eventual occurrence of both the two diseases. Read More

    Oxidative stress in the pathogenesis of systemic scleroderma: An overview.
    J Cell Mol Med 2018 Apr 17. Epub 2018 Apr 17.
    Center for Gender-Specific Medicine, Biomarkers Unit, Rome, Italy.
    Systemic sclerosis (SSc) is a rare disorder of the connective tissue characterized by fibrosis of the skin, skeletal muscles and visceral organs. Additional manifestations include activation of the immune system and vascular injury. SSc causes disability and death as the result of end-stage organ failure. Read More

    Evaluation of membrane-bound and soluble forms of HLA-G in Systemic Sclerosis.
    Clin Exp Immunol 2018 Apr 16. Epub 2018 Apr 16.
    Department of Internal Medicine, Clinical Immunology Unit, University of Genoa and Ospedale Policlinico San Martino, Genoa (Italy).
    Systemic sclerosis (SSc) is a complex disease characterized by immune dysregulation, extensive vascular damage and wide-spread fibrosis. Human leukocyte antigen-G (HLA-G) is a non-classic class I major histocompatibility complex (MHC) molecule characterized by complex immuno-modulating properties. HLA-G is expressed on the membrane of different cell lineages in both physiological and pathological conditions. Read More

    Venous Thromboembolism in Systemic Sclerosis: Prevalence, Risk Factors, and Effect on Survival.
    J Rheumatol 2018 Apr 15. Epub 2018 Apr 15.
    From the Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital; Division of Rheumatology, Department of Medicine, University of Toronto; Institute of Health Policy, Management and Evaluation; Department of Anesthesia and Institute of Health Policy, Management and Evaluation, University of Toronto; Department of Anesthesia and Pain Management, Toronto General Hospital, Toronto, Ontario, Canada; King Fahad Central Hospital, Ministry of Health, Jizan, Saudi Arabia. Dr. Johnson is supported by a Canadian Institutes of Health Research Clinician Scientist Award, the Oscar and Eleanor Markovitz Fund for Scleroderma Research, and the Freda Fejer Fund of the Arthritis Research Foundation. Dr. Wijeysundera is supported in part by a New Investigator Award from the Canadian Institutes of Health Research and a Merit Award from the Department of Anesthesia at the University of Toronto. S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, University of Toronto, and Institute of Health Policy, Management and Evaluation, University of Toronto; N. Hakami, MD, Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, University of Toronto, and King Fahad Central Hospital, Ministry of Health; Z. Ahmad, MD, Toronto Scleroderma Program, Mount Sinai Hospital, Division of Rheumatology, Department of Medicine, University of Toronto; D.N. Wijeysundera, MD, PhD, Department of Anesthesia and Pain Management, Toronto General Hospital, and Department of Anesthesia and Institute of Health Policy, Management and Evaluation, University of Toronto. Address correspondence to Dr. S. Johnson, Division of Rheumatology, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst St., Toronto, Ontario M5T 2S8, Canada. E-mail: Accepted for publication December 14, 2017.
    Objective: Whether systemic sclerosis (SSc) confers increased risk of venous thromboembolism (VTE) is uncertain. We evaluated the prevalence, risk factors, and effect of VTE on SSc survival.

    Methods: A cohort study was conducted of subjects with SSc who fulfilled the American College of Rheumatology/European League Against Rheumatism classification criteria between 1970 and 2017. Read More

    Phosphorylated TDP-43 (pTDP-43) aggregates in the axial skeletal muscle of patients with sporadic and familial amyotrophic lateral sclerosis.
    Acta Neuropathol Commun 2018 Apr 13;6(1):28. Epub 2018 Apr 13.
    Institute of Academic Medicine (IAM) in the Houston Methodist Research Institute (HMRI), Houston Methodist Hospital, 6565 Fannin Street, Houston, TX, 77030, USA.
    Muscle atrophy with weakness is a core feature of amyotrophic lateral sclerosis (ALS) that has long been attributed to motor neuron loss alone. However, several studies in ALS patients, and more so in animal models, have challenged this assumption with the latter providing direct evidence that muscle can play an active role in the disease. Here, we examined the possible role of cell autonomous pathology in 148 skeletal muscle samples from 57 ALS patients, identifying phosphorylated TAR DNA-binding protein (pTDP-43) inclusions in the muscle fibers of 19 patients (33. Read More

