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    Epithelial Fli1 deficiency drives systemic autoimmunity and fibrosis: Possible roles in scleroderma.
    J Exp Med 2017 Feb 23. Epub 2017 Feb 23.
    Department of Dermatology, University of Tokyo Graduate School of Medicine, Bunkyo-ku, Tokyo 113-8655, Japan.
    Systemic sclerosis (SSc), or scleroderma, is a multisystem autoimmune disorder characterized by vasculopathy and fibrosis in the skin and internal organs, most frequently in the esophagus and lungs. Hitherto, studies on SSc pathogenesis centered on immune cells, vascular cells, and fibroblasts. Although dysregulated keratinocytes in SSc have been recently reported, the contribution of epithelial cells to pathogenesis remains unexplored. Read More

    Intravenous immunoglobulins in systemic sclerosis: Data from a French nationwide cohort of 46 patients and review of the literature.
    Autoimmun Rev 2017 Feb 13. Epub 2017 Feb 13.
    Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000, Lille, France; INSERM, U995, F-59000, Lille, France; CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000, Lille, France; Centre National de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique), F-59000, Lille, France; Healh Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET). Electronic address:
    Background: As intravenous immunoglobulins (IVIG) exhibit immunomodulatory and antifibrotic properties, they may be a relevant treatment for systemic sclerosis (SSc). The objectives of this work were thus to report on the efficacy and safety of IVIG in a population of SSc patients and to review the available literature.

    Methods: 46 patients from 19 French centers were retrospectively recruited. Read More

    Six-minute walk test in or out in evaluation of systemic sclerosis patients?
    Clin Exp Rheumatol 2017 Feb 15. Epub 2017 Feb 15.
    Department of Rheumatology, Ghent University Hospital; and Department of Internal Medicine, Ghent University, Belgium.
    Objectives: Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the leading causes of death in systemic sclerosis (SSc) patients. Although the six-minute walk test (6MWT) is used for evaluating ILD and PAH in clinical practice, no data are available on six-minute walk distance (6MWD) and oxygen desaturation in SSc patients without ILD and PAH.

    Methods: Prospectively collected data of the 6MWTs at baseline and 6-month follow-up of 300 consecutive SSc patients, included in the Ghent University Hospital Systemic Sclerosis Unit between May 2006 and April 2015 were analysed. Read More

    Changes in the pattern of death of 987 patients with systemic sclerosis from 1990 to 2009 from the nationwide Spanish Scleroderma Registry (RESCLE).
    Clin Exp Rheumatol 2017 Feb 6. Epub 2017 Feb 6.
    Vall d'Hebron University Hospital, Department of Internal Medicine, Barcelona, Spain.
    Objectives: To determine the changes in the pattern of death of patients with systemic sclerosis (SSc) throughout 20 years.

    Methods: Data were collected from the Spanish Scleroderma Registry (RESCLE), retrospective multicentre database from 1990 to 2009. SSc-related and SSc-non related causes of death were assessed. Read More

    Relationship between calcium channel blockers and skin fibrosis in patients with systemic sclerosis.
    Clin Exp Rheumatol 2017 Jan 31. Epub 2017 Jan 31.
    St. Joseph's Healthcare Hamilton, ON; and Division of Rheumatology, Department of Medicine, McMaster University, Hamilton, ON, Canada.
    Objectives: Recent experimental evidence suggests that calcium channel blockers (CCBs) may have anti-fibrotic effects on liver and pulmonary fibrosis. We aimed to investigate whether use of CCBs was associated with the skin fibrosis in patients with systemic sclerosis (SSc).

    Methods: Based on the 5-year follow-up data from the Canadian Scleroderma Research Group registry, we used the generalised estimating equations (GEE) model to assess the relationship between use of CCBs and the primary outcome of skin fibrosis measured by the modified Rodnan skin score (mRSS). Read More

    Patient phenotypes in fibromyalgia comorbid with systemic sclerosis or rheumatoid arthritis: influence of diagnostic and screening tests. Screening with the FiRST questionnaire, diagnosis with the ACR1990 and revised ACR 2010 criteria.
    Clin Exp Rheumatol 2017 Feb 8. Epub 2017 Feb 8.
    Rheumatology Department, Cochin Hospital, Paris Descartes University, Paris, France.
    Objectives: Fibromyalgia (FM) may occur with rheumatoid arthritis (RA) and systemic sclerosis (SSc), and debate remains about its diagnosis. We aimed to use three FM tools (a screening tool (FiRST), diagnostic criteria (ACR 1990 and revised 2010), to compare FM prevalence between RA and SSc patients, to describe the phenotypes of patients with comorbid FM, and to analyze links between FM and secondary Sjögren's syndrome (SS).

