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lung activity disease systemic sclerosis

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https://www.readbyqxmd.com/read/28919455/is-there-a-role-for-il-17-in-the-pathogenesis-of-systemic-sclerosis
#1
REVIEW
Carlo Chizzolini, Aleksandra Maria Dufour, Nicolò Costantino Brembilla
In systemic sclerosis (SSc) immuno-inflammatory events are central to disease development. Amongst other mediators of inflammation, interleukin 17 (IL-17) and Th17 cells have been reported to be increased in the peripheral blood and target organs including involved skin in SSc. They participate and amplify inflammatory responses by inducing the production of cytokines such as IL-6, chemokines such as CCL2 and CXCL8 (IL-8), matrix metalloproteinases-1, -2, -9 and the expression of adhesion molecules in stromal cells including fibroblasts and endothelial cells...
September 14, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28891418/two-years-follow-up-of-an-open-label-pilot-study-of-treatment-with-rituximab-in-patients-with-early-diffuse-cutaneous-systemic-sclerosis
#2
Karin Melsens, Els Vandecasteele, Ellen Deschepper, Valérie Badot, Daniel Blockmans, Guy Brusselle, Ellen De Langhe, Michel De Pauw, Claire Debusschere, Saskia Decuman, Liselotte Deroo, Frédéric Houssiau, Jan Lenaerts, Yves Piette, Kristof Thevissen, Marie Vanthuyne, René Westhovens, Sara Wijnant, Filip De Keyser, Vanessa Smith
OBJECTIVES: Following results in open-label studies of rituximab in patients with systemic sclerosis, a Belgian three-centre initiative was launched to explore safety and efficacy of rituximab in early, diffuse cutaneous systemic sclerosis (dcSSc). METHODS: Open-label study of 17 patients with early dcSSc, treated with two courses of rituximab, at month 0 and 6. Clinical examination, lung function testing, echocardiography, disease activity score (DAS) and functional status were performed at baseline and over 24 months of follow-up...
September 11, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28831595/juvenile-systemic-sclerosis-experience-from-a-tertiary-care-center-from-india
#3
Narendra Kumar Bagri, Dinesh Raj, Jasmeet Kaur, Harish Punia, Isha Saini, Rakesh Lodha, S K Kabra
Juvenile systemic sclerosis (JSSc) is a rare disorder with paucity of information on its treatment and longterm  outcome. Herein, we are sharing our experience with this rare entity. Case records of children, diagnosed to have systemic sclerosis attending Pediatric Rheumatology Clinic at All India Institute of Medical Sciences, New Delhi from January 1998 to June 2016 were reviewed. The demographic, clinical, laboratory, treatment and outcome details were recorded. Disease outcome was classified arbitrarily as controlled, partly controlled or non-responsive/progressive based on: (A) ability to perform activities of daily life (ADL) and (B) presence or absence of musculoskeletal symptoms, skin changes (ulceration/progressive digital pitting/gangrene), and visceral organ involvement (dyspahgia, cardiopulmonary symptoms)...
August 22, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28814429/nintedanib-inhibits-macrophage-activation-and-ameliorates-vascular-and-fibrotic-manifestations-in-the-fra2-mouse-model-of-systemic-sclerosis
#4
Jingang Huang, Christiane Maier, Yun Zhang, Alina Soare, Clara Dees, Christian Beyer, Ulrike Harre, Chih-Wei Chen, Oliver Distler, Georg Schett, Lutz Wollin, Jörg H W Distler
BACKGROUND: Nintedanib is an inhibitor targeting platelet-derived growth factor receptor, fibroblast growth factor receptor and vascular endothelial growth factor receptor tyrosine kinases that has recently been approved for the treatment of idiopathic pulmonary fibrosis. The aim of this study was to analyse the effects of nintedanib in the fos-related antigen-2 (Fra2) mouse model of systemic sclerosis (SSc). METHODS: The effects of nintedanib on pulmonary arterial hypertension with proliferation of pulmonary vascular smooth muscle cells (PVSMCs) and luminal occlusion, on microvascular disease with apoptosis of microvascular endothelial cells (MVECs) and on fibroblast activation with myofibroblast differentiation and accumulation of extracellular matrix were analysed...
