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    Performance of the Patient-Reported Outcomes Measurement Information System-29 in scleroderma: a Scleroderma Patient-centered Intervention Network Cohort Study.
    Rheumatology (Oxford) 2017 Apr 18. Epub 2017 Apr 18.
    Department of Medicine, McGill UniversityMontréal, Québec, Canada.
    Objective.: The Patient-Reported Outcomes Measurement Information System (PROMIS)-29 assesses seven health-related quality of life domains plus pain intensity. The objective was to examine PROMIS-29v2 validity and explore clinical associations in patients with SSc. Read More

    Natriuretic peptide receptor guanylyl cyclase-A pathway counteracts glomerular injury evoked by aldosterone through p38 mitogen-activated protein kinase inhibition.
    Sci Rep 2017 Apr 21;7:46624. Epub 2017 Apr 21.
    Department of Nephrology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
    Guanylyl cyclase-A (GC-A) signaling, a natriuretic peptide receptor, exerts renoprotective effects by stimulating natriuresis and reducing blood pressure. Previously we demonstrated massive albuminuria with hypertension in uninephrectomized, aldosterone-infused, and high salt-fed (ALDO) systemic GC-A KO mice with enhanced phosphorylation of p38 mitogen-activated protein kinase (MAPK) in podocytes. In the present study, we examined the interaction between p38 MAPK and GC-A signaling. Read More

    Understanding the role of antibodies as markers of cancer coexistence in systemic sclerosis: Comment on the Article by Shah et al.
    Arthritis Rheumatol 2017 Apr 20. Epub 2017 Apr 20.
    Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. Universidad Autónoma de Barcelona, Barcelona, Spain.

    Short term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis.
    Arthritis Rheumatol 2017 Apr 20. Epub 2017 Apr 20.
    Royal Brompton and Harefield NHS Foundation Trust, London, UK.
    Objective: To determine the prognostic value of pulmonary function test (PFT) trends at one and two years in interstitial lung disease associated with systemic sclerosis (SSc-ILD).

    Methods: The prognostic significance of PFT trends at one year (n=162), and two years (n=140) was examined against 15 year survival. PFT trends, expressed as continuous and categorical change in separate analyses, were examined against mortality in univariate and multivariate models. Read More

    Unifying mechanism for different fibrotic diseases.
    Proc Natl Acad Sci U S A 2017 Apr 19. Epub 2017 Apr 19.
    Institute for Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine, Stanford, CA 94305;
    Fibrotic diseases are not well-understood. They represent a number of different diseases that are characterized by the development of severe organ fibrosis without any obvious cause, such as the devastating diseases idiopathic pulmonary fibrosis (IPF) and scleroderma. These diseases have a poor prognosis comparable with endstage cancer and are uncurable. Read More

    PIF* promotes brain re-myelination locally while regulating systemic inflammation- clinically relevant multiple sclerosis M.smegmatis model.
    Oncotarget 2017 Mar;8(13):21834-21851
    Università Cattolica del S. Cuore, Institute of General Pathology, Largo Francesco Vito, 100168 Rome, Italy.
    Neurologic disease diagnosis and treatment is challenging. Multiple Sclerosis (MS) is a demyelinating autoimmune disease with few clinical forms and uncertain etiology. Current studies suggest that it is likely caused by infection(s) triggering a systemic immune response resulting in antigen/non-antigen-related autoimmune response in central nervous system (CNS). Read More

    Treatment of psoriasis with ustekinumab improved skin tightening in systemic sclerosis.
    Clin Exp Rheumatol 2017 Apr 19. Epub 2017 Apr 19.
    Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.
    Systemic sclerosis (SSc) is a chronic autoimmune disease characterised by fibrosis, inflammation and vasculopathy in the skin and internal organs. Recently, several articles described that Th17 cells, IL-23 and IL-17 levels were significantly elevated in the peripheral blood or fibrotic sites of SSc. We report a case of SSc and psoriasis administered ustekinumab, IL-12/IL-23 inhibitor. Read More