    Methods: Consecutive adult patients with confirmed RA or SSc from four university hospitals were tested with the three FM tools. Read More

    Short-term cerebral activity alterations after surgery in patients with unilateral mesial temporal lobe epilepsy associated with hippocampal sclerosis: A longitudinal resting-state fMRI study.
    Seizure 2017 Jan 3;46:43-49. Epub 2017 Jan 3.
    Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China; Southwestern Medical Center, University of Texas, Dallas, TX, USA. Electronic address:
    Purpose: Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS), as a brain network disorder, has given increased importance to resective surgery. We aimed to examine the short-term postoperative functional network reorganization in patients with unilateral MTLE-HS using resting-state functional magnetic resonance imaging (Rs-fMRI) data with a longitudinal design.

    Method: Seventeen (10 right-side and 7 left-side) patients with unilateral MTLE-HS were recruited. Read More

    Reasons for Not Participating in Scleroderma Patient Support Groups: A Cross-sectional Study.
    Arthritis Care Res (Hoboken) 2017 Feb 19. Epub 2017 Feb 19.
    Scleroderma Support Group Project Advisory Team Members.
    Objective: Peer-led support groups are an important resource for many people with scleroderma (systemic sclerosis; SSc). Little is known, however, about barriers to participation. The objective of this study was to identify reasons why some people with SSc do not participate in SSc support groups. Read More

    Diagnostic Red Flags: Steroid-Treated Malignant CNS Lymphoma Mimicking Autoimmune Inflammatory Demyelination.
    Brain Pathol 2017 Feb 18. Epub 2017 Feb 18.
    Institute of Neuropathology, University Medical Center Göttingen, Göttingen, Germany.
    The presence of inflammation and demyelination in a central nervous system (CNS) biopsy points towards a limited, yet heterogeneous group of pathologies, of which multiple sclerosis (MS) represents one of the principal considerations. Inflammatory demyelination has also been reported in patients with clinically suspected primary central nervous system lymphoma (PCNSL), especially when steroids had been administered prior to biopsy acquisition. The histopathological changes induced by corticosteroid treatment can range from mild reduction to complete disappearance of lymphoma cells. Read More

    Development and validation of a patient-reported outcome instrument for skin involvement in patients with systemic sclerosis.
    Ann Rheum Dis 2017 Feb 17. Epub 2017 Feb 17.
    Division of Rheumatology and Clinical Immunology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
    Objectives: We developed a patient-reported outcome (PRO) instrument to assess the skin-related quality of life in patients with systemic sclerosis (SSc).

    Methods: Participants with SSc provided input on skin-related health effects through focus groups. We developed items for scleroderma skin PRO (SSPRO) to encompass these effects. Read More

    Towards developing criteria for scleroderma renal crisis: A scoping review.
    Autoimmun Rev 2017 Feb 14. Epub 2017 Feb 14.
    Faculty of Medicine, McGill University, Montreal, Quebec, Canada; Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Medicine, Division of Rheumatology, Jewish General Hospital, Montreal, Quebec, Canada.
    Objective: The absence of a gold standard for scleroderma renal crisis (SRC) has hindered our understanding of this problem. The objective of this scoping review was to identify the criteria used to define SRC in order to guide the development of a consensus definition for SRC.

