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lung activity disease systemic sclerosis

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https://www.readbyqxmd.com/read/28432700/serum-h-ficolin-levels-clinical-association-with-interstitial-lung-disease-in-patients-with-systemic-sclerosis
#1
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
#2
Anne Elizabeth 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. 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, -3, and -6, 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...
April 6, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28365359/leflunomide-prevents-ros-induced-systemic-fibrosis-in-mice
#3
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
#4
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
#5
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...
March 17, 2017: Rheumatology
https://www.readbyqxmd.com/read/28330499/a-novel-multi-network-approach-reveals-tissue-specific-cellular-modulators-of-fibrosis-in-systemic-sclerosis
#6
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
#7
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
#8
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...
March 16, 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
#9
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
#10
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
#11
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...
December 9, 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28018840/hand-impairment-in-systemic-sclerosis-various-manifestations-and-currently-available-treatment
#12
Amber Young, Rajaie Namas, Carole Dodge, Dinesh Khanna
Systemic sclerosis (SSc) is an autoimmune disease initially recognized by hand involvement due to characteristic Raynaud's phenomenon (RP), puffy hands, skin thickening, and contractures resembling claw deformities. SSc contributes to hand impairment through inflammatory arthritis, joint contractures, tendon friction rubs (TFRs), RP, digital ulcers (DU), puffy hands, skin sclerosis, acro-osteolysis, and calcinosis. These manifestations, which often co-exist, can contribute to difficulty with occupational activities and activities of daily living (ADL), which can result in impaired quality of life...
September 2016: Current Treatment Options in Rheumatology
https://www.readbyqxmd.com/read/27940596/predicting-cardiopulmonary-involvement-in-patients-with-systemic-sclerosis-complementary-value-of-nailfold-videocapillaroscopy-patterns-and-disease-specific-autoantibodies
#13
Iris M Markusse, Jessica Meijs, Berber de Boer, Jaap A Bakker, H Pascal C Schippers, Anne A Schouffoer, Nina Ajmone Marsan, Lucia J M Kroft, Maarten K Ninaber, Tom W J Huizinga, Jeska K de Vries-Bouwstra
OBJECTIVE: To evaluate the prevalence of anti-extractable nuclear antigen (anti-ENA) antibodies in Dutch SSc patients and the predictive power of the combination of specific anti-ENA antibodies and nailfold videocapillaroscopy (NVC) patterns to improve identification of patients with high risk for cardiopulmonary involvement. METHODS: A total of 287 patients (79%) from the Leiden SSc-Cohort had data available on NVC-pattern (no SSc-specific, early, active, late) and anti-ENA antibodies...
December 10, 2016: Rheumatology
https://www.readbyqxmd.com/read/27720780/epigenetics-in-fibrosis
#14
REVIEW
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
https://www.readbyqxmd.com/read/27708971/low-vitamin-d-status-in-systemic-sclerosis-and-the-impact-on-disease-phenotype
#15
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: European Journal of Rheumatology
https://www.readbyqxmd.com/read/27683525/the-clinical-value-of-soluble-urokinase-plasminogen-activator-receptor-supar-levels-in-autoimmune-connective-tissue-disorders
#16
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
https://www.readbyqxmd.com/read/27650313/the-nlrp3-inflammasome-in-pathogenic-particle-and-fibre-associated-lung-inflammation-and-diseases
#17
REVIEW
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...
September 20, 2016: Particle and Fibre Toxicology
https://www.readbyqxmd.com/read/27629736/the-safety-of-intravenous-cyclophosphamide-in-the-treatment-of-rheumatic-diseases
#18
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
https://www.readbyqxmd.com/read/27621593/spotlight-on-tocilizumab-and-its-potential-in-the-treatment-of-systemic-sclerosis
#19
REVIEW
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
https://www.readbyqxmd.com/read/27621285/the-european-scleroderma-trials-and-research-group-eustar-task-force-for-the-development-of-revised-activity-criteria-for-systemic-sclerosis-derivation-and-validation-of-a-preliminarily-revised-eustar-activity-index
#20
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...
January 2017: Annals of the Rheumatic Diseases
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