    Suppression of Th1 differentiation by tryptophan supplementation in vivo.
    Amino Acids 2017 Apr 18. Epub 2017 Apr 18.
    DKTK Clinical Cooperation Unit Neuroimmunology and Brain Tumor Immunology, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, Heidelberg, Germany.
    Metabolism of the essential amino acid tryptophan (trp) is a key endogenous immunosuppressive pathway restricting inflammatory responses. Tryptophan metabolites promote regulatory T cell (Treg) differentiation and suppress proinflammatory T helper cell (Th)1 and Th17 phenotypes. It has been shown that treatment with natural and synthetic tryptophan metabolites can suppress autoimmune neuroinflammation in preclinical animal models. Read More

    Deficient Adipogenesis of Scleroderma Patient and Healthy African American Monocytes.
    Front Pharmacol 2017 3;8:174. Epub 2017 Apr 3.
    Division of Rheumatology and Immunology, Department of Medicine, Medical University of South CarolinaCharleston, SC, USA.
    Monocytes from systemic sclerosis (SSc, scleroderma) patients and healthy African Americans (AA) are deficient in the regulatory protein caveolin-1 leading to enhanced migration toward chemokines and fibrogenic differentiation. While dermal fibrosis is the hallmark of SSc, loss of subcutaneous adipose tissue is a lesser-known feature. To better understand the etiology of SSc and the predisposition of AA to SSc, we studied the adipogenic potential of SSc and healthy AA monocytes. Read More

    Telemedicine for patients with rheumatic diseases: Systematic review and proposal for research agenda.
    Semin Arthritis Rheum 2017 Mar 22. Epub 2017 Mar 22.
    Chair of Rheumatology and Rheumatology Unit, University Clinic and AOU of Cagliari, SS 554-09 042 Monserrato, Cagliari, Italy.
    Objective: To systematically review the scientific literature regarding tele-rheumatology and draw conclusions about feasibility, effectiveness, and patient satisfaction.

    Methods: PubMed, Scopus, and Cochrane database searches were performed (April 2016) using relevant MeSH and keyword terms for telemedicine and rheumatic diseases. Articles were selected if reporting outcomes for feasibility, effectiveness, and patient satisfaction and methodologically appraised using the Cochrane Collaboration's tool for assessing risk of bias and a modified version of CONSORT 2010 Statement. Read More

    A review of the role and clinical utility of anti-Ro52/TRIM21 in systemic autoimmunity.
    Rheumatol Int 2017 Apr 17. Epub 2017 Apr 17.
    Western Health, Melbourne, VIC, Australia.
    Anti-Ro52/tripartite motif-containing 21 (TRIM21) is a ubiquitous antibody found in a number of systemic autoimmune conditions including Sjögren's syndrome, systemic lupus erythematosus and systemic sclerosis, appearing in about half of these patients. Once coupled with its closely related antibody, anti-Ro60 as the anti-SSA antibody, anti-Ro52 is emerging as a unique antibody with direct pathogenic disease involvement and distinct clinical properties. As a result, recent attention has turned to this antibody and its clinical associations and utility. Read More

    Primary Sjögren's syndrome with diffuse cystic lung changes developed systemic lupus erythematosus: a case report and literature review.
    Oncotarget 2017 Mar 8. Epub 2017 Mar 8.
    Department of Respiratory Medicine, Qilu Hospital of Shandong University, Jinan, China.
    Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease that can occur as a unique existence (primary Sjögren's syndrome) or merge with other systemic diseases like systemic lupus erythematosus (SLE), rheumatoid arthritis or systemic sclerosis (secondary Sjögren's syndrome). Data on the two diseases occurrence order are inadequate. Primary Sjögren's syndrome (pSS) may relatively uncommonly lead to diffuse cystic lung changes. Read More

    Systemic sclerosis.
    Lancet 2017 Apr 13. Epub 2017 Apr 13.
    University of Michigan, Ann Arbor, MI, USA.
    Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Read More