August 16, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28730919/an-update-on-biomarker-discovery-and-use-in-systemic-sclerosis
#5
Takashi Matsushita, Kazuhiko Takehara
Systemic sclerosis (SSc) is an autoimmune disease characterized by excessive extracellular matrix deposition in the skin and internal organs. Three major abnormalities, autoimmunity, vasculopathy, and fibrosis, are considered to play important roles in the pathophysiology of SSc. SSc is a heterogeneous disease with clinical features, disease progress, therapeutic response, and prognosis. Therefore, identification of biomarkers, which can predict the course of the disease, is required for the progress of clinical practice...
July 25, 2017: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/28727155/mucosal-associated-invariant-mait-cells-are-deficient-in-systemic-sclerosis
#6
Arsène Mekinian, Thibaut Mahevas, Mohamad Mohty, Vincent Jachiet, Sébastien Rivière, Olivier Fain, Béatrice Gaugler
OBJECTIVES: Systemic sclerosis (SSc) is an autoimmune disease, characterized by fibrosis of the skin and other organs, vascular impairment and deficient immune responses. Mucosal associated invariant T cells (MAIT) cells have been involved in various inflammatory and autoimmune diseases. The aim of this study was to determine the frequencies of MAIT cells in the blood of patients with systemic sclerosis (SSc), and to compare their distribution in the different types of SSc. PATIENTS AND METHODS: Blood samples from SSc patients and healthy controls were examined by flow cytometer to analyze the frequencies of MAIT and γδ T cells...
July 18, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/28664834/design-of-a-randomised-placebo-controlled-clinical-trial-of-nintedanib-in-patients-with-systemic-sclerosis-associated-interstitial-lung-disease-senscis%C3%A2
#7
Oliver Distler, Kevin K Brown, Jörg H W Distler, Shervin Assassi, Toby M Maher, Vincent Cottin, John Varga, Carl Coeck, Martina Gahlemann, Wiebke Sauter, Hendrik Schmidt, Kristin B Highland
OBJECTIVES: Nintedanib is a tyrosine kinase inhibitor approved for the treatment of idiopathic pulmonary fibrosis (IPF). The pathological pathways involved in fibrogenesis in IPF and interstitial lung disease associated with systemic sclerosis (SSc-ILD) show commonalities; both involve fibroblast activation, myofibroblast accumulation and deposition of extracellular matrix. The SENSCIS™ trial is a randomised, placebo-controlled Phase III trial that will evaluate the efficacy and safety of nintedanib in patients with SSc-ILD (NCT02597933)...
June 29, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28664832/clinical-determinants-of-elevated-systolic-pulmonary-artery-pressure-measured-by-transthoracic-doppler-echocardiography-in-early-systemic-sclerosis
#8
Patricia E Carreira, Loreto Carmona, Beatriz E Joven, Estibaliz Loza, Jose Luis Andreu, Gabriela Riemekasten, Serena Vettori, Yannick Allanore, Alexandra Balbir-Gurman, Paolo Airò, Ulrich A Walker, Nemanja Damjanov, Lidia P Ananieva, Simona Rednic, László Czirják, Oliver Distler, Dominique Farge, Roger Hesselstrand, Ada Corrado, Paola Caramaschi, Mohammed Tikly, Marco Matucci-Cerinic
OBJECTIVES: To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Doppler-echocardiography (TTE) in patients with early systemic sclerosis (SSc). METHODS: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research (EUSTAR) database was performed. SSc patients with <3 years from the first non-Raynaud's phenomenon (RP) symptom at baseline EUSTAR visit, were selected...
