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lung activity disease systemic sclerosis

Steven O'Reilly
Fibrosis is a common and important disease. It is a pathological state due to excessive scar formation mediated by an increase in activated fibroblasts that express alpha smooth muscle actin and copious amounts of extracellular matrix molecules. Epigenetics is an area of research that encompasses three main mechanisms: methylation, histone modifications to the tails of histones and also non-coding RNAs including long and short non-coding RNAs. These three mechanisms all seek to regulate gene expression without a change in the underlying DNA sequence...
October 6, 2016: Molecular Aspects of Medicine
Laura Groseanu, Violeta Bojinca, Tania Gudu, Ioana Saulescu, Denisa Predeteanu, Andra Balanescu, Florian Berghea, Daniela Opris, Andreea Borangiu, Cosmin Constantinescu, Magda Negru, Ruxandra Ionescu
OBJECTIVE: Vitamin D has pleiotropic effects including immunomodulatory, cardioprotective, and antifibrotic properties and is thus able to modulate the three main links in scleroderma pathogenesis. The aim of the study was to evaluate the level of vitamin D in patients with systemic sclerosis and to analyze the associations between the concentration of vitamin D and the features of systemic sclerosis. MATERIAL AND METHODS: Fifty-one consecutive patients were evaluated for visceral involvement, immunological profile, activity, severity scores, and quality of life...
June 2016: Eur J Rheumatol
Barna Vasarhelyi, Gergely Toldi, Attila Balog
The assessment of the general inflammatory condition of patients with autoimmune connective tissue disorders (ACTD) is a major challenge. The use of traditional inflammatory markers including CRP-levels and erythrocyte sedimentation rate (ESR) is limited by several preanalytical factors and their low specificities. Soluble urokinase plasminogen activator receptor (suPAR) is one of the novel candidate markers that is increasingly used in immune mediated disorders. In our studies we compared suPAR levels of patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and ankylosing spondylitis with those of healthy controls...
April 2016: EJIFCC
Mutlay Sayan, Brooke T Mossman
The concept of the inflammasome, a macromolecular complex sensing cell stress or danger signals and initiating inflammation, was first introduced approximately a decade ago. Priming and activation of these intracellular protein platforms trigger the maturation of pro-inflammatory chemokines and cytokines, most notably, interleukin-1β (IL-1β) and IL-18, to promulgate innate immune defenses. Although classically studied in models of gout, Type II diabetes, Alzheimer's disease, and multiple sclerosis, the importance and mechanisms of action of inflammasome priming and activation have recently been elucidated in cells of the respiratory tract where they modulate the responses to a number of inhaled pathogenic particles and fibres...
2016: Particle and Fibre Toxicology
Patryk J Woytala, Ewa Morgiel, Anna Łuczak, Katarzyna Czesak-Woytala, Piotr Wiland
BACKGROUND: The therapeutic effects of cyclophosphamide (CP) in the treatment of systemic rheumatic diseases are related to its immune suppressive activity. However effective, the application of CP is restricted due to multiple adverse effects. OBJECTIVES: This retrospective study was conducted to determine the frequency of adverse effects attributed to CP toxicity. MATERIAL AND METHODS: The study involved 65 patients (17 male; 48 female) receiving intravenous CP between October 2007 and December 2010...
May 2016: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Lazaros I Sakkas
Systemic sclerosis (SSc) is a multisystem disease characterized by extensive collagen deposition in skin and internal organs, fibrointimal microvasculopathy, and activation of the immune system. T cells and B cells can promote fibrosis in SSc. Interleukin (IL)-6 is implicated in the pathogenesis of SSc. IL-6 is increased in the peripheral blood and lesional skin from patients with SSc, and induces fibroblast collagen production directly and indirectly by inducing profibrotic M2 macrophages. IL-6 also induces Th17 differentiation and promotes B cell differentiation toward Ig-producing plasma cells...