    Methods: We conducted a search in three databases: Medline, Embase and non-Ovid Pubmed. Read More

    Future Directions in Imaging Neurodegeneration.
    Curr Neurol Neurosci Rep 2017 Jan;17(1)
    Nantz National Alzheimer Center and Neuroimaging, Houston Methodist Neurological Institute, Houston Methodist Hospital, New York Medical College, Weill Medical College of Cornell University, 6560 Fannin Street, Scurlock 802, Houston, TX, 77030, USA.
    Neuroimaging comprises a powerful set of instruments to diagnose various neurodegenerative disorders, clarifies their neurobiology, and monitors their treatment. Magnetic resonance imaging depicts volume changes, as well as abnormalities in functional and structural connectivity. Positron emission tomography (PET) allows for the quantification of regional cerebral metabolism, characteristically altered in Alzheimer's disease, amyotrophic lateral sclerosis, diffuse Lewy-body disease, and the frontotemporal dementias. Read More

    Morphea "en coup de sabre": An unusual oral presentation.
    J Clin Exp Dent 2017 Feb 1;9(2):e315-e318. Epub 2017 Feb 1.
    DDS, MSc, Department of Oral Medicine and Oral Pathology, Universidad Andres Bello, Viña del Mar, Chile. Avenida Valparaíso 1560, Viña del Mar, Chile.
    Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Oral involvement is unusual and it may produce white linear fibrotic areas with a scar-like appearance, atrophy of tongue papillae, gingival recession and alveolar bone resorption. We report a case of a 13-year-old girl who consulted for progressive recession on the attached gingiva of her upper left incisors. Read More

    Inhibition of phosphodiesterase 4 (PDE4) reduces dermal fibrosis by interfering with the release of interleukin-6 from M2 macrophages.
    Ann Rheum Dis 2017 Feb 16. Epub 2017 Feb 16.
    Department of Internal Medicine 3, Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany.
    Objectives: To investigate the disease-modifying effects of phosphodiesterase 4 (PDE4) inhibition in preclinical models of systemic sclerosis (SSc).

    Methods: We studied the effects of PDE4 inhibition in a prevention and a treatment model of bleomycin-induced skin fibrosis, in the topoisomerase mouse model as well as in a model of sclerodermatous chronic graft-versus-host disease. To better understand the mode of action of PDE4 blockade in preclinical models of SSc, we investigated fibrosis-relevant mediators in fibroblasts and macrophages from healthy individuals and patients suffering from diffuse-cutaneous SSc on blockade of PDE4. Read More

    Pulmonary hypertension in connective tissue diseases: an update.
    Int J Rheum Dis 2017 Feb 16. Epub 2017 Feb 16.
    Department ofClinical Immunology & Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India.
    Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. Read More

    Clinical efficacy and safety of bathing with Chinese medicine Taohong Siwu Decoction () for treatment of diffuse cutaneous systemic sclerosis: A randomized, placebo-controlled trial.
    Chin J Integr Med 2017 Feb 15. Epub 2017 Feb 15.
    Department of Immunology and Rheumatology, Gansu Provincial Hospital, Lanzhou, 730000, China.
    Objective: To examine the efficacy and safety of bathing therapy with Taohong Siwu Decoction (, TSD) in the treatment of early-stage, mild-moderate diffuse cutaneous systemic sclerosis (dcSSc).

    Methods: This randomized, placebo-controlled trial enrolled 148 men and women (18-60 years) with dcSSc (disease duration ≤12 months) and baseline modified Rodnan skin score (MRSS) ≥10. Patients were randomized into a TSD group (71 cases bathing with TSD plus oral prednisone) or control group (71 cases bathing with placebo plus oral prednisone). Read More

    Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS).
    Ann Rheum Dis 2017 Feb 10. Epub 2017 Feb 10.
    UCL Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK.
    Objectives: The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches.

    Methods: This was a prospective, observational cohort study of early dcSSc (within three years of onset of skin thickening). Read More

    Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases.
    Int J Cardiol 2017 Feb 1. Epub 2017 Feb 1.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Background-aim: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis.

    Patients-methods: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. Read More

    Review article: pathogenesis and clinical manifestations of gastrointestinal involvement in systemic sclerosis.
    Aliment Pharmacol Ther 2017 Feb 9. Epub 2017 Feb 9.
    Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Sidney Kimmel Medical College, Philadelphia, PA, USA.
    Background: Gastrointestinal tract (GIT) involvement is a common cause of debilitating symptoms in patients with systemic sclerosis (SSc). There are no disease modifying therapies for this condition and the treatment remains symptomatic, largely owing to the lack of a clear understanding of its pathogenesis.