    Structural Validity of the Rheumatology Attitudes Index in Systemic Sclerosis: Analysis from the UCLA Scleroderma Quality of Life Study.
    J Rheumatol 2017 Apr 15. Epub 2017 Apr 15.
    From the San Diego State University (SDSU)/University of California (UC) San Diego Joint Doctoral Program in Clinical Psychology, Department of Psychology, San Diego; Department of Psychology, California State University, Dominguez Hills; SDSU, Department of Psychology, San Diego; David Geffen School of Medicine, University of California, Los Angeles (UCLA) School of Medicine, Los Angeles, California; University of Michigan Health System, Ann Arbor, Michigan, USA. Supported by the grant, Evaluation of Health-Related Quality of Life in Systemic Sclerosis from the Scleroderma Foundation Inc. Dr. Khanna has been funded by the US National Institutes of Health (NIH)/US National Institute of Arthritis and Musculoskeletal and Skin Diseases) grants K24 AR063120 and K23 AR053858. Dr. Khanna has served as a consultant for Bayer and Genentech. S. Gholizadeh, Doctoral Student, MS, MSc, MPH, SDSU/UC San Diego Joint Doctoral Program in Clinical Psychology, Department of Psychology; S.D. Mills, Doctoral Student, MS, MPH, SDSU/UC San Diego Joint Doctoral Program in Clinical Psychology, Department of Psychology; R.S. Fox, Postdoctoral Fellow, PhD, MPH, SDSU/UC San Diego Joint Doctoral Program in Clinical Psychology, Department of Psychology; E.L. Merz, Assistant Professor, PhD, MPH, California State University, Dominguez Hills, Department of Psychology; S.C. Roesch, Professor, PhD, SDSU, Department of Psychology; P.J. Clements, MD, Professor Emeritus, David Geffen School of Medicine, UCLA; S. Kafaja, MD, Clinical Instructor, David Geffen School of Medicine, UCLA; D.E. Furst, MD, Professor, David Geffen School of Medicine, UCLA; D. Khanna, MD, Professor, University of Michigan Health System; V.L. Malcarne, PhD, Professor, SDSU/UC San Diego Joint Doctoral Program in Clinical Psychology, and Department of Psychology, SDSU. Address correspondence to Professor V.L. Malcarne, Doctoral Training Facility, 6363 Alvarado Court, Suite 103, San Diego, California 92120- 4913, USA. E-mail: Accepted for publication February 7, 2017.
    Objective: To evaluate the structural validity of the Rheumatology Attitudes Index (RAI), a widely used measure of rheumatic disease-related helplessness in patients with systemic sclerosis (SSc).

    Methods: Patients with physician-confirmed SSc from the University of California, Los Angeles (UCLA) Scleroderma Quality of Life Study (n = 208) received clinical examinations and completed self-report questionnaires. The structural validity of the RAI was examined through confirmatory and exploratory factor analysis (CFA/EFA). Read More

    Original article: Varicella vaccination elicits a humoral and cellular response in children with rheumatic diseases using immune suppressive treatment.
    Vaccine 2017 Apr 12. Epub 2017 Apr 12.
    Laboratory for Translational Immunology, Paediatric Immunology, University Medical Center Utrecht, Utrecht, The Netherlands; Department of Paediatric Rheumatology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
    Objective: To assess humoral and cellular responses to live-attenuated varicella zoster virus (VZV) vaccination of patients with juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM) or juvenile scleroderma (JScle) compared to those of healthy controls (HC).

    Methods: Before, 4-6weeks and one year after VZV vaccination, blood samples of patients and HC were collected. VZV-specific antibody concentrations were measured by ELISA and multiplex immune-assay. Read More

    The epigenetic mechanism for discordance of autoimmunity in monozygotic twins.
    J Autoimmun 2017 Apr 12. Epub 2017 Apr 12.
    Department of Dermatology, Second Xiangya Hospital, Central South University, Hunan Key Laboratory of Medical Epigenomics, Changsha, Hunan, China. Electronic address:
    Monozygotic twins share an identical DNA sequence but are not truly "identical". In fact, when it comes to health and disease, they may often display some level of phenotypic discordance. The cause of this discordance is often unknown. Read More

    Toxic Memories in Systemic Sclerosis.
    J Invest Dermatol 2017 May;137(5):1003-1004
    Faculty of Health and Life Sciences, Immunology and Cell Biology Group, Northumbria University, Ellison Building, Newcastle Upon Tyne, UK. Electronic address:
    Systemic sclerosis is an autoimmune disease characterized by T-cell infiltration in the skin that leads to fibrosis, which can be life-limiting. Although T cells are important, it is not known which types mediate the fibrosis. The work presented by Li et al shows that memory CD8(+) cytotoxic T cells mediate fibrosis via the secretion of IL-13. Read More