2016: Drug Design, Development and Therapy
Gabriele Valentini, Michele Iudici, Ulrich A Walker, Veronika K Jaeger, Murray Baron, Patricia Carreira, László Czirják, Christopher P Denton, Oliver Distler, Eric Hachulla, Ariane L Herrick, Otylia Kowal-Bielecka, Janet Pope, Ulf Müller-Ladner, Gabriela Riemekasten, Jerome Avouac, Marc Frerix, Suzana Jordan, Tünde Minier, Elise Siegert, Voon H Ong, Serena Vettori, Yannick Allanore
BACKGROUND: Validity of European Scleroderma Study Group (EScSG) activity indexes currently used to assess disease activity in systemic sclerosis (SSc) has been criticised. METHODS: Three investigators assigned an activity score on a 0-10 scale for 97 clinical charts. The median score served as gold standard. Two other investigators labelled the disease as inactive/moderately active or active/very active. Univariate-multivariate linear regression analyses were used to define variables predicting the 'gold standard', their weight and derive an activity index...
September 12, 2016: Annals of the Rheumatic Diseases
Florence Morin, Niloufar Kavian, Carole Nicco, Olivier Cerles, Christiane Chéreau, Frédéric Batteux
Systemic sclerosis (SSc) is a connective tissue disorder characterized by fibrosis of the skin and inner organs, vasculopathy, and immunological abnormalities. Recent insights on the implication of STAT3, AKT, and Wnt/β-catenin in fibrosis have prompted us to investigate, in a mouse model of ROS-induced SSc, the effects of niclosamide, an antihelmintic drug that inhibits both of these signaling pathways. SSc was induced in BALB/c mice by daily s.c. injections of hypochlorous acid (HOCl). Mice were treated or not every other day, 5 d a week, for 6 wk, by niclosamide...
September 9, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
N Del Papa, F Onida, E Zaccara, G Saporiti, W Maglione, E Tagliaferri, R Andracco, D Vincenti, T Montemurro, L Mircoli, C Vitali, A Cortelezzi
We retrospectively evaluated the efficacy of autologous hematopoietic stem cell transplantation (AHSCT) in 18 patients with rapidly progressive diffuse cutaneous systemic sclerosis (rp-dcSSc), and compared their disease outcomes with those of 36 demographically- and clinically-matched patients treated with conventional therapies. Cutaneous involvement, by performing modified Rodnan skin score (mRss), lung diffusion capacity, by measuring diffusing capacity of lung for carbon monoxide (DLCO), and disease activity, by applying the European Scleroderma Study Group (ESSG) scoring system, were the outcome variables measured at the baseline time and then every 12 months for the following 60 months in both the AHSCT-treated patients and the control group...
August 22, 2016: Bone Marrow Transplantation
Ryosuke Saigusa, Yoshihide Asano, Kouki Nakamura, Takashi Yamashita, Yohei Ichimura, Takehiro Takahashi, Tetsuo Toyama, Takashi Taniguchi, Ayumi Yoshizaki, Miki Miyazaki, Zenshiro Tamaki, Shinichi Sato
OBJECTIVES: Intravenous pulse cyclophosphamide (IVCY) is the first-line treatment for systemic sclerosis-associated interstitial lung disease (SSc-ILD). So far, there is no useful predictive marker for IVCY efficacy against SSc-ILD, although potential candidates are parameters reflecting vascular activation. Since plasma levels of plasmin-α2-plasmin inhibitor complex (PIC) serve as a potential biomarker of SSc vasculopathy, we evaluated the usefulness of plasma PIC levels as an indicator for IVCY efficacy against SSc-ILD...
August 19, 2016: Modern Rheumatology
Adriana Morales-Cárdenas, Camila Pérez-Madrid, Liliana Arias, Paulina Ojeda, María Paula Mahecha, Adriana Rojas-Villarraga, Jorge A Carrillo-Bayona, Juan-Manuel Anaya
Systemic sclerosis (SSc) is a multi-systemic autoimmune disease that mainly affects the skin, lungs, gastrointestinal tract, heart and kidneys. Pulmonary disease in patients with SSc is strongly associated with mortality. The mechanisms involved into its pathophysiology include the activation of autoimmune cells and hyperplasia of fibroblasts with an increased capacity to produce collagen and diminished collagen breakdown. Although pulmonary biopsy is the gold standard for the diagnosis of interstitial lung disease in SSc, the most commonly used method is high-resolution computed tomography due to its high sensitivity and specificity...