    Aims: To investigate novel aspects of the pathogenesis of gastrointestinal involvement in SSc. Read More

    Recent advances in managing systemic sclerosis.
    F1000Res 2017 30;6:88. Epub 2017 Jan 30.
    Division of Rheumatology, Medicine III, University Medical Center and Faculty of Medicine Carl Gustav Carus at the TU Dresden, Dresden, Germany.
    How the main components in systemic sclerosis-namely autoimmunity, vasculopathy, and fibrosis-fit together is still not sufficiently clear. However, vascular treatment options are well established, the body of evidence for the efficacy of immunomodulatory approaches is increasing, and now at least one hopeful substance that may directly interfere with fibrosis is being tested. Although we still wait for important breakthroughs, there is grounds for hope that better therapeutic options will be available in the near future. Read More

    Characteristics of human adipose derived stem cells in scleroderma in comparison to sex and age matched normal controls: implications for regenerative medicine.
    Stem Cell Res Ther 2017 Feb 7;8(1):23. Epub 2017 Feb 7.
    Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK.
    Background: Adipose-derived stem cells (ADSCs) are emerging as an alternative stem cell source for cell-based therapies. Recent data suggest that autologous ADSC-enriched micrografting improves the effects of facial involvement in systemic sclerosis (SSc). We have extensively characterised ADSCs from SSc patients and compared their phenotype and function to healthy age- and sex-matched control ADSCs. Read More

    Phenotypic Alterations Involved in CD8+ Treg Impairment in Systemic Sclerosis.
    Front Immunol 2017 19;8:18. Epub 2017 Jan 19.
    Center of Excellence for Biomedical Research, University of Genoa, Genoa, Italy; Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, Genoa, Italy.
    Systemic sclerosis (SSc) is a connective tissue disease characterized by tissue fibrosis, vasculopathy, and autoimmunity. Although the exact pathogenetic mechanisms behind SSc remain to be fully elucidated, a great deal of evidence suggests the existence of an unbalanced ratio between the effector and regulatory arms of the immune system. With regard to the T regulatory (Treg) compartment, we observed that CD8+ Treg subsets display functional defects in SSc-affected patients. Read More

    A Case of Eosinophilic Fasciitis Associated with Pyoderma Gangrenosum.
    J Eur Acad Dermatol Venereol 2017 Feb 1. Epub 2017 Feb 1.
    Department of Internal Medicine, Henry Ford Hospital, Detroit, MI, 48202.
    Eosinophilic fasciitis (EF) is a rare, systemic inflammatory disease that is characterized by symmetrical swelling and scleroderma-like indurations of the distal extremities and trunk, often accompanied by pain and eosinophilia. Rarely, muscle degeneration may occur. Histologically, EF is marked by thickened, fibrotic fascia and fascial inflammation with lymphocytes and eosinophils. Read More

    Biomechanical podiatric evaluation in an Italian cohort of patients with systemic sclerosis: A pilot study.
    Eur J Rheumatol 2016 Dec 1;3(4):169-174. Epub 2016 Dec 1.
    Division of Rheumatology, Department Of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
    Objective: Foot problems are often present in Systemic Sclerosis (SSc) patients, however studies regarding podiatric problems related to SSc are lacking and there are no data evaluating the foot biomechanical changes. The aim of the present pilot study was to evaluate podiatric problems in an Italian cohort of SSc patients by assessing received podiatric services, foot pain and disability and biomechanical foot deformity.

    Material And Methods: 25 consecutive SSc patients were enrolled from the Division of Rheumatology, University of Florence. Read More

    Reduced brain atrophy rates are associated with lower risk of disability progression in patients with relapsing multiple sclerosis treated with cladribine tablets.
    Mult Scler 2017 Jan 1:1352458517690269. Epub 2017 Jan 1.
    Biostatistics Unit, Department of Health Sciences, University of Genoa, Genoa, Italy.
    Background: Neuroimaging studies have used magnetic resonance imaging-derived methods to assess brain volume loss in multiple sclerosis (MS) as a reliable measure of diffuse tissue damage.