    Leveraging Molecular Data Analysis to Understand Drug Response in Systemic Sclerosis.
    J Invest Dermatol 2017 May;137(5):1000-1002
    Vall d'Hebron Hospital Research Institute, Rheumatology Research Group, Barcelona, Spain. Electronic address:
    Taroni et al. integrate and analyze genomic information generated from different clinical trials on systemic sclerosis, a disease for which there is yet no Food and Drug Administration-approved therapy. By using a recent network analysis approach based on tissue-specific functionality, they leverage the biological information extracted from molecular profiling studies and report findings that could be useful for patient stratification. Read More

    Clinical significance of fibromyalgia syndrome in different rheumatic diseases: Relation to disease activity and quality of life.
    Reumatol Clin 2017 Apr 11. Epub 2017 Apr 11.
    Rheumatology Department, Faculty of Medicine, Cairo University, Egypt. Electronic address:
    Objective: To describe the frequencies of fibromyalgia syndrome (FMS) in various rheumatic diseases; rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and Behçets disease (BD) patients and to study the relation to clinical manifestations and quality of life (QoL).

    Patients And Methods: 160 patients (50 RA, 50 SLE, 30 SSc and 30 BD) and matched corresponding healthy controls were included. Disease activity was assessed using disease activity score in 28 joints (DAS28) for RA, SLE Disease Activity index (SLEDAI), modified Rodnan skin score for SSc and BD Current Activity Form (BDCAF). Read More

    Lung transplantation in systemic sclerosis: a single center cohort study.
    Joint Bone Spine 2017 Apr 10. Epub 2017 Apr 10.
    Lung Transplant Unit, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig de la Vall d'Hebron 119-129, 08035, Barcelona, Spain.
    Objective: Lung transplantation (LT) has been proposed as a treatment for advanced Interstitial Lung Disease (ILD) and/or Pulmonary Hypertension (PH) associated to Systemic Sclerosis (SSc) but few studies have been reported. The aim of this study was to describe the clinical features, complications and survival of a single-center cohort of patients with SSc that underwent LT and to compare their survival with a group of non-SSc transplanted patients.

    Methods: Fifteen patients with SSc were transplanted between May 2005 and April 2015. Read More

    Maintenance treatment of ANCA-associated vasculitides.
    Clin Exp Rheumatol 2017 Apr 5. Epub 2017 Apr 5.
    Department of Internal Medicine, Referral Centre for Rare Systemic and Autoimmune Diseases, Vasculitis and Scleroderma, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France.
    ANCA-associated vasculitis(AAV)covers a group of systemic necrotising vasculitides characterised by inflammation of small vessels, sometimes with granuloma, and associated with autoantibodies to neutrophil cytoplasmic proteases (proteinase-3 or myeloperoxidase). Potentially lethal if not promptly diagnosed and treated, AAV in most patients can be induced into remission with the current treatment modalities. However, the risk of relapse remains high, necessitating prolonged immunosuppressive or immunomodulating maintenance therapy, whose optimal duration remains undetermined. Read More

    Corrigendum: Agonistic Anti-PDGF Receptor Autoantibodies from Patients with Systemic Sclerosis Impact Human Pulmonary Artery Smooth Muscle Cells Function In Vitro.
    Front Immunol 2017 12;8:381. Epub 2017 Apr 12.
    Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche , Ancona , Italy.
    [This corrects the article on p. 75 in vol. 8, PMID: 28228756. Read More

    Evaluation of Autoantibodies in Patients with Primary and Secondary Sjogren's Syndrome.
    Open Rheumatol J 2017 31;11:10-15. Epub 2017 Jan 31.
    Immco Diagnostics / Trinity Biotech, Buffalo, NY, USA; Department of Oral Diagnostic Sciences, SUNY at Buffalo School of Dental Medicine, Buffalo, NY, USA.
    Background: Antibodies to salivary gland protein 1 (SP1), carbonic anhydrase 6 (CA6) and parotid secretory protein (PSP) were discovered in an animal model of Sjogren's syndrome (SS). Their expression was noted in patients with SS, especially those with lower focus scores on lip biopsies and those with early disease lacking antibodies to Ro and La.