August 4, 2016: Autoimmunity Reviews
Muriel Elhai, Jérôme Avouac, Anna Maria Hoffmann-Vold, Nadira Ruzehaji, Olivia Amiar, Barbara Ruiz, Hassina Brahiti, Matthieu Ponsoye, Maxime Fréchet, Anne Burgevin, Sonia Pezet, Jérémy Sadoine, Thomas Guilbert, Carole Nicco, Hisaya Akiba, Vigo Heissmeyer, Arun Subramaniam, Robert Resnick, Øyvind Molberg, André Kahan, Gilles Chiocchia, Yannick Allanore
Treatment for fibrosis represents a critical unmet need, because fibrosis is the leading cause of death in industrialized countries, and there is no effective therapy to counteract the fibrotic process. The development of fibrosis relates to the interplay between vessel injury, immune cell activation, and fibroblast stimulation, which can occur in various tissues. Immunotherapies have provided a breakthrough in the treatment of immune diseases. The glycoprotein OX40-OX40 ligand (OX40L) axis offers the advantage of a targeted approach to costimulatory signals with limited impact on the whole immune response...
July 5, 2016: Proceedings of the National Academy of Sciences of the United States of America
Siri Opsahl Hetlevik, Berit Flatø, Marite Rygg, Ellen Berit Nordal, Cathrine Brunborg, Helene Hetland, Vibke Lilleby
OBJECTIVES: To describe the characteristics, outcome and predictive factors of juvenile mixed connective tissue disease (JMCTD) in a nationwide cohort of patients. METHODS: We examined 55 patients with JMCTD after a mean disease duration of 16.2 years (SD 10.0). Patients were registered according to Kasukawa's criteria. Remission criteria were defined according to those for juvenile idiopathic arthritis, plus absence of cytopenia, myositis, progressive sclerodactyly, lung and oesophageal manifestations...
June 9, 2016: Annals of the Rheumatic Diseases
Alexandra Maria Giovanna Brunasso, Cesare Massone
In systemic sclerosis (SSc), the development of fibrosis seems to be a consequence of the initial ischemic process related to an endothelial injury. The initial trigger event in SSc is still unknown, but circulating progenitor cells (CPCs) might play a key role. Such cells have the ability to traffic into injury sites, exhibiting inflammatory features of macrophages, tissue remodeling properties of fibroblasts, and vasculogenesis functions of endothelial cells. The different subsets of CPCs described thus far in SSc arise from a pool of circulating monocyte precursors (CD14 (+) cells) and probably correspond to a different degree of differentiation of a single cell of origin...
2016: F1000Research
Monica Lucattelli, Silvia Fineschi, Enrico Selvi, Estrella Garcia Gonzalez, Barbara Bartalesi, Giovanna De Cunto, Sauro Lorenzini, Mauro Galeazzi, Giuseppe Lungarella
BACKGROUND: Ajulemic acid (AjA) is a synthetic analogue of tetrahydrocannabinol that can prevent and limit progression of skin fibrosis in experimental systemic sclerosis. In this study we investigated whether AjA also prevents and modulates lung fibrosis induced by bleomycin (BLM) when administered in mice during the inflammatory or the fibrogenic phase of the model. METHODS: The anti-inflammatory and antifibrotic efficacy of AjA was evaluated in DBA/2 mice treated orally once a day starting either at day 0 (preventive treatment) or at day 8 (therapeutic treatment) after a single intratracheal instillation of BLM...
May 6, 2016: Respiratory Research
T Takahashi, Y Asano, K Nakamura, T Yamashita, R Saigusa, Y Ichimura, T Toyama, T Taniguchi, A Yoshizaki, Z Tamaki, Y Tada, M Sugaya, T Kadono, S Sato
BACKGROUND: LL-37 is an antimicrobial peptide with pleiotropic effects on immune system, angiogenesis, and tissue remodeling, which are cardinal pathological events in systemic sclerosis (SSc). OBJECTIVES: To elucidate the potential role of LL-37 in SSc. METHODS: The expression of target molecules was evaluated by immunostaining and quantitative reverse transcription-real time PCR in human and murine skin. The mechanisms regulating LL-37 expression in endothelial cells were examined by gene silencing technique and chromatin immunoprecipitation...