June 20, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28631694/-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
#9
M Yu Krylov, L P Ananyeva, О А Koneva, M N Starovoytova, O V Desinova, O B Ovsyannikova, E N Aleksandrova, A A Novikov, I A Guseva, N V Konovalova, D A Varlamov
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. RESULTS: The carriers of the T allele showed a statistically significant association with SSC, a diffuse form (DF), the presence of interstitial lung disease (ILD), cardiac injury (CI), and seropositivity for anti-topoisomerase I antibodies (ATA)...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28432700/serum-h-ficolin-levels-clinical-association-with-interstitial-lung-disease-in-patients-with-systemic-sclerosis
#10
Takuya Miyagawa, Yoshihide Asano, Yuka de Mestier, Ryosuke Saigusa, Takashi Taniguchi, Takashi Yamashita, Kouki Nakamura, Megumi Hirabayashi, Shunsuke Miura, Yohei Ichimura, Takehiro Takahashi, Ayumi Yoshizaki, Tomomitsu Miyagaki, Makoto Sugaya, Shinichi Sato
Ficolins, a group of oligomeric lectins consisting of three isoforms (H-, L- and M-ficolin), contribute to innate immunity via activating the complement pathway and/or acting directly as opsonins against pathogens and apoptotic cells. Because apoptotic cells likely drive the development of systemic sclerosis (SSc) partly through innate immunity, we assessed the clinical association of serum H-ficolin levels in SSc patients. Despite no difference in serum H-ficolin levels between SSc and control subjects, SSc patients with decreased serum H-ficolin levels tended to have a higher prevalence of interstitial lung disease (ILD)...
April 22, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28385812/sirtuin-7-is-decreased-in-pulmonary-fibrosis-and-regulates-the-fibrotic-phenotype-of-lung-fibroblasts
#11
Anne E Wyman, Zahid Noor, Rita Fishelevich, Virginia Lockatell, Nirav G Shah, Nevins W Todd, Sergei P Atamas
Pulmonary fibrosis is a severe condition with no cure and limited therapeutic options. A better understanding of its pathophysiology is needed. Recent studies have suggested that pulmonary fibrosis may be driven by accelerated aging-related mechanisms. Sirtuins (SIRTs), particularly SIRT1, SIRT3, and SIRT6, are well-known mediators of aging; however, limited data exist on the contribution of sirtuins to lung fibrosis. We assessed the mRNA and protein levels of all seven known sirtuins in primary lung fibroblasts from patients with idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) in comparison with lung fibroblasts from healthy controls...
June 1, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28365359/leflunomide-prevents-ros-induced-systemic-fibrosis-in-mice
#12
Florence Morin, Niloufar Kavian, Sandrine Chouzenoux, Olivier Cerles, Carole Nicco, 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 into the polarization of macrophages in scleroderma and into the implication of STAT6 and KLF4 in this process have prompted us to investigate the effects of the inhibition of STAT6 signaling pathway by leflunomide in mice. SSc was induced in BALB/c mice by daily subcutaneous injections of hypochlorous acid (HOCl) or bleomycin. Mice were treated (or not) every other day, for 4 or 6 weeks, by leflunomide...
March 30, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28341058/anti-fibrotic-effects-of-soluble-guanylate-cyclase-stimulators-and-activators-a-review-of-the-preclinical-evidence
#13
Peter Sandner, Johannes Peter Stasch
It is now well established that the NO-sGC-cGMP signal transduction system mediates many different physiological functions in almost every conceivable organ system; this has been best characterized in the cardiovascular system where NO-driven cGMP production exerts a plethora of cytoprotective and anti-atherogenic effects, including dilatation, inhibition of vascular smooth muscle proliferation, blockade of leukocyte recruitment, and anti-platelet activity. Accordingly, dysfunctional NO-sGC-cGMP mediated signaling is perceived as the underlying pathophysiological cause of many cardiovascular and non-cardiovascular diseases...
January 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28340090/predictive-value-of-european-scleroderma-group-activity-index-in-an-early-scleroderma-cohort
#14
Tatiana Nevskaya, Murray Baron, Janet E Pope
Objective: To estimate the effect of disease activity, as measured by the European Scleroderma Research Group Activity Index (EScSG-AI), on the risk of subsequent organ damage in a large systemic sclerosis (SSc) cohort. Methods: Of 421 SSc patients from the Canadian Scleroderma Research Group database with disease duration of ⩽ 3 years, 197 who had no evidence of end-stage organ damage initially and available 3 year follow-up were included. Disease activity was assessed by the EScSG-AI with two variability measures: the adjusted mean EScSG-AI (the area under the curve of the EScSG-AI over the observation period) and persistently active disease/flare...