    Prediction of organ involvement in systemic sclerosis by serum biomarkers and peripheral endothelial function.
    Clin Exp Rheumatol 2018 Apr 12. Epub 2018 Apr 12.
    Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
    Objectives: To identify prognostic factors among serum biomarkers and endothelial vasodilator function findings in patients with systemic sclerosis (SSc).

    Methods: This is a clinical observational study. We assessed 60 consecutive SSc patients (44 limited cutaneous-type, 16 diffuse cutaneous-type). Read More

    Articular Surgery of the Ischemic Hand in Systemic Scleroderma: A Vascular Basis for Arthrodesis and Arthroplasty.
    J Hand Surg Am 2018 Apr 9. Epub 2018 Apr 9.
    Division of Plastic and Reconstructive Surgery, Brigham & Women's Hospital/Harvard Medical School, Boston, MA.
    Hallmark deformities of systemic scleroderma are early onset and progressively disabling flexion contractures of the proximal interphalangeal (PIP) joints often in conjunction with extension or, less frequently, flexion contractures of the metacarpophalangeal (MCP) joints. Although surgical correction is generally recommended, a prevailing reluctance for operative treatment exists owing to the inherent ischemia of the disease with its potentially compromised healing capacity. Nonetheless, with recognition and preservation of the tenuous but well-defined and constant periarticular vascular networks of the PIP and MCP joints, articular reconstruction with uncomplicated wound healing can prove consistently successful for patients with scleroderma. Read More

    Diffuse dermatophytosis occurring on dimethyl fumarate therapy.
    Mult Scler 2018 Apr 1:1352458517741207. Epub 2018 Apr 1.
    Multiple Sclerosis Research Institute, Philadelphia, PA, USA.
    Background: Opportunistic infections have occurred during dimethyl fumarate (DMF) therapy.

    Objective: Diffuse skin dermatophytosis which occurred during DMF therapy in the setting of lymphopenia is described.

    Methods And Results: The clinical course, the lymphocyte subset profile, and dermatologic evaluations were reviewed. Read More

    Oesophageal diameter is associated with severity but not progression of systemic sclerosis-associated interstitial lung disease.
    Respirology 2018 Apr 11. Epub 2018 Apr 11.
    Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
    Background And Objective: It is unknown whether oesophageal disease is associated with systemic sclerosis-associated interstitial lung disease (SSc-ILD) severity, progression or mortality.

    Methods: High-resolution computed tomography (HRCT) scans from 145 SSc-ILD patients were scored for fibrosis score, oesophageal diameter and presence of hiatal hernia. Fibrosis asymmetry was calculated as: (most affected side - least affected side)/(most affected side + least affected side). Read More

    Efficacy and safety of rituximab in systemic sclerosis: French retrospective study and literature review.
    Autoimmun Rev 2018 Apr 7. Epub 2018 Apr 7.
    Department of Internal Medicine, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique - Hôpitaux de Paris, Hôpital Saint-Antoine, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Sorbonne Universités, UMPC University Paris 06, INSERM U938, Centre de Recherche Saint-Antoine (CRSA), Paris, France. Electronic address:
    Objective: To describe safety and efficacy of rituximab in patients with systemic sclerosis.