    Methods: In the CLARITY study ( NCT00213135), the effect of 2 years' treatment with cladribine tablets on annualized percentage brain volume change (PBVC/y) was evaluated in patients with relapsing MS (RMS). Read More

    Cell Transplant 2017 Jan 31. Epub 2017 Jan 31.
    Scleroderma Clinic, Rheumatology Department, ASST G. Pini-CTO, Milano, Italy.

    Mesenchymal stromal cells (MSCs) have received attention as an ideal source of regenerative cells because of their multi-potential abilities. Adipose tissue (AT) is an attractive source of MSCs. Recent studies have shown that autologous fat grafting may be effective in the treatment of systemic sclerosis (SSc), but no specific study exists aimed at investigating whether AT-MSCs from SSc patients maintain normal phenotypic and functional characteristics. Read More

    Quantitative nanohistological investigation of scleroderma: an atomic force microscopy-based approach to disease characterization.
    Int J Nanomedicine 2017 11;12:411-420. Epub 2017 Jan 11.
    Department of Biomaterials and Tissue Engineering, UCL Eastman Dental Institute.
    Scleroderma (or systemic sclerosis, SSc) is a disease caused by excess crosslinking of collagen. The skin stiffens and becomes painful, while internally, organ function can be compromised by the less elastic collagen. Diagnosis of SSc is often only possible in advanced cases by which treatment time is limited. Read More

    Gastrointestinal Manifestations of Systemic Sclerosis.
    J Scleroderma Relat Disord 2016 18;1(3):247-256. Epub 2016 Oct 18.
    Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, MI, USA.
    In patients with systemic sclerosis (SSc), gastrointestinal (GI) tract involvement is almost universal. Any segment of the GI tract from mouth to anus can be involved, and GI symptoms are a frequent cause of morbidity. In severe cases, GI tract involvement can progress to the point of malnutrition requiring parenteral nutrition. Read More

    Bone marrow-derived mesenchymal stem cells expressing thioredoxin 1 attenuate bleomycin-induced skin fibrosis and oxidative stress in scleroderma.
    J Invest Dermatol 2017 Jan 26. Epub 2017 Jan 26.
    Department of Dermatology, Tongji Hospital, Tongji University School of Medicine, 389 Xin-Cun Road, Shanghai 200065. Electronic address:
    Systemic sclerosis (SSc) is an autoimmune disorder that affects multiple organs. It is characterised by a thickening of the dermis and connective tissue caused by collagen accumulation, and vascular injuries that induce hypoxia. The present study investigated the therapeutic potential of bone marrow-derived mesenchymal stem cells (BMSCs) expressing thioredoxin 1 (Trx-1) in treating SSc-mediated skin disease following transplantation into a bleomycin-induced murine model. Read More

    Survival and outcomes after lung transplantation for non-scleroderma connective tissue-related interstitial lung disease.
    J Heart Lung Transplant 2016 Dec 31. Epub 2016 Dec 31.
    Division of Pulmonary and Critical Care, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania; Division of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts. Electronic address:
    Background: Patients with non-scleroderma connective tissue-related lung disease (NS-CTLD), including rheumatoid arthritis, idiopathic inflammatory myopathies, Sjögren syndrome, mixed connective tissue disease, and systemic lupus erythematosus, may be at risk for worse outcomes after lung transplantation because of immune dysregulation or extrapulmonary manifestations of their underlying disease. We compared survival, acute and chronic rejection, and extrapulmonary organ dysfunction after transplantation in patients with NS-CTLD and idiopathic pulmonary fibrosis (IPF).

    Methods: This was a retrospective cohort study of patients with NS-CTLD and IPF who were listed in the Scientific Registry of Transplant Recipients and underwent lung transplantation from May 5, 2005, to March 1, 2016. Read More

    Potential pathophysiological pathways that can explain the positive effects of exercise on fatigue in multiple sclerosis: A scoping review.
    J Neurol Sci 2017 Feb 4;373:307-320. Epub 2017 Jan 4.
    Section of Sport Science, Department of Public Health, Aarhus University, Aarhus C, Denmark.
    Background: Fatigue is one of the most common and most disabling symptoms of multiple sclerosis (MS). It is a multidimensional and complex symptom with multifaceted origins, involving both central and peripheral fatigue mechanisms. Exercise has proven to be safe for people with MS, with cumulating evidence supporting significant reductions in fatigue. Read More