    Objective: The current studies evaluated these autoantibodies in patients with long-standing SS expressing high levels of anti-Ro antibodies and in patients with Sjogren's syndrome secondary to systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). Read More

    Diverging effects of diabetes mellitus in patients with peripheral artery disease and abdominal aortic aneurysm and the role of advanced glycation end-products: ARTERY study - protocol for a multicentre cross-sectional study.
    BMJ Open 2017 Apr 11;7(4):e012584. Epub 2017 Apr 11.
    Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.
    Introduction: Diabetes mellitus is a well-defined risk factor for peripheral artery disease (PAD), but protects against the development and growth of abdominal aortic aneurysm (AAA). Diabetes mellitus is associated with arterial stiffening and peripheral arterial media sclerosis. Advanced glycation end-products (AGEs) are increased in diabetes mellitus and cardiovascular disease. Read More

    Protein Tyrosine Phosphatases in Systemic Sclerosis: Potential Pathogenic Players and Therapeutic Targets.
    Curr Rheumatol Rep 2017 May;19(5):28
    Division of Rheumatology, Allergy and Immunology, Department of Medicine, University of California, San Diego, 9500 Gilman Drive MC #0656, La Jolla, CA, 92093, USA.
    Purpose Of Review: The pathogenesis of systemic sclerosis depends on a complex interplay between autoimmunity, vasculopathy, and fibrosis. Reversible phosphorylation on tyrosine residues, in response to growth factors and other stimuli, critically regulates each one of these three key pathogenic processes. Protein tyrosine kinases, the enzymes that catalyze addition of phosphate to tyrosine residues, are known players in systemic sclerosis, and tyrosine kinase inhibitors are undergoing clinical trials for treatment of this disease. Read More

    Elevated levels of TL1A are associated with disease activity in patients with systemic sclerosis.
    Clin Rheumatol 2017 Apr 10. Epub 2017 Apr 10.
    Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
    TL1A is a member of the TNF superfamily. It performs significantly in the pathogenesis of rheumatic and autoimmune diseases partly through regulating the Th17 pathway. The clinical implication of circulating TL1A in patients with systemic sclerosis (SSc) remains unclear, and correlation between TL1A and Th17-related cytokines in the pathogenesis of SSc needs to be discussed. Read More

    Vitamin D supplementation in the prevention and management of major chronic diseases not related to mineral homeostasis in adults: research for evidence and a scientific statement from the European society for clinical and economic aspects of osteoporosis and osteoarthritis (ESCEO).
    Endocrine 2017 May 7;56(2):245-261. Epub 2017 Apr 7.
    Bone Metabolic Diseases Unit, Department of Surgery and Translational Medicine, University Hospital of Florence and University of Florence, Florence, Italy.
    Introduction: Optimal vitamin D status promotes skeletal health and is recommended with specific treatment in individuals at high risk for fragility fractures. A growing body of literature has provided indirect and some direct evidence for possible extraskeletal vitamin D-related effects.

    Purpose And Methods: Members of the European Society for Clinical and Economic Aspects of Osteoporosis and Osteoarthritis have reviewed the main evidence for possible proven benefits of vitamin D supplementation in adults at risk of or with overt chronic extra-skeletal diseases, providing recommendations and guidelines for future studies in this field. Read More

    Niclosamide: Beyond an antihelminthic drug.
    Cell Signal 2017 Apr 4. Epub 2017 Apr 4.
    Department of Medicine, Duke University Medical Center, Durham, NC 27710, United States.
    Niclosamide is an oral antihelminthic drug used to treat parasitic infections in millions of people worldwide. However recent studies have indicated that niclosamide may have broad clinical applications for the treatment of diseases other than those caused by parasites. These diseases and symptoms may include cancer, bacterial and viral infection, metabolic diseases such as Type II diabetes, NASH and NAFLD, artery constriction, endometriosis, neuropathic pain, rheumatoid arthritis, sclerodermatous graft-versus-host disease, and systemic sclerosis. Read More

    Importance of alternative-site blood glucose testing in the diagnosis of artifactual hypoglycaemia in systemic scleroderma.
    Diabetes Metab 2017 Apr 4. Epub 2017 Apr 4.
    Inserm UMRS 1166, diabetology-metabolism department, Sorbonne université, Pierre-et-Marie-Curie university (UPMC), Paris 6, institute of cardiometabolism and nutrition (ICAN), Pitié-Salpêtrière-Charles-Foix hospital, AP-HP, Paris, France. Electronic address:

