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lung disease scleroderma

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https://www.readbyqxmd.com/read/28217959/anti-rnpc3-antibodies-as-a-marker-of-cancer-associated-scleroderma
#1
Ami A Shah, George Xu, Antony Rosen, Laura K Hummers, Fredrick M Wigley, Stephen J Elledge, Livia Casciola-Rosen
INTRODUCTION: Prior studies have demonstrated an increased risk of cancer-associated scleroderma in patients with RNA polymerase III (POL) autoantibodies and in patients negative for anti-centromere (CENP), anti-topoisomerase-1 (TOPO), and anti-POL antibodies (referred to as CENP/TOPO/POL (CTP)-Negative). In a recent study of 16 CTP-negative scleroderma patients with coincident cancer, we found that 25% had autoantibodies to RNPC3, a member of the minor spliceosome complex. In this investigation, we validated the relationship between anti-RNPC3 antibodies and cancer and examined the associated clinical phenotype in a large sample of scleroderma patients...
February 19, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28160477/the-arachidonate-5-lipoxygenase-activating-protein-gene-polymorphism-is-associated-with-the-risk-of-scleroderma-related-interstitial-lung-disease-a-multicentre-european-scleroderma-trials-and-research-group-eustar-study
#2
Otylia Kowal-Bielecka, Sylwia Chwiesko-Minarowska, Pawel L Bernatowicz, Yannick Allanore, Timothy Radstake, Marco Matucci-Cerinic, Jasper Broen, Roger Hesselstrand, Dorota Krasowska, Gabriella Riemekasten, Madelon Vonk, Oksana Kowalczuk, Marek Bielecki, Robert Milewski, Lech Chyczewski, Jacek Niklinski, Krzysztof Kowal
No abstract text is available yet for this article.
February 4, 2017: Rheumatology
https://www.readbyqxmd.com/read/28131664/survival-and-outcomes-after-lung-transplantation-for-non-scleroderma-connective-tissue-related-interstitial-lung-disease
#3
Andrew M Courtwright, Souheil El-Chemaly, Paul F Dellaripa, Hilary J Goldberg
BACKGROUND: Patients with non-scleroderma connective tissue-related lung disease (NS-CTLD), including rheumatoid arthritis, idiopathic inflammatory myopathies, Sjögren syndrome, mixed connective tissue disease, and systemic lupus erythematosus, may be at risk for worse outcomes after lung transplantation because of immune dysregulation or extrapulmonary manifestations of their underlying disease. We compared survival, acute and chronic rejection, and extrapulmonary organ dysfunction after transplantation in patients with NS-CTLD and idiopathic pulmonary fibrosis (IPF)...
December 31, 2016: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28088339/evidence-based-management-of-systemic-sclerosis-navigating-recommendations-and-guidelines
#4
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/28079847/relationship-between-abnormalities-on-high-resolution-computerized-tomography-pulmonary-function-and-bronchoalveolar-lavage-in-progressive-systemic-sclerosis
#5
Gulfidan Cakmak, Tuba Selcuk Can, Sule Gundogdu, Canan Akman, Hande Ikitimur, Benan Musellim, Gul Ongen
:  Introduction and aim: Progressive systemic sclerosis (pSS) is a multisystemic connective tissue disease characterized by fibrosis of the skin and internal organs including lung. The mechanisms that leads to progressive lung fibrosis in scleroderma remain obscure. In this study, we aimed to investigate the correlation between HRCT findings and patients' clinical and functional status and the degree of alveolitis based on the BAL resultsMaterials and methods: 65 patients with pSS were evaluated...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28065485/systemic-sclerosis-sine-scleroderma-in-mexican-patients-case-reports
#6
Olga Vera-Lastra, Christian Alexis Sauceda-Casas, María Del Pilar Cruz Domínguez, Sergio Alberto Mendoza Alvarez, Jesús Sepulceda-Delgado
: Systemic sclerosis sine scleroderma (ssSSc) is a form of systemic sclerosis that is characterized by Raynaud's phenomenon (RP), visceral involvement without thickening of skin and anticentromere antibodies (ACA). We studied 10 ssSsc patients with a prevalence of 2%. The clinical signs were: RP 9/10, esophageal manifestations 8/10, pulmonary arterial hypertension 4/10, interstitial lung disease 4/10, cardiac signs 3/10 and ACA 8/10. CONCLUSION: In patients with RP, esophageal dysmotility, interstitial lung disease and pulmonary arterial hypertension should be tested for ACA in order to establish a prompt diagnosis and treatment of ssSSc...
January 3, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28038680/changes-in-plasma-cxcl4-levels-are-associated-with-improvements-in-lung-function-in-patients-receiving-immunosuppressive-therapy-for-systemic-sclerosis-related-interstitial-lung-disease
#7
Elizabeth R Volkmann, Donald P Tashkin, Michael D Roth, Philip J Clements, Dinesh Khanna, Daniel E Furst, Maureen Mayes, Julio Charles, Chi-Hong Tseng, Robert M Elashoff, Shervin Assassi
BACKGROUND: Increased circulatory levels of the chemokine CXCL4 have been associated with the presence of interstitial lung disease (ILD) in an observational study of patients with systemic sclerosis (SSc). The purpose of the present study was to evaluate the relationship between baseline CXCL4 level and extent of ILD in the context of a randomized controlled trial and to determine whether changes in CXCL4 levels in response to immunosuppression are associated with future progression of SSc-ILD...