    "It's not me, it's not really me." Insights from patients on living with systemic sclerosis: An interview study.
    Arthritis Care Res (Hoboken) 2017 Jan 27. Epub 2017 Jan 27.
    Sydney School of Public Health, University of Sydney, Sydney, NSW, 2006.
    Objectives: Patients with systemic sclerosis suffer severe physical limitations and psychological morbidity but their lived experience remains underrepresented and is reflected in the scarcity of evidence based patient-centered interventions. We aimed to describe patients' perspectives of systemic sclerosis to inform strategies to improve their care.

    Methods: Face-to-face semi-structured interviews were conducted with 30 adult patients with limited or diffuse systemic sclerosis in Australia. Read More

    Endothelial dysfunction, microvascular damage and ischemic peripheral vasculopathy in systemic sclerosis.
    Clin Hemorheol Microcirc 2017 Jan 27. Epub 2017 Jan 27.
    Clinical Immunology Unit, Centro Hospitalar do Porto (CHP), Instituto de Ciências Biomédicas Abel Salazar, University of Porto (ICBAS/UP), Multidisciplinar Unit of biomedical investigation (UMIB), Porto, Portugal.
    Objective: To evaluate endothelial dysfunction and microvascular damage in secondary Raynaud Phenomenon (SRP) and Systemic sclerosis (SSc)-associated patients as possible predictors of ischemic fingertip digital ulcers (DU) in a 3-year clinical follow-up.

    Methods: Flow-mediated dilatation (FMD), nailfold videocapillaroscopy (NVC), endothelin-1 (ET-1) and asymmetric dimethylarginine (ADMA) were analysed in a 3-year observational cohort study of 77 SRP patients with systemic sclerosis. The primary outcome was the occurrence of a new DU. Read More

    CT Abnormalities in the Sacroiliac Joints of Patients With Diffuse Idiopathic Skeletal Hyperostosis.
    AJR Am J Roentgenol 2017 Jan 26:1-4. Epub 2017 Jan 26.
    2 Department of Diagnostic Imaging, Sheba Medical Center, Tel Hashomer, affiliated to the Sackler School of Medicine, Tel Aviv 52621, Israel.
    Objective: The purpose of this study is to characterize sacroiliac joints (SIJs) findings at CT of patients with diffuse idiopathic skeletal hyperostosis (DISH), a condition characterized (using the Resnick classification criteria) by ossification of at least four contiguous vertebrae in the thoracic spine and preserved disk space, but without radiographic evidence of intraarticular SIJ abnormalities.

    Materials And Methods: Pelvic CT examinations of 104 patients with DISH (fulfilling the Resnick criteria on spinal CT) and 106 age- and sex-matched control subjects whose entire spine lacked CT evidence of DISH (total, 149 men and 61 women; mean [± SD] age, 72.3 ± 8. Read More

    Human cytomegalovirus (HCMV) UL44 and UL57 specific antibody responses in anti-HCMV-positive patients with systemic sclerosis.
    Clin Rheumatol 2017 Jan 26. Epub 2017 Jan 26.
    Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Viopolis, 40500, Larissa, Thessaly, Greece.
    The role of human cytomegalovirus (HCMV) has been postulated as a trigger of systemic sclerosis (SSc). The aim of the study was to assess the prevalence of antibodies against HCMV UL44 and UL57 antigens not tested in the past. Sixty SSc patients, 40 multiple sclerosis and 17 normal controls (NCs), all anti-HCMV positive, were tested by immunoblotting. Read More

    Kidney transplantation in patients with systemic sclerosis: a nationwide multicenter study.
    Transpl Int 2017 Jan 25. Epub 2017 Jan 25.
    Centre Hospitalier Universitaire de Rouen, Nephrology Dialysis Transplantation, Rouen, Haute-Normandie, FR.
    Kidney transplantation is one of the therapeutic options for end-stage renal disease (ESRD) in systemic sclerosis (SS). Current evidence demonstrates poorer patient and graft survival after transplantation in SS than in other primary kidney diseases. All the patients presenting ESRD associated with SS who had received a kidney allograft between 1987 and 2013 were systematically included from 20 French kidney transplantation centres. Read More

