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 The most effective prokinetics belonging to the serotonin (5-HT4) receptor agonists class were withdrawn due to cardiac toxicity in relation to modest 5-HT4 receptor affinity.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

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

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

    [The influence of STAT4 rs7574865 (G/T) polymorphism on the risk of clinical and immunological phenotypes of systemic sclerosis in a Russian patient population: Results of a pilot study].
    Ter Arkh 2017 ;89(5):20-25
    All-Russian Research Institute of Agricultural Biotechnology, Moscow, Russia.
    Aim: To examine the association of signal transducer and activator transcription 4 (STAT4) rs7574865 G/T polymorphism with a predisposition to systemic sclerosis (SSC) and associated clinical and autoimmune phenotypes in a Russian population.

    Subjects And Methods: A total of 102 patients with SSC and 103 healthy individuals as controls were examined. STAT4 rs7574865 polymorphism was investigated by real-time polymerase chain reaction. Read More

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

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

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

    Evaluation and management approaches for scleroderma lung disease.
    Ther Adv Respir Dis 2017 Jun 1:1753465817713680. Epub 2017 Jun 1.
    Division of Rheumatology, Massachusetts General Hospital, 55 Fruit Street, Yawkey 2C-2100, Boston, MA 02114, USA.
    Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are leading causes of morbidity and mortality in systemic sclerosis (SSc). As symptoms are often under-reported in SSc, early screening of ILD and PAH is of paramount importance, and early treatment may be associated with better clinical outcomes. Serologies are particularly helpful in identifying patients at risk for pulmonary involvement. Read More

    Primary Progressive Multiple Sclerosis: Putting Together the Puzzle.
    Front Neurol 2017 31;8:234. Epub 2017 May 31.
    Department of Neurology, Ulm University, Ulm, Germany.
    The focus of multiple sclerosis research has recently turned to the relatively rare and clearly more challenging condition of primary progressive multiple sclerosis (PPMS). Many risk factors such as genetic susceptibility, age, and Epstein-Barr virus (EBV) infection may interdepend on various levels, causing a complex pathophysiological cascade. Variable pathological mechanisms drive disease progression, including inflammation-associated axonal loss, continuous activation of central nervous system resident cells, such as astrocytes and microglia as well as mitochondrial dysfunction and iron accumulation. Read More

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

    [Differential diagnosis of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy].
    Zh Nevrol Psikhiatr Im S S Korsakova 2017 ;117(4):75-80
    Research Сenter of Neurology, Moscow, Russia.
    Cerebral autosomal dominant arteriopathy with subcortical infarctions and leucoencephalopathy (CADASIL) is an inherited CNS disease, which is caused by mutations in the NOTCH3 gene. Selective disorders of small vessels underlie the disease pathogenesis. Clinically CADASIL is characterized by headaches, multiple stroke-like disorders (in most cases transient ischemic attacks and lacunar strokes), and different focal neurological symptoms and dementia. Read More

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

    Collagen Anomalies as Clues for Diagnosis: Part 2.
    Am J Dermatopathol 2017 Jun 6. Epub 2017 Jun 6.
    *Department of Dermatology, Hospital Universitario Doctor Peset, Valencia, Spain; †Department of Dermatology, Complexo Hospitalario de A Coruña, A Coruña, Spain; ‡Department of Dermatology, Hospital General Universitario de Ciudad Real, Ciudad Real, Spain; §Department of Dermatopathology, Hospital General de Mexico Doctor Eduardo Liceaga, Mexico City, Mexico; ¶Department of Dermatology, Hospital Militar Central de Lima, Lima, Perú; ‖Department of Pathology, Hospital Universtario Virgen de La Macarena, Sevilla, Spain; and **Department of Dermatology, Fundación Jiménez Diaz, Universidad Autónoma, Madrid, Spain.
    Background: Collagen is the most abundant protein in the body and the main structural component of the skin.

