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lung activity disease systemic sclerosis

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https://www.readbyqxmd.com/read/28303747/self-reported-physical-capacity-and-activity-in-patients-with-systemic-sclerosis-and-matched-controls
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/27613696/niclosamide-prevents-systemic-sclerosis-in-a-reactive-oxygen-species-induced-mouse-model
#15
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
https://www.readbyqxmd.com/read/27548467/autologous-hematopoietic-stem-cell-transplantation-has-better-outcomes-than-conventional-therapies-in-patients-with-rapidly-progressive-systemic-sclerosis
#16
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...
January 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27538472/plasma-plasmin-%C3%AE-2-plasmin-inhibitor-complex-levels-may-predict-the-effect-of-cyclophosphamide-for-systemic-sclerosis-related-interstitial-lung-disease
#17
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
https://www.readbyqxmd.com/read/27497912/pulmonary-involvement-in-systemic-sclerosis
#18
REVIEW
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...
November 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27298374/ox40l-blockade-protects-against-inflammation-driven-fibrosis
#19
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
https://www.readbyqxmd.com/read/27283334/long-term-outcome-in-juvenile-onset-mixed-connective-tissue-disease-a-nationwide-norwegian-study
#20
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...
January 2017: Annals of the Rheumatic Diseases
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