    Methods: We included 13 patients with systemic sclerosis treated with rituximab and pooled with 40 additional patients from the literature. SSc rituximab untreated patients were matched to rituximab treated ones. Read More

    Treatment of Genetic Forms of Nephrotic Syndrome.
    Front Pediatr 2018 26;6:72. Epub 2018 Mar 26.
    Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany.
    Idiopathic steroid-resistant nephrotic syndrome (SRNS) is most frequently characterized by focal segmental glomerulosclerosis (FSGS) but also other histological lesions, such as diffuse mesangial sclerosis. In the past two decades, a multitude of genetic causes of SRNS have been discovered raising the question of effective treatment in this cohort. Although no controlled studies are available, this review will discuss treatment options including pharmacologic interventions aiming at the attenuation of proteinuria in genetic causes of SRNS, such as inhibitors of the renin-angiotensin-aldosterone system and indomethacin. Read More

    Evaluation of Microglial Activation in Multiple Sclerosis Patients Using Positron Emission Tomography.
    Front Neurol 2018 26;9:181. Epub 2018 Mar 26.
    Division of Clinical Neurosciences, Turku University Hospital and University of Turku, Turku, Finland.
    Understanding the mechanisms underlying progression in multiple sclerosis (MS) is one of the key elements contributing to the identification of appropriate therapeutic targets for this under-managed condition. In addition to plaque-related focal inflammatory pathology typical for relapsing remitting MS there are, in progressive MS, widespread diffuse alterations in brain areas outside the focal lesions. This diffuse pathology is tightly related to microglial activation and is co-localized with signs of neurodegeneration. Read More

    Characterizing Microstructural Tissue Properties in Multiple Sclerosis with Diffusion MRI at 7 T and 3 T: The Impact of the Experimental Design.
    Neuroscience 2018 Apr 7. Epub 2018 Apr 7.
    Dept. of Cognitive Neuroscience, Faculty of Psychology & Neuroscience, Maastricht University, Maastricht, Netherlands.
    The recent introduction of advanced magnetic resonance (MR) imaging techniques to characterize focal and global degeneration in multiple sclerosis (MS), like the Composite Hindered and Restricted Model of Diffusion, or CHARMED, diffusional kurtosis imaging (DKI) and Neurite Orientation Dispersion and Density Imaging (NODDI) made available new tools to image axonal pathology non-invasively in vivo. These methods already showed greater sensitivity and specificity compared to conventional diffusion tensor-based metrics (e.g. Read More

    Lung transplantation for scleroderma lung disease: An international, multicenter, observational cohort study.
    J Heart Lung Transplant 2018 Mar 15. Epub 2018 Mar 15.
    Service de Chirurgie Thoracique, Vasculaire et Transplantation Cardio-pulmonaire, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France; Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France; UMR_S 999, Université Paris-Sud, INSERM, Hôpital Marie Lannelongue, Le Plessis Robinson, France. Electronic address:
    Background: Due to its multisystemic nature, scleroderma is considered a relative contraindication to lung transplantation at many centers. However, recent studies suggest similar post-transplant outcomes in patients with scleroderma compared to those with other causes of interstitial lung disease (ILD). Furthermore, it remains unknown whether scleroderma-associated pulmonary arterial hypertension (PAH) influences post-transplant outcomes. Read More

    Autoantibodies in Serum of Systemic Scleroderma Patients: Peptide-Based Epitope Mapping Indicates Increased Binding to Cytoplasmic Domains of CXCR3.
    Front Immunol 2018 22;9:428. Epub 2018 Mar 22.
    Department of Rheumatology, University of Lübeck, Lübeck, Germany.
    Systemic sclerosis (SSc) is a severe chronic autoimmune disease with high morbidity and mortality. Sera of patients with SSc contain a large variety of autoantibody (aab) reactivities. Among these are functionally active aab that bind to G protein-coupled receptors (GPCR) such as C-X-C motif chemokine receptor 3 (CXCR3) and 4 (CXCR4). Read More

    Applied Bayesian Methods in the Rheumatic Diseases.
    Rheum Dis Clin North Am 2018 May;44(2):361-370
    Institute of Health Policy, Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada; Division of Rheumatology, Department of Paediatrics, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.
    The use of applied Bayesian methods is increasing in rheumatology. Using the Bayes theorem, past evidence is updated with new data. Preexisting data are expressed as a prior probability distribution or prior. Read More