    Objective: To provide a review of the histopathology of collagen alterations and to propose a classification with the most important types of collagen anomalies in dermatopathology. The authors describe some of the main morphological clues of collagen anomalies for specific diagnosis of some cutaneous inflammatory and neoplastic conditions. Read More

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

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

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

    Manufacture of Autologous CD34+ Selected Grafts in the NIAID-Sponsored HALT-MS and SCOT Multicenter Clinical Trials for Autoimmune Diseases.
    Biol Blood Marrow Transplant 2017 Jun 5. Epub 2017 Jun 5.
    National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA. Electronic address:
    To ensure comparable grafts for autologous HCT in NIAID-sponsored IND protocols for multiple sclerosis (HALT-MS) and systemic sclerosis (SCOT), a DMF approach to control manufacture was implemented, including a common Master Production Batch Record, and site-specific SOPs with "Critical Elements". We assessed comparability of flow cytometry and controlled rate cryopreservation among sites, and stability of cryopreserved grafts, using hematopoietic progenitor cells (HPC) from healthy donors. Auto-CD34+HPC graft specifications included ≥70% viable CD34+ cells before cryopreservation. Read More

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

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

    Staining of HLA-DR, Iba1 and CD68 in human microglia reveals partially overlapping expression depending on cellular morphology and pathology.
    J Neuroimmunol 2017 Aug 20;309:12-22. Epub 2017 Apr 20.
    Neuroimmunology Research Group, Netherlands Institute for Neuroscience, Meibergdreef 47, 1105 BA Amsterdam, The Netherlands. Electronic address:
    HLA-DR, Iba1 and CD68 are widely used microglia markers in human tissue. However, due to differences in gene regulation, they may identify different activation stages of microglia. Here, we directly compared the expression of HLA-DR, Iba1 and CD68 in microglia with different phenotypes, ranging from ramified to amoeboid, to foamy phagocytizing macrophages, in adjacent sections immunocytochemically double stained for two of the markers. Read More

    Poor survival in patients with scleroderma and pulmonary hypertension due to heart failure with preserved ejection fraction.
    Pulm Circ 2017 Apr-Jun;7(2):409-420. Epub 2017 Mar 27.
    1 Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University, Baltimore, MD, USA.
    Pulmonary hypertension due to heart failure with preserved ejection fraction (PH-HFpEF) has been poorly studied in patients with systemic sclerosis (SSc). We sought to compare clinical characteristics and survival of SSc patients with PH-HFpEF (SSc-PH-HFpEF) versus pulmonary arterial hypertension (SSc-PAH). We hypothesized that patients with SSc-PH-HFpEF have a similar poor overall prognosis compared with patients with SSc-PAH when matched for total right ventricular load. Read More

    Pulmonary arterial hypertension in scleroderma: care gaps in screening.
    Arthritis Res Ther 2017 Jun 6;19(1):128. Epub 2017 Jun 6.
    Department of Medicine, Division of Rheumatology, University of Western Ontario, St. Joseph's Health Care, 268 Grosvenor Street, London, ON, N6A 4V2, Canada.
    One in six patients with systemic sclerosis will develop pulmonary arterial hypertension (PAH). Screening with echocardiography and possibly pulmonary function testing (to determine the diffusing capacity of carbon monoxide) is recommended to detect PAH at a less severe stage. However, real-world screening programs have problems. Read More

    Mortality in the Waikato Hospital Systemic Sclerosis Cohort.
    Int J Rheum Dis 2017 Jun 4. Epub 2017 Jun 4.
    Waikato Clinical School, University of Auckland, Auckland, New Zealand.
    Objective: To characterize the causes of mortality and standardised mortality ratio in a cohort of patients with systemic sclerosis (SSc).

    Methods: A cohort of 132 patients enrolled at the Waikato Systemic Sclerosis Clinic was prospectively followed from 2005 to 2016. Patient demographics, diagnoses and laboratory reports were used to assess risk of mortality and generate standardised mortality ratios (SMR). Read More

    Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension.
    Arthritis Res Ther 2017 Jun 2;19(1):122. Epub 2017 Jun 2.
    Department of Medicine, The University of Melbourne at St Vincent's Hospital, 41 Victoria Parade, Fitzroy, 3065, Melbourne, Victoria, Australia.
    Background: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). We sought to determine survival, predictors of mortality, and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH.

    Methods: We studied consecutive SSc patients with newly diagnosed (incident) World Health Organization (WHO) Group 1 PAH enrolled in a prospective cohort between 2009 and 2015. Read More

    Use of Patterned Collagen Coated Slides to Study Normal and Scleroderma Lung Fibroblast Migration.
    Sci Rep 2017 Jun 1;7(1):2628. Epub 2017 Jun 1.
    Centre for Rheumatology and Connective Tissue Disease, Royal Free Hospital Campus, University College Medical School, Rowland Hill Street, London, NW3 2PF, UK.
    Systemic sclerosis (SSc) is a spreading fibrotic disease affecting the skin and internal organs. We aimed to model pathogenic fibroblast migration in SSc in order to identify enhancing factors, measure the effect of migrating cells on underlying extracellular matrix (ECM) and test possible therapeutic inhibitors. Novel patterned collagen substrates were used to investigate alignment and migration of skin and lung fibroblasts from SSc patients and healthy controls. Read More