April 23, 2016: British Journal of Dermatology
Catherine H Miele, Kristin Schwab, Rajeev Saggar, Erin Duffy, David Elashoff, Chi-Hong Tseng, Sam Weigt, Deepshikha Charan, Fereidoun Abtin, Jimmy Johannes, Ariss Derhovanessian, Jeffrey Conklin, Kevin Ghassemi, Dinesh Khanna, Osama Siddiqui, Abbas Ardehali, Curtis Hunter, Murray Kwon, Reshma Biniwale, Michelle Lo, Elizabeth Volkmann, David Torres Barba, John A Belperio, David Sayah, Thomas Mahrer, Daniel E Furst, Suzanne Kafaja, Philip Clements, Michael Shino, Aric Gregson, Bernard Kubak, Joseph P Lynch, David Ross, Rajan Saggar
RATIONALE: Consideration of lung transplantation in patients with systemic sclerosis (SSc) remains guarded, often due to the concern for esophageal dysfunction and the associated potential for allograft injury and suboptimal post-lung transplantation outcomes. OBJECTIVES: The purpose of this study was to systematically report our single-center experience regarding lung transplantation in the setting of SSc, with a particular focus on esophageal dysfunction. METHODS: We retrospectively reviewed all lung transplants at our center from January 1, 2000 through August 31, 2012 (n = 562), comparing the SSc group (n = 35) to the following lung transplant diagnostic subsets: all non-SSc (n = 527), non-SSc diffuse fibrotic lung disease (n = 264), and a non-SSc matched group (n = 109)...
June 2016: Annals of the American Thoracic Society
Kenneth Michael Pollard
Inhalation of dust containing crystalline silica is associated with a number of acute and chronic diseases including systemic autoimmune diseases. Evidence for the link with autoimmune disease comes from epidemiological studies linking occupational exposure to crystalline silica dust with the systemic autoimmune diseases systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis. Although little is known regarding the mechanism by which silica exposure leads to systemic autoimmune disease, there is a voluminous literature on silica exposure and silicosis that may help identify immune processes that precede development of autoimmunity...
2016: Frontiers in Immunology
Marta Cossu, Romina Andracco, Alessandro Santaniello, Maurizio Marchini, Adriana Severino, Monica Caronni, Timothy Radstake, Lorenzo Beretta
OBJECTIVE: To improve knowledge of vasculopathy in SSc through the assessment of serum levels of circulating angiogenetic and endothelial dysfunction markers in patients at different stages of the disease. METHODS: Sera from 224 subjects were obtained and concentrations of angiopoietin-2, chemokine (C-X-C motif) ligand (CXCL)-16 (CXCL16), E-selectin, soluble intercellular adhesion molecule-1, IL-8 (CXCL8), soluble vascular adhesion molecule-1 and VEGF were determined by a Luminex assay...
June 2016: Rheumatology
D M Wuttge, A Andreasson, E Tufvesson, Å C M Johansson, A Scheja, T Hellmark, R Hesselstrand, L Truedsson
OBJECTIVES: In systemic sclerosis (SSc)-related interstitial lung disease (ILD), elevated eosinophil counts in bronchoalveolar lavage are associated with a worse outcome. We hypothesized that eosinophils may be activated in the peripheral circulation, thereby increasing their recruitment to affected tissues and contributing to inflammation and fibrosis. The aim of this study was to characterize the blood eosinophils in SSc patients. METHOD: Expression levels of surface markers CD11b, CD44, CD48, CD54, CD69, CD81, and HLA-DR on CD16(low)CD9(high)-expressing eosinophils were measured by flow cytometry in whole blood from SSc patients (n = 32) and controls (n = 11)...
2016: Scandinavian Journal of Rheumatology
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