    Fluoroscopy-guided intra-articular steroid injection for sternoclavicular joint arthritis secondary to limited cutaneous systemic sclerosis: a case report.
    Korean J Pain 2017 Jan 30;30(1):59-61. Epub 2016 Dec 30.
    Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Erciyes University, School of Medicine, Kayseri, Turkey.
    We report a case of fluoroscopy-guided intraarticular steroid injection for sternoclavicular joint (SCJ) arthritis caused by limited cutaneous systemic sclerosis (SSc). A 50-year-old woman diagnosed with limited cutaneous SSc presented with swelling and pain in the right SCJ. MRI revealed signs of inflammation consistent with right-sided sternoclavicular joint arthritis. Read More

    Innate Immunity in Systemic Sclerosis.
    Curr Rheumatol Rep 2017 Jan;19(1)
    Department of Applied Sciences, Faculty of Health and Life Sciences, Northumbria University, Ellison Building, Newcastle Upon Tyne, NE2 8ST, UK.
    Purpose Of Review: Systemic sclerosis (SSc) is a heterogeneous autoimmune disease which has defined three hallmarks: Small vessel vasculopathy, production of autoantibodies and fibroblast dysfunction. The exact aetiology of the disease remains unknown, due to the complex nature of the cellular signalling pathways involved. However, there is strong and consistent evidence that the innate system, in particular toll-like receptor signalling, is contributing to the progression and perhaps onset of systemic sclerosis. Read More

    Artifactual Hypoglycaemia in Systemic Sclerosis and Raynaud's Phenomenon: A Clinical Case Report and Short Review.
    Case Rep Endocrinol 2016 28;2016:7390927. Epub 2016 Dec 28.
    Department of Endocrinology and Metabolism, Concord Repatriation General Hospital, Concord, Sydney, NSW 2139, Australia; Sydney Medical School, University of Sydney, Sydney, NSW 2005, Australia.
    Background. Artifactual hypoglycaemia, defined as a discrepancy between glucometer (capillary) and plasma glucose levels, may lead to overtreatment and costly investigations. It is not infrequently observed in patients with Raynaud's phenomenon due to vascular capillary distortion, yet this is clinically underappreciated. Read More

    Self-care behaviors and trajectory management by people with scleroderma in northeastern Thailand.
    Int J Nurs Pract 2017 Jan 23. Epub 2017 Jan 23.
    Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
    Scleroderma, particularly systemic sclerosis, is a chronic illness that affects a person's physical, emotional, psychosocial, and spiritual dimensions. To investigate self-care deficits, health behaviors, and trajectory management of Thai people with systemic sclerosis, an exploratory case study method was used through interviews and patient records. Self-care deficit nursing theory and the chronic illness trajectory framework guided the research. Read More

    Kidney disease in hepatitis B surface antigen-positive children: experience from a centre in south-west Nigeria and a review of the Nigerian literature.
    Paediatr Int Child Health 2017 Jan 23:1-7. Epub 2017 Jan 23.
    f Centre for Chronic Disease, School of Medicine , The University of Queensland , Brisbane , Australia.
    Background: Kidney disease is an important extra-hepatic manifestation of hepatitis B virus (HBV) infection. However, there is paucity of recent literature on kidney disease in children and adolescents with HBV infection from several parts of sub-Saharan Africa including Nigeria.

    Objective: To review the pattern of kidney disease in hepatitis B surface antigen (HBsAg)-positive children and adolescents seen at a tertiary hospital in south-west Nigeria. Read More

    [Plasma exchange in nephrology: Indications and technique].
    Nephrol Ther 2017 Feb 16;13(1):43-55. Epub 2017 Jan 16.
    Service des urgences néphrologiques et transplantation rénale, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France; Université Pierre-et-Marie-Curie, 4, place Jussieu, 75005 Paris, France.
    Plasma exchange is a non-selective apheresis technique that can be performed by filtration or centrifugation allowing rapid purification of high molecular weight pathogens. An immunosuppressive treatment is generally associated to reduce the rebound effect of the purified substance. Substitution solutes such as human albumin and macromolecules are needed to compensate for plasma extraction. Read More

    Histopathological findings in systemic sclerosis-related myopathy: fibrosis and microangiopathy with lack of cellular inflammation.
    Ther Adv Musculoskelet Dis 2017 Jan 3;9(1):3-10. Epub 2016 Oct 3.
    Scleroderma Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy.
    Objectives: The objective of this study was to identify specific histopathological features of skeletal muscle involvement in systemic sclerosis (SSc) patients.