    The role of endothelial cells in the vasculopathy of systemic sclerosis: A systematic review.
    Autoimmun Rev 2017 May 29. Epub 2017 May 29.
    Ghent University, Department of Internal Medicine, Ghent, Belgium; Ghent University Hospital, Department of Rheumatology, Ghent, Belgium.
    Introduction: Systemic sclerosis (SSc) is an autoimmune connective tissue disorder characterized by fibroproliferative vasculopathy, immunological abnormalities and progressive fibrosis of multiple organs including the skin. In this study, all English speaking articles concerning the role of endothelial cells (ECs) in SSc vasculopathy and representing biomarkers are systematically reviewed and categorized according to endothelial cell (EC) (dys)function in SSc.

    Methods: A sensitive search on behalf of the EULAR study group on microcirculation in Rheumatic Diseases was developed in Pubmed, The Cochrane Library and Web of Science to identify articles on SSc vasculopathy and the role of ECs using the following Mesh terms: (systemic sclerosis OR scleroderma) AND pathogenesis AND (endothelial cells OR marker). Read More

    Periostin in Mature Stage Localized Scleroderma.
    Ann Dermatol 2017 Jun 11;29(3):268-275. Epub 2017 May 11.
    Department of Dermatology, SMG-SNU Boramae Medical Center, Seoul, Korea.
    Background: Periostin is a novel matricellular protein expressed in many tissues, including bone, periodontal ligament, and skin. Although its expression is prominent in various fibrotic conditions, studies of periostin in localized scleroderma are rare.

    Objective: To investigate the expression of periostin and other molecules in localized scleroderma. Read More

    Disconnection as a mechanism for social cognition impairment in multiple sclerosis.
    Neurology 2017 May 31. Epub 2017 May 31.
    From the Department of Neurology (S.B., C.M., L.S., I.S., L.C.) and Medical Imaging Department (R.F.-M.), Centro Hospitalar e Universitário de Coimbra; and Visual Neuroscience Laboratory, Institute for Biomedical Imaging and Life Sciences (IBILI) (C.A., O.C.d., J.P., M.C.-B.), Faculty of Medicine (S.B., A.A., L.S., I.S., L.C.), Centre for Neuroscience and Cell Biology (CNC), IBILI (C.A., O.C.d., J.P., M.C.-B., I.S.), and Institute for Nuclear Sciences Applied to Health (ICNAS) (C.A., O.C.d., J.P., M.C.-B.), University of Coimbra, Portugal.
    Objective: To assess the contribution of microstructural normal-appearing white matter (NAWM) damage to social cognition impairment, specifically in the theory of mind (ToM), in multiple sclerosis (MS).

    Methods: We enrolled consecutively 60 patients with MS and 60 healthy controls (HC) matched on age, sex, and education level. All participants underwent ToM testing (Eyes Test, Videos Test) and 3T brain MRI including conventional and diffusion tensor imaging sequences. Read More

    Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.
    J Am Soc Nephrol 2017 May 31. Epub 2017 May 31.
    Division of Pediatric Nephrology, University Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany;
    We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models. Read More

    Very early and early systemic sclerosis in the Spanish scleroderma Registry (RESCLE) cohort.
    Autoimmun Rev 2017 May 28. Epub 2017 May 28.
    Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
    Objectives: According to the existence of subclinical organ involvement pre-scleroderma should be divided into two subsets: very early and early disease. Pre-scleroderma patients included in the Spanish Scleroderma Registry (RESCLE) Cohort were reclassified into subsets. Differences were evaluated and the risk of progression to definite systemic sclerosis was estimated. Read More

    Successful treatment of infliximab in a patient with scleroderma: a case report.
    Medicine (Baltimore) 2017 Jun;96(22):e6737
    Department of Rheumatology and Immunology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, China.
    Rationale: Systemic Scleroderma (SSc) is a rare connective tissue disease clinically characterized by cutaneous sclerosis and variable systemic involvement. No drug is currently available to effectively reverse the fibrotic process in SSc. Previous reports have suggested that the tumor necrosis factor (TNF) antagonists could be useful for the treatment of fibrotic disorders. Read More