    Propensity Score Methods for Bias Reduction in Observational Studies of Treatment Effect.
    Rheum Dis Clin North Am 2018 May;44(2):203-213
    Institute of Health Policy, Management and Evaluation, University of Toronto, 155 College Street, Toronto, Ontario M5T 3M6, Canada; Dalla Lana School of Public Health, University of Toronto, 155 College Street, Toronto, Ontario M5T 3M7, Canada; Division of Rheumatology, Department of Paediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.
    A challenge to the use of observational data to study treatment effects is the issue of confounding. Noncomparability of exposed and nonexposed subjects can lead to biased estimation of the treatment effect. The propensity score is a balancing score that can be used to form matched groups, or pairs, that are not systematically different and enable nonbiased comparisons between groups. Read More

    Does nailfold capillaroscopy help predict future outcomes in systemic sclerosis? A systematic literature review.
    Semin Arthritis Rheum 2018 Feb 14. Epub 2018 Feb 14.
    Department of Pharmacy and Pharmacology, University of Bath, Bath, UK; Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Upper Borough Walls, Bath, Bath BA1 1RL, UK. Electronic address:
    Background: Nailfold capillaroscopy (NC) is an important diagnostic tool in systemic sclerosis (SSc). Confirmation of NC as a prognostic factor could facilitate earlier intervention and slow disease progression in SSc. We undertook a systematic literature review to evaluate the prognostic value of NC in predicting SSc disease progression. Read More

    Different profile of cytokine production in patients with systemic sclerosis and association with clinical manifestations.
    Immunol Lett 2018 Mar 27;198:12-16. Epub 2018 Mar 27.
    Laboratório de Imunomodulação e Novas Abordagens Terapêuticas - Núcleo de Pesquisa em Inovação Terapêutica (LINAT-NUPIT) - UFPE, Av. Prof. Moraes Rego, 1235, Cidade Universitária, Recife, PE, CEP: 50670-901, Brazil.
    Immune dysregulation is a central process in the pathogenesis of systemic sclerosis (SSc). Cytokines produced by lymphocytes and monocytes are important mediators and induce tissue damage, recruit additional inflammatory cells, and promote extracellular matrix production and fibrosis. In the present research, we aimed to study the associations between levels of cytokines in serum and culture supernatants from peripheral blood mononuclear cells (PBMCs) and clinical manifestations in SSc patients. Read More

    Serum level of endostatin and digital ulcers in systemic sclerosis patients.
    Int Wound J 2018 Mar 30. Epub 2018 Mar 30.
    Department of Clinical Medicine, Sapienza University of Rome, Rome, Italy.
    Patients with systemic sclerosis (SSc) are at a high risk of the development of ischaemic digital ulcers (DUs) that can be complicated with infections, gangrene, and osteomyelitis. The aim of this study is to evaluate the role of endostatin in scleroderma DUs.In total, 90 SSc patients were enrolled in this study. Read More

    The Influence of Connective Tissue Disease in Breast Reconstruction: A National Database Analysis.
    Ann Plast Surg 2018 Apr;80(4 Suppl 4):S182-S188
    From the Division of Plastic and Reconstructive Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.
    Background: Patients with connective tissue diseases (CTD), or collagen vascular diseases, are at risk of potentially higher morbidity after surgical procedures. We aimed to investigate the complication profile in CTD versus non-CTD patients who underwent breast reconstruction on a national scale.

    Methods: A retrospective analysis of the Healthcare Cost and Utilization Project NIS Database between 2010 and 2014 was conducted for patients 18 years or older admitted for immediate autologous or implant breast reconstruction. Read More

    Soluble klotho as a marker of renal fibrosis and podocyte injuries in human kidneys.
    PLoS One 2018 28;13(3):e0194617. Epub 2018 Mar 28.
    Department of Internal Medicine, Soonchunhyang University Cheonan Hospital, Cheonan, Korea.
    Klotho deficiency is relevant to renal fibrosis and podocyte injury in vivo and in vitro. We examined whether histological findings of renal biopsy specimens were associated with the levels of soluble klotho in humans. We investigated renal biopsy specimens of 67 patients and detailed microscopic findings were reviewed. Read More

    Improving life expectancy of patients with scleroderma. Results from the South Australian Scleroderma Register.
    Intern Med J 2018 Mar 23. Epub 2018 Mar 23.
    Department of Immunology, Flinders Medical Centre, Adelaide, Australia.
    Background: Scleroderma is a rare connective tissue disorder characterised by inflammation, vasculopathy and excessive fibrosis. Patients with scleroderma are known to have decreased life expectancy.