July 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28330499/a-novel-multi-network-approach-reveals-tissue-specific-cellular-modulators-of-fibrosis-in-systemic-sclerosis
#15
Jaclyn N Taroni, Casey S Greene, Viktor Martyanov, Tammara A Wood, Romy B Christmann, Harrison W Farber, Robert A Lafyatis, Christopher P Denton, Monique E Hinchcliff, Patricia A Pioli, J Matthew Mahoney, Michael L Whitfield
BACKGROUND: Systemic sclerosis (SSc) is a multi-organ autoimmune disease characterized by skin fibrosis. Internal organ involvement is heterogeneous. It is unknown whether disease mechanisms are common across all involved affected tissues or if each manifestation has a distinct underlying pathology. METHODS: We used consensus clustering to compare gene expression profiles of biopsies from four SSc-affected tissues (skin, lung, esophagus, and peripheral blood) from patients with SSc, and the related conditions pulmonary fibrosis (PF) and pulmonary arterial hypertension, and derived a consensus disease-associate signature across all tissues...
March 23, 2017: Genome Medicine
https://www.readbyqxmd.com/read/28303747/self-reported-physical-capacity-and-activity-in-patients-with-systemic-sclerosis-and-matched-controls
#16
H Pettersson, A Åkerström, A Nordin, E Svenungsson, H Alexanderson, C Boström
OBJECTIVE: The aim was to investigate differences in self-reported physical capacity and activity between systemic sclerosis (SSc) patients and population-based controls, as well as between patients with normal-mild, or moderate-severe, lung disease and their respective controls. METHOD: The study included 106 patients fulfilling the American College of Rheumatology SSc criteria and 106 controls, individually matched for age and gender. Patients were subdivided into normal-mild and moderate-severe lung disease based on results from pulmonary function tests and SSc severity scale...
March 17, 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/28302901/treatment-of-renal-angiomyolipoma-and-other-hamartomas-in-patients-with-tuberous-sclerosis-complex
#17
REVIEW
Joshua A Samuels
Tuberous sclerosis complex is an autosomal dominant genetic disease characterized by growth of benign tumors (hamartomas) in multiple organs, especially the kidneys, brain, heart, lungs, and skin. Tuberous sclerosis complex is usually caused by a mutation in either the tuberous sclerosis complex 1 or tuberous sclerosis complex 2 gene, resulting in constitutive activation of mammalian target of rapamycin signaling. Currently, mammalian target of rapamycin inhibitors are recommended in adult patients with tuberous sclerosis complex for the treatment of asymptomatic, growing renal angiomyolipoma that are >3 cm in diameter and pediatric or adult patients with brain lesions (subependymal giant cell astrocytoma) that either are growing or are not amenable to surgical resection...
July 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28284853/pulmonary-magnetic-resonance-imaging-is-similar-to-chest-tomography-in-detecting-inflammation-in-patients-with-systemic-sclerosis
#18
Carolina de Souza Müller, Danny Warszawiak, Eduardo Dos Santos Paiva, Dante Luiz Escuissato
Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are prevalent complications of systemic sclerosis (SS) and are currently the leading causes of death related to the disease. The accurate recognition of these conditions is therefore of utmost importance for patient management. A study was carried out with 24 SS patients being followed at the Rheumatology Department of the Hospital de Clínicas of Universidade Federal do Paraná (UFPR) and 14 healthy volunteers, with the objective of evaluating the usefulness of lung magnetic resonance imaging (MRI) when assessing ILD in SS patients...
February 20, 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28275485/serum-kl-6-and-surfactant-protein-d-as-monitoring-and-predictive-markers-of-interstitial-lung-disease-in-patients-with-systemic-sclerosis-and-mixed-connective-tissue-disease
#19
Hideaki Yamakawa, Eri Hagiwara, Hideya Kitamura, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Koji Okudela, Tae Iwasawa, Tamiko Takemura, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Interstitial lung disease (ILD) is frequent complication of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The disease is heterogeneous, and its outcome is unpredictable. Some patients have severe and progressive deterioration of ILD, which is the leading cause of mortality. We aimed to determine whether serum levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) correlate with SSc/MCTD-associated ILD activity. METHODS: We retrospectively analyzed the medical records of 40 patients with SSc/MCTD-associated ILD: 29 patients with SSc and 11 patients with MCTD...
February 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28088339/evidence-based-management-of-systemic-sclerosis-navigating-recommendations-and-guidelines
#20
REVIEW
Russell Edward Pellar, Janet Elizabeth Pope
OBJECTIVES: Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed. METHODS: The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted...
June 2017: Seminars in Arthritis and Rheumatism
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