    Methods: A total of 35 out of 112 SSc-patients (32%, including 81% female and 68% diffuse scleroderma) presenting clinical, biological and electromyographic (EMG) features of muscle weakness, were included. Patients underwent vastus lateralis biopsy, assessed for individual pathologic features including fibrosis [type I collagen (Coll-I), transforming growth factor β (TGF-β)], microangiopathy [cluster of differentiation 31 (CD31), pro-angiogenic vascular endothelial growth factor A (VEGF-A), anti-angiogenic VEGF-A165b], immune/ inflammatory response [CD4, CD8, CD20, human leucocyte antigens ABC (HLA-ABC)], and membranolytic attack complex (MAC). Read More

    Prevalence and Severity of Depression and Anxiety in Patients With Systemic Sclerosis: An Epidemiologic Survey and Investigation of Clinical Correlates.
    J Clin Rheumatol 2017 Mar;23(2):80-86
    From the Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
    Background: Systemic sclerosis (SSc) is a chronic multisystem connective tissue disorder with detrimental impact on quality of life. Patients with SSc face emotional distress and frequently meet criteria for a psychiatric disorder. However, the pattern of psychiatric manifestations may vary according to socioethnic background. Read More

    DT MRI microstructural cortical lesion damage does not explain cognitive impairment in MS.
    Mult Scler 2017 Jan 1:1352458516689147. Epub 2017 Jan 1.
    Neuroimaging Research Unit, Institute of Experimental Neurology, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy/Department of Neurology, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.
    Objective: We combined double inversion recovery (DIR) and diffusion tensor (DT) magnetic resonance imaging (MRI) to quantify the severity of cortical lesion (CL) microstructural tissue abnormalities in a large cohort of relapse-onset multiple sclerosis (MS) patients and its contribution to cognitive dysfunction.

    Methods: DIR, DT, dual-echo, and three-dimensional (3D) T1-weighted scans were acquired from 149 MS patients and 40 controls. Cognitively impaired (CI) patients had ⩾2 abnormal neuropsychological tests. Read More

    Exosomes isolated from serum of systemic sclerosis patients display alterations in their content of profibrotic and antifibrotic microRNA and induce a profibrotic phenotype in cultured normal dermal fibroblasts.
    Clin Exp Rheumatol 2017 Jan 13. Epub 2017 Jan 13.
    The Scleroderma Center and The Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, PA, USA.
    Objectives: Exosomes are lipid bilayer-bound microvesicles containing various macromolecules including numerous microRNA (miRNA). Exosomes mediate intercellular communication by fusing and releasing their macromolecular content into target cells. Here, we analysed the content of profibrotic and antifibrotic miRNAs in exosomes isolated from the serum of systemic sclerosis (SSc) patients and tested their ability to induce a profibrotic phenotype in normal human dermal fibroblasts in vitro. Read More

    Occupational and environmental scleroderma. Systematic review and meta-analysis.
    Clin Rheumatol 2017 Mar 14;36(3):569-582. Epub 2017 Jan 14.
    Autoimmune Diseases Unit. Department of Internal Medicine, Bellvitge University Hospital-IDIBELL. L'Hospitalet de Llobregat, Feixa Llarga s/n, Hospitalet de Llobregat, Barcelona, Spain.
    The etiology of systemic sclerosis (SSc) remains unknown; however, several occupational and environmental factors have been implicated. Our objective was to perform a meta-analysis of all studies published on SSc associated with occupational and environmental exposure. The review was undertaken by means of MEDLINE and SCOPUS from 1960 to 2014 and using the terms: "systemic," "scleroderma," or "systemic sclerosis/chemically induced" [MesH]. Read More

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