    Novel insights into systemic autoimmune rheumatic diseases using shared molecular signatures and an integrative analysis.
    Epigenetics 2017 Apr 7:1-8. Epub 2017 Apr 7.
    a Lady Davis Research Institute , Montréal , QC , Canada.
    We undertook this study to identify DNA methylation signatures of three systemic autoimmune rheumatic diseases (SARDs), namely rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis, compared to healthy controls. Using a careful design to minimize confounding, we restricted our study to subjects with incident disease and performed our analyses on purified CD4(+) T cells, key effector cells in SARD. We identified differentially methylated (using the Illumina Infinium HumanMethylation450 BeadChip array) and expressed (using the Illumina TruSeq stranded RNA-seq protocol) sites between cases and controls, and investigated the biological significance of this SARD signature using gene annotation databases. Read More

    Lung Transplantation in Patients With Systemic Sclerosis.
    Curr Rheumatol Rep 2017 May;19(5):23
    Division of Rheumatology, University of California, San Francisco, 513 Parnassus Ave, Medical Science Building, S-847, San Francisco, CA, 94143, USA.
    Purpose Of Review: The purpose of this study was to highlight recent findings on evaluation for lung transplantation and outcomes after transplantation in patients with systemic sclerosis (scleroderma).

    Recent Findings: The recognition that extra-pulmonary disease manifestations can seriously compromise post-transplant outcomes has changed the way patients are screened for lung transplant candidacy. Reluctance to transplant subjects affected by scleroderma has been driven by the complexity and multisystem nature of this disease. Read More

    Geniposide inhibited endothelial-mesenchymal transition via the mTOR signaling pathway in a bleomycin-induced scleroderma mouse model.
    Am J Transl Res 2017 15;9(3):1025-1036. Epub 2017 Mar 15.
    Division of Rheumatology and Clinical Immunogenetics, Department of Internal Medicine, University of Texas Health Science Center at Houston Houston 77030, Texas, USA.
    Aim: Geniposide is an iridoid glycoside isolated from the gardenia plant. It has multiple biological activities. The roles of geniposide in systemic sclerosis (SSc) and in endothelial-to-mesenchymal transition (EndMT) are unclear. Read More

    Recent Advances and Challenges of mTOR Inhibitors Use in the Treatment of Patients with Tuberous Sclerosis Complex.
    Oxid Med Cell Longev 2017 12;2017:9820181. Epub 2017 Mar 12.
    Laboratory of Pharmacology & Experimental Therapeutics, Institute for Biomedical Imaging and Life Sciences (IBILI), Faculty of Medicine, University of Coimbra, Coimbra, Portugal; Centre for Neuroscience and Cell Biology-Institute for Biomedical Imaging and Life Sciences (CNC.IBILI) Research Consortium, University of Coimbra, Coimbra, Portugal.
    Tuberous sclerosis complex (TSC) is a genetic condition characterized by the presence of benign, noninvasive, and tumor-like lesions called hamartomas that can affect multiple organ systems and are responsible for the clinical features of the disease. In the majority of cases, TSC results from mutations in the TSC1 and TSC2 genes, leading to the overactivation of the mammalian target of rapamycin (mTOR) signalling pathway, which controls several cell functions, including cell growth, proliferation, and survival. The establishment of a connection between TSC and mTOR led to the clinical use of drugs known as mTOR inhibitors (like rapamycin, also known as sirolimus and everolimus), which are becoming an increasingly interesting tool in the management of TSC-associated features, such as subependymal giant cell astrocytomas, renal angiomyolipomas, and also epilepsy. Read More

    Infrared thermal imaging in connective tissue diseases.
    Reumatologia 2017 22;55(1):38-43. Epub 2017 Mar 22.
    Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.
    Infrared thermal imaging (IRT) is a non-invasive, non-contact technique which allows one to measure and visualize infrared radiation. In medicine, thermal imaging has been used for more than 50 years in various clinical settings, including Raynaud's phenomenon and systemic sclerosis. Imaging and quantification of surface body temperature provides an indirect measure of the microcirculation's overall performance. Read More

    Nailfold digital capillaroscopic findings in patients with diffuse and limited cutaneous systemic sclerosis.
    Reumatologia 2017 22;55(1):15-23. Epub 2017 Mar 22.
    Shiraz Geriatric Research Center, Shiraz University of Medical Sciences, Iran.
    Background: Systemic sclerosis (SSc) is a chronic disease with microvascular damage. Nailfold capillaroscopy is a non-invasive method used for evaluating capillaries in SSc. Its findings could be related to the internal organ involvement and SSc course. Read More