    Aim: To investigate changes in life expectancy in patients with scleroderma over a 30 year period. Read More

    Antibodies against chemokine receptors CXCR3 and CXCR4 predict progressive deterioration of lung function in patients with systemic sclerosis.
    Arthritis Res Ther 2018 Mar 22;20(1):52. Epub 2018 Mar 22.
    Department of Rheumatology and Clinical Immunology, Charité University Hospital, Berlin, Germany.
    Background: The chemokine receptors CXCR3 and CXCR4 are involved in the pathogenesis of fibrosis, a key feature of systemic sclerosis (SSc). It is hypothesized that immunoglobulin (Ig)G antibodies (abs) against these two receptors are present in patients with SSc and are associated with clinical findings.

    Methods: Anti-CXCR3 and anti-CXCR4 ab levels were measured in 449 sera from 327 SSc patients and in 234 sera from healthy donors (HD) by enzyme-linked immunosorbent assay (ELISA). Read More

    Effect of 6-min Walk Test on pro-BNP Levels in Patients with Pulmonary Arterial Hypertension.
    Lung 2018 Mar 21. Epub 2018 Mar 21.
    Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of North Carolina, Chapel Hill, USA.
    Background: Plasma pro-BNP (brain natriuretic peptide) levels are often elevated in response to right ventricular (RV) volume and pressure overload, parameters potentially affected by exercise. Plasma pro-BNP levels change in association with long-term changes in pulmonary hemodynamics, thereby serving as a potential biomarker in pulmonary arterial hypertension (PAH). The 6-min Walk Test (6MWT) and pro-BNP level are often checked in a single office visit. Read More

    Scleroderma-related choroiditis.
    Oman J Ophthalmol 2018 Jan-Apr;11(1):75-77
    Department of Uvea and Ocular Pathology, Sankara Nethralaya, Chennai, Tamil Nadu, India.
    A 34-year-old female a known scleroderma patient presented to us with ocular manifestations in the form choroiditis along with optic nerve involvement. Blood investigations were done, and the ocular findings were confirmed by fundus fluorescein angiography. She was successfully managed with systemic steroid therapy. Read More

    Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis.
    Eur Respir J 2018 Apr 4;51(4). Epub 2018 Apr 4.
    Centre for Pulmonary Hypertension, Thorax Clinic at the University Hospital Heidelberg, Heidelberg, Germany.
    The objective of this study was to evaluate the incidence of pulmonary hypertension (PH) and determining factors in patients with systemic sclerosis (SSc) and a diffusing capacity of the lung for carbon monoxide () <60% predicted.In this bicentric, prospective cohort study, patients with SSc were clinically assessed at baseline and after 3 years, including right heart catheterisation (RHC). Analysis of determining factors for the development of PH was performed using univariate and multivariate analyses. Read More

    Gene Profiling in Patients with Systemic Sclerosis Reveals the Presence of Oncogenic Gene Signatures.
    Front Immunol 2018 6;9:449. Epub 2018 Mar 6.
    Immunology Area, Pediatric Hospital Bambino Gesù, Rome, Italy.
    Systemic sclerosis (SSc) is a rare connective tissue disease characterized by three pathogenetic hallmarks: vasculopathy, dysregulation of the immune system, and fibrosis. A particular feature of SSc is the increased frequency of some types of malignancies, namely breast, lung, and hematological malignancies. Moreover, SSc may also be a paraneoplastic disease, again indicating a strong link between cancer and scleroderma. Read More

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