    Use of Smooth Muscle Myosin Heavy Chain as an Effective Marker of Follicular Dendritic Cells.
    Appl Immunohistochem Mol Morphol 2017 May 25. Epub 2017 May 25.
    *Department of Pathology, Pennsylvania Hospital (University of Pennsylvania Health System), Philadelphia, PA †Department of Pathology, University of South Alabama Medical Center, Mobile, AL.
    Smooth muscle myosin heavy chain (SMMHC) is a major structural component of the contractile apparatus in smooth muscle cells. Even though it is considered a relatively specific marker for terminal smooth muscle cell differentiation, expression in other cell types such as follicular dendritic cells (FDCs) has rarely been reported. To determine whether SMMHC represents an effective FDC marker in lymphoid tissues, we compared the immunohistochemical results for SMMHC with those of the traditional FDC markers podoplanin (D2-40) and CD21. Read More

    Fibrosing myopathy in systemic sclerosis associates with higher mortality.
    Arthritis Care Res (Hoboken) 2017 May 23. Epub 2017 May 23.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
    Objective: To determine if a unique subtype of scleroderma muscle disease exists by comparing the clinical features of systemic sclerosis (SSc) patients with predominant fibrosis on muscle biopsy to those with inflammatory muscle histopathology.

    Methods: This retrospective, cross-sectional study included SSc patients with muscle weakness and an available muscle biopsy. Biopsies with fibrosis but without inflammation/necrosis were designated as "fibrosing myopathy" and those with inflammation and/or necrosis were assigned a category of "inflammatory myopathy". Read More

    Performance of Anti-nuclear Antibodies for Classifying Systemic Lupus Erythematosus: a Systematic Literature Review and Meta-regression of Diagnostic Data.
    Arthritis Care Res (Hoboken) 2017 May 23. Epub 2017 May 23.
    Rheumatology, Clinical Immunology and Allergy, University of Crete Medical School, Heraklion, Greece.
    Objective: To review the published literature on the performance of indirect immunofluorescence (IIF)-HEp-2 ANA testing for classification of SLE.

    Methods: A systematic literature search was conducted in the MEDLINE, EMBASE and COCHRANE databases for articles published between January 1990 and October 2015. The research question was structured according to PICO (Population, Intervention, Comparator, Outcome) format rules, and PRISMA recommendations were followed where appropriate. Read More

    Efficacy of mycophenolate mofetil and oral cyclophosphamide on skin thickness: Post-hoc analyses from the Scleroderma Lung Study I and II.
    Arthritis Care Res (Hoboken) 2017 May 23. Epub 2017 May 23.
    Division of Rheumatology, University of Michigan Scleroderma Program, Ann Arbor, MI, USA.
    Objectives: To assess the efficacy of mycophenolate mofetil (MMF) and cyclophosphamide (CYC) on the modified Rodnan skin score (mRSS) in participants enrolled in the Scleroderma Lung Study (SLS)-I and II.

    Methods: SLS-I participants received daily oral CYC or matching placebo for one year, whereas SLS-II participants received daily MMF for 2 years or daily oral CYC for 1 year followed by placebo for second year. We assessed the impact of MMF and CYC on the mRSS in SLS-II over 24-month period. Read More

    FGF-23, Klotho and Vitamin D Levels in Scleroderma.
    Iran J Public Health 2017 Apr;46(4):530-536
    Dept. of Clinical Biochemistry and Laboratory Medicine, Tabriz University of Medical Sciences, Tabriz, Iran.
    Background: Scleroderma is a chronic connective tissue disease of unknown etiology. Vitamin D and parathyroid hormone (PTH) that play particular functions in calcium and phosphate homeostasis may be involved in the etiology of this disorder. Klotho, the co-receptor of the fibroblast growth factor 23 (FGF-23), can interfere with calcium and phosphate metabolism. Read More

    Coexistence of diabetes mellitus type 1 with diffuse systemic sclerosis - case report and literature review.
    Reumatologia 2017 28;55(2):104-107. Epub 2017 Apr 28.
    Department of Rheumatology and Connective Tissue Diseases, Medical University, Lublin, Poland.
    Diabetic sclerodactyly is a frequently recognized skin finding that may occur in patients with diabetes mellitus but coexistence of diabetes and systemic sclerosis is rare. We describe a case of coexistence of type 1 diabetes mellitus and systemic sclerosis in 42-year-old man with the history of Raynaud's phenomenon, progressive diffuse hardening of the skin and sclerodactyly, slowly worsening with time. The medical history included type 1 diabetes since childhood with microvascular complications. Read More