    Sirtuin 7 is Decreased in Pulmonary Fibrosis and Regulates the Fibrotic Phenotype of Lung Fibroblasts.
    Am J Physiol Lung Cell Mol Physiol 2017 Apr 6:ajplung.00473.2016. Epub 2017 Apr 6.
    University of Maryland School of Medicine.
    Pulmonary fibrosis is a severe condition with no cure and limited therapeutic options. Better understanding of its pathophysiology is needed. Recent studies have suggested that pulmonary fibrosis may be driven by accelerated aging-related mechanisms. Read More

    Ester-to-amide rearrangement of ethanolamine-derived prodrugs of sobetirome with increased blood-brain barrier penetration.
    Bioorg Med Chem 2017 May 23;25(10):2743-2753. Epub 2017 Mar 23.
    Program in Chemical Biology, Department of Physiology & Pharmacology, Oregon Health & Science University, 3181 SW Sam Jackson Park Rd, Portland, OR 97239, United States. Electronic address:
    Current therapeutic options for treating demyelinating disorders such as multiple sclerosis (MS) do not stimulate myelin repair, thus creating a clinical need for therapeutic agents that address axonal remyelination. Thyroid hormone is known to play an important role in promoting developmental myelination and repair, and CNS permeable thyromimetic agents could offer an increased therapeutic index compared to endogenous thyroid hormone. Sobetirome is a clinical stage thyromimetic that has been shown to have promising activity in preclinical models related to MS and X-linked adrenoleukodystrophy (X-ALD), a genetic disease that involves demyelination. Read More

    Retinal nerve fiber layer thickness and neuropsychiatric manifestations in systemic lupus erythematosus.
    Lupus 2017 Jan 1:961203317703496. Epub 2017 Jan 1.
    2 Department of Rheumatology, Tel-Aviv Sourasky Medical Center, Faculty of Medicine, Tel-Aviv University, Israel.
    Background Cognitive impairment is frequent in systemic lupus erythematosus. Atrophy of the corpus callosum and hippocampus have been reported in patients with systemic lupus erythematosus, and diffusion tensor imaging studies have shown impaired white matter integrity, suggesting that white matter damage in systemic lupus erythematosus may underlie the cognitive impairment as well as other neuropsychiatric systemic lupus erythematosus manifestations. Retinal nerve fiber layer thickness, as assessed by optical coherence tomography, has been suggested as a biomarker for white matter damage in neurologic disorders such as multiple sclerosis, Alzheimer's disease and Parkinson's disease. Read More

    TDP-43 and FUS en route from the nucleus to the cytoplasm.
    FEBS Lett 2017 Apr 5. Epub 2017 Apr 5.
    BioMedical Center (BMC), Ludwig-Maximilians-University Munich, 82152, Planegg-Martinsried, Germany.
    Misfolded or mislocalized RNA-binding proteins (RBPs) and, consequently, altered mRNA processing, can cause neuronal dysfunction, eventually leading to neurodegeneration. Two prominent examples are the RBPs TDP-43 (TAR DNA binding protein of 43 kDa) and FUS (Fused in sarcoma), which form pathological mRNP aggregates in patients suffering from amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), two devastating neurodegenerative disorders. Here, we review the multiple functions of TDP-43 and FUS in mRNA processing, both in the nucleus and in the cytoplasm. Read More

    Aerobic and resistance exercise in systemic sclerosis: State of the art.
    Musculoskeletal Care 2017 Apr 5. Epub 2017 Apr 5.
    Department of Neurobiology, Care Sciences and Society, Division of Physiotherapy, Karolinska Institutet, and, Functional Area Occupational Therapy & Physiotherapy, Allied Health Professionals Function, Karolinska University Hospital, Stockholm, Sweden.
    Introduction: Patients with systemic sclerosis (SSc) experience reduced exercise capacity and muscle strength compared with healthy subjects. There are also indications of reduced levels of physical activity.