    Serum prolidase activity in systemic sclerosis.
    Clin Rheumatol 2017 May 22. Epub 2017 May 22.
    Medical Faculty, Department of Medical Biochemistry, Sutcu Imam University, Kahramanmaras, Turkey.
    Systemic sclerosis, also known as scleroderma, is a complex systemic inflammatory autoimmune disease that targets the vasculature and connective tissue-producing cells and components of the innate and adaptive immune systems. The disease is characterized by a hardening of the skin and an increased synthesis of collagen . Prolidase is a specific imidodipeptidase involved in collagen degradation. Read More

    Echocardiographic Predictors for Worsening of Six-Minute Walk Distances in Patients With Systemic Sclerosis (Scleroderma).
    Am J Cardiol 2017 Jul 26;120(2):315-321. Epub 2017 Apr 26.
    Department of Cardiovascular Medicine, Tokushima University Hospital, Tokushima, Japan.
    Change in 6-minute walk distance (6MWD) has been used as a clinical marker in pulmonary hypertension. Determinants and worsening of 6MWD remain a matter of debate because nonpulmonary factors have an impact on the 6MWD. We hypothesized that future reduction of 6MWD in patients with systemic sclerosis (SSc) was more closely associated with cardiac dysfunction. Read More

    Sirt1 ameliorates systemic sclerosis by targeting the mTOR pathway.
    J Dermatol Sci 2017 May 3. Epub 2017 May 3.
    Division of Rheumatology, Huashan Hospital, Fudan University, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, China. Electronic address:
    Background: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by inflammation and fibrosis. Our previous research has indicated that Sirtuin1 (Sirt1) plays a role in the regulation of TNF-α-induced inflammation; however, whether Sirt1 may inhibit the progress of SSc by blocking inflammation remains unknown.

    Objective: We aimed to investigate the function of Sirt1 in SSc. Read More

    Cardiopulmonary assessment of patients with systemic sclerosis for hematopoietic stem cell transplantation: recommendations from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party and collaborating partners.
    Bone Marrow Transplant 2017 May 22. Epub 2017 May 22.
    Department of Haematology, Sheffield Teaching Hospitals NHS Foundation Trust, University of Sheffield, Sheffield, UK.
    Systemic sclerosis (SSc) is a rare disabling autoimmune disease with a similar mortality to many cancers. Two randomized controlled trials of autologous hematopoietic stem cell transplantation (AHSCT) for SSc have shown significant improvement in organ function, quality of life and long-term survival compared to standard therapy. However, transplant-related mortality (TRM) ranged from 3-10% in patients undergoing HSCT. Read More

    Increased proportions of functionally impaired regulatory T cell subsets in systemic sclerosis.
    Clin Immunol 2017 May 15. Epub 2017 May 15.
    Department of Immunology and Biotechnology, University of Pécs, Clinical Center, Szigeti út 12, 7624 Pécs, Hungary. Electronic address:
    Treg abnormalities have been implicated in the pathogenesis of systemic sclerosis (SSc). Treg subpopulations and their cytokines, IL-10 and TGF-β in the peripheral blood of early stage SSc patients were investigated. We hypothesized that epigenetically regulated methylation of the FOXP3 promoter and enhancer regions are altered in Tregs of SSc patients, which might be involved in the T cell imbalance. Read More

    Validity of the Workers Productivity and Activity Impairment Questionnaire: Specific Health Problem (WPAI:SHP) in patients with systemic sclerosis.
    Clin Exp Rheumatol 2017 May 8. Epub 2017 May 8.
    Department of Medicine, The University of Melbourne at St. Vincent's Hospital; and Department of Rheumatology, St. Vincent's Hospital, Melbourne, Australia.
    Objectives: To evaluate the construct validity of the Workers Productivity and Impairment Activity Index: Specific Health Problem (WPAI:SHP) in Australian systemic sclerosis (SSc) patients.

    Methods: SSc patients, identified through the Australian Scleroderma Cohort Study database, completed the WPAI:SHP and a quality of life instrument (PROMIS-29) cross-sectionally. The construct validity of the WPAI:SHP was assessed by the correlations between the WPAI:SHP and a range of SSc health states. Read More

    Standardization of the modified Rodnan skin score for use in clinical trials of systemic sclerosis.
    J Scleroderma Relat Disord 2017 Jan-Apr;2(1):11-18
    Centre for Rheumatology, Royal Free and University College London Medical School, London, UK.
    The modified Rodnan skin score (mRSS) is a measure of skin thickness and is used as a primary or secondary outcome measure in clinical trials of systemic sclerosis (scleroderma). This state-of-art review provides a historical perspective of the development of the mRSS, summarizes the performance of mRSS as an outcome measure, provides guidance on assessing mRSS, and makes recommendations for incorporation of the mRSS into clinical trials. Read More

    Circulating Anti-Nuclear Antibodies in Systemic Sclerosis: Utility in Diagnosis and Disease Subsetting.
    J Nippon Med Sch 2017 ;84(2):56-63
    Department of Allergy and Rheumatology, Nippon Medical School.
    The presence of circulating anti-nuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). Currently, a variety of SSc-specific ANAs, including anticentromere, anti-topoisomerase I, and anti-RNA polymerase III antibodies, have been well characterized, and their commercial kits are available worldwide. Since these autoantibodies are specifically detected in SSc patients and are associated with unique sets of disease manifestations, they are widely used in routine clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvements and prognosis. Read More

    IL-10-producing regulatory B cells (B10 cells), IL-17+ T cells and autoantibodies in systemic sclerosis.
    Clin Immunol 2017 May 11. Epub 2017 May 11.
    Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa 40500, Greece. Electronic address:
    We aimed to analyze IL-10+ Breg (B10) cells, found to be reduced in systemic sclerosis (SSc), in relation to SSc-specific autoAbs and IL-17+ and IFNγ+ T cells in SSc. Peripheral blood B10 cells from 26 patients with SSc positive for anti-Topo I or anti-Cen autoAbs, and 12 healthy controls (HC) were studied by flow cytometry. IL-17+ and IFNγ+ T cells were also studied. Read More

    Clinical characteristics and survival of systemic sclerosis patients with pulmonary hypertension and elevated wedge pressure: Observations from the PHAROS cohort.
    Respirology 2017 May 12. Epub 2017 May 12.
    Division of Rheumatology, Georgetown University, Washington, DC, USA.
    Background And Objective: Systemic sclerosis (SSc) is a complex autoimmune disease commonly associated with pulmonary hypertension (PH). When associated with elevated pulmonary artery wedge pressure (PAWP), pulmonary artery pressure (PAP) is either in-proportion (post-capillary PH) or higher than expected (combined PH) relative to the increased PAWP.

    Methods: Patients from the PHAROS registry (a prospective observational cohort of SSc-PH patients) who had mean PAP ≥ 25 and PAWP > 15 on right heart catheterization were stratified based on diastolic pressure gradient (DPG). Read More

    Different aggregation states of a nuclear localization signal-tagged 25-kDa C-terminal fragment of TAR RNA/DNA-binding protein 43 kDa.
    Genes Cells 2017 Jun 12;22(6):521-534. Epub 2017 May 12.
    Laboratory of Molecular Cell Dynamics, Faculty of Advanced Life Science, Hokkaido University, Sapporo, Japan.
    The mechanism and cause of motor neuronal cell death in amyotrophic lateral sclerosis (ALS), a devastating neurodegenerative disorder, are unknown; gain of function of oligomers and aggregation of misfolded proteins, including carboxyl-terminal fragments (CTFs) of TAR RNA/DNA-binding protein 43 kDa (TDP-43), have been proposed as important causative factors in the onset of ALS. We recently reported that a nuclear localization signal (NLS)-tagged 25-kDa CTF of TDP-43 (TDP25) could decrease the cell-death proportion compared with that promoted by TDP25. Here, we show oligomeric states of NLS-TDP25 and its detailed localization property using super-resolution fluorescence microscopy, FRET, fluorescence recovery after photobleaching, and fluorescence correlation spectroscopy analysis. Read More

    Disturbed microcirculation in the hands of patients with systemic sclerosis detected by fluorescence optical imaging: a pilot study.
    Arthritis Res Ther 2017 May 8;19(1):87. Epub 2017 May 8.
    Department of Rheumatology and Clinical Immunology, Charité University Hospital, Berlin, Germany.
    Background: Utilising fluorescence optical imaging (FOI), the distribution of an intravenously applied colouring agent indocyanine green (ICG) can be analysed with the potential to identify malperfusion by little to no tissue enhancement. Systemic sclerosis (SSc) is characterised by the presence of digital ulcers reflecting progressive vasculopathy. The objective was to investigate the potential of FOI in the detection of disturbed microcirculation in the hands and fingers of patients with SSc and to link FOI findings to clinical signs of ischemia such as digital ulcers and pitting scars. Read More

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