    Objective: To present the current knowledge of physical exercise in SSc. Read More

    Systemic frequencies of T helper 1 and T helper 17 cells in patients with age-related macular degeneration: A case-control study.
    Sci Rep 2017 Apr 4;7(1):605. Epub 2017 Apr 4.
    Clinical Eye Research Unit, Department of Ophthalmology, Zealand University Hospital, Roskilde, Denmark.
    Age-related macular degeneration (AMD) is a degenerative disease of the retina and a leading cause of irreversible vision loss. We investigated the systemic differences in the frequency of T helper (Th) 1 and Th17 cells in patients with non-exudative and exudative AMD and compared to age-matched controls. Flow cytometry was used to determine the systemic frequency of Th1 (CD4(+)CXCR3(+)IL12RB2(+)) and Th17 (CD4(+)CCR6(+)IL23R(+)) cells, and percentage of CD4(+) T-cells expressing CXCR3, IL12RB2, CCR6, IL23R, and co-expressing CXCR3 and CCR6. Read More

    Mycophenolate versus Placebo for Systemic Sclerosis-Related Interstitial Lung Disease: An Analysis of Scleroderma Lung Studies I and II.
    Arthritis Rheumatol 2017 Apr 4. Epub 2017 Apr 4.
    Department of Biomathematics, University of California, Los Angeles, USA.
    Objective: To compare mycophenolate (MMF) with placebo for the treatment of systemic sclerosis-related interstitial lung disease (SSc-ILD).

    Methods: Participants enrolled in the placebo arm of Scleroderma Lung Study (SLS) I and the MMF arm of SLS II were included. SLS I randomized participants to oral cyclophosphamide (CYC) versus placebo for 1 year, while SLS II randomized participants to MMF for 2 years versus oral CYC for 1 year followed by 1 year of placebo. Read More

    Fatigue in patients with systemic sclerosis and hypothyroidism. A review of the literature and report of our experience.
    Clin Exp Rheumatol 2017 Apr 4. Epub 2017 Apr 4.
    Department of Medical, Surgical, Maternal, Paediatric and Adult Sciences, University of Modena & Reggio Emilia, Modena, Italy.
    Persistent fatigue (defined as ongoing exhaustion, disproportionate to exertion and not adequately alleviated by rest) reduces health-related quality of life of systemic sclerosis (SSc) patients. Fatigue in SSc is associated with reduced capacity to carry out daily activities, work disability and impaired physical function. Clinical studies demonstrated a high prevalence of autoimmune thyroiditis and hypothyroidism in patients with SSc. Read More

    UVA1 for diffuse cutaneous systemic sclerosis in a Fitzpatrick skin type VI patient: outcomes in the modified Rodnan skin score.
    Acta Reumatol Port 2017 Jan 31. Epub 2017 Jan 31.
    Centro Hospitalar e Universitário de Coimbra.
    Introduction: Cutaneous sclerosis can lead to important mobility impairment. Ultraviolet (UV) A1 phototherapy may improve skin sclerosis, although most of the studies have been with Caucasian patients.

    Material And Methods: A 44-year-old patient, Fitzpatrick skin type VI, was being followed up with the diagnosis of diffuse cutaneous systemic sclerosis. Read More

    Placental Stromal Cell Therapy for Experimental Autoimmune Encephalomyelitis: The Role of Route of Cell Delivery.
    Stem Cells Transl Med 2017 Apr 29;6(4):1286-1294. Epub 2016 Sep 29.
    Laboratory of Radiobiology and Biotechnology, Sharett Institute of Oncology, Hadassah Hebrew University Medical Center, Jerusalem, Israel.
    Multiple sclerosis (MS) is an immune-mediated disease of the central nervous system (CNS) with no effective treatment available for the chronic-progressive stage. Cell therapy is a promising therapeutic approach for attenuating the immune-mediated CNS process. Isolated and expanded human placental stromal cells (hPSCs) possess potent immunomodulatory and trophic properties, making them a good candidate for MS therapy. Read More

    The impact of transcriptional factor Fli1 deficiency on the regulation of angiogenesis.
    Exp Dermatol 2017 Mar 31. Epub 2017 Mar 31.
    Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan.
    The insufficiency of Friend leukemia virus integration 1 (Fli1), a member of the Ets family transcription factors, is implicated in the pathogenesis of vasculopathy associated with systemic sclerosis (SSc). Fli1 deficiency accelerates early steps of angiogenesis, including detachment of pre-existing pericytes and extracellular matrix degradation by endothelial proteinases, but the impact of Fli1 deficiency on the other steps of angiogenesis has not been investigated. Therefore, we evaluated the effect of Fli1 deficiency on migration, proliferation, cell survival, and tube formation of human dermal microvascular endothelial cells (HDMECs). Read More

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