December 30, 2016: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28012804/improved-cough-and-cough-specific-quality-of-life-in-patients-treated-for-scleroderma-related-interstitial-lung-disease-ssc-ild-results-of-scleroderma-lung-study-ii
#8
Donald P Tashkin, Elizabeth R Volkmann, Chi-Hong Tseng, Michael D Roth, Dinesh Khanna, Daniel E Furst, Philip J Clements, Arthur Theodore, Suzanne Kafaja, Grace Hyun Kim, Jonathan Goldin, Edgar Ariolla, Robert M Elashoff
BACKGROUND: Cough is a common symptom of scleroderma-related interstitial lung disease (SSc-ILD), but its relationship to other characteristics of SSc-ILD, impact on cough-specific QoL and response to therapy for SSc-ILD have not been well studied. METHODS: We investigated frequent cough (FC) in SSc-ILD patients (N=142) enrolled in Scleroderma Lung Study II, a randomized controlled trial comparing mycophenolate mofetil (MMF) and oral cyclophosphamide (CYC) as treatments for ILD...
December 22, 2016: Chest
https://www.readbyqxmd.com/read/27943045/the-association-of-sociodemographic-and-objectively-assessed-disease-variables-with-fatigue-in-systemic-sclerosis-an-analysis-of-785-canadian-scleroderma-research-group-registry-patients
#9
Brooke Levis, Linda Kwakkenbos, Marie Hudson, Murray Baron, Brett D Thombs
Fatigue is prevalent among patients with systemic sclerosis (SSc). To date, studies investigating fatigue in SSc have been hampered by the instruments used to measure fatigue in SSc and have included patient-reported rather than objectively-rated measures of disease. The Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F) scale is a validated measure for assessing fatigue in SSc that, compared to other instruments, provides good coverage of the full range of the fatigue spectrum. The objective of this study was to assess sociodemographic and objectively-rated disease-related associates of fatigue, as measured by the FACIT-F, in a large sample of patients with SSc...
December 10, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27931056/-guideline-s2k-awmf-of-the-deutsche-gesellschaft-f%C3%A3-r-pneumologie-und-beatmungsmedizin-and-the-deutsche-gesellschaft-f%C3%A3-r-arbeitsmedizin-und-umweltmedizin-diagnostics-and-expert-opinion-in-the-occupational-disease-no-4101-silicosis-including-coal-worker-s-pneumoconiosis
#10
X Baur, M Heger, R M Bohle, K G Hering, K Hofmann-Preiß, D Nowak, A Tannapfel, H Teschler, T Voshaar, T Kraus
During the last 1.5 years an update of the guideline on silicosis was made by an interdisciplinary working group. New medical and scientific knowledge and the experience in expert opinion practice were taken into account.By preparing the initial guideline in 2010 standardization of diagnostics and adaption of the "Moers convention" which was not based on medical knowledge was in the focus, whereas the current update deals with fine emendation and extension, especially of the compensation rate (adaption with the Reichenhall recommendation)...
December 2016: Pneumologie
https://www.readbyqxmd.com/read/27815256/toll-like-receptor-4-activation-attenuates-profibrotic-response-in-control-lung-fibroblasts-but-not-in-fibroblasts-from-patients-with-ipf
#11
Simone Ebener, Sandra Barnowski, Carlos Wotzkow, Thomas M Marti, Elena Lopez-Rodriguez, Bruno Crestani, Fabian Blank, Ralph A Schmid, Thomas Geiser, Manuela Funke
Idiopathic pulmonary fibrosis (IPF) is a devastating lung disease with a median survival of 3 yr. IPF deteriorates upon viral or bacterial lung infection although pulmonary infection (pneumonia) in healthy lungs rarely induces fibrosis. Bacterial lipopolysaccharide (LPS) activates Toll-like receptor 4 (TLR4), initiating proinflammatory pathways. As TLR4 has already been linked to hepatic fibrosis and scleroderma, we now investigated the role of TLR4 in IPF fibroblasts. Lung tissue sections from patients with IPF were analyzed for TLR4 expression...
January 1, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/27719973/imaging-of-pulmonary-manifestations-of-connective-tissue-diseases
#12
REVIEW
Jitesh Ahuja, Deepika Arora, Jeffrey P Kanne, Travis S Henry, J David Godwin
Connective tissue diseases (CTDs) are a heterogeneous group of conditions characterized by circulating autoantibodies and autoimmune-mediated organ damage. Common CTDs with lung manifestations are rheumatoid arthritis, scleroderma or systemic sclerosis, Sjögren syndrome, polymyositis/dermatomyositis, systemic lupus erythematosis, mixed connective tissue disease, and undifferentiated connective tissue disease. The most common histopathologic patterns of CTD-related interstitial lung disease are nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and lymphoid interstitial pneumonia...
November 2016: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/27708971/low-vitamin-d-status-in-systemic-sclerosis-and-the-impact-on-disease-phenotype
#13
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/27703420/musculoskeletal-involvement-in-ssc-is-associated-with-worse-scores-on-short-form-36-and-scleroderma-health-assessment-questionnaire-and-lower-tumor-necrosis-factor-%C3%AE-gene-expression-in-peripheral-blood-mononuclear-cells
#14
Eliza R Pelrine, Marie-Dominique Ah-Kioon, Meng Zhang, Franck J Barrat, Robert F Spiera, Jessica K Gordon
BACKGROUND: Systemic sclerosis (SSc) is characterized by a wide variety of symptoms and disease manifestations including joint pain, gastrointestinal dysfunction, interstitial lung disease, and cardiomyopathy. QUESTIONS/PURPOSES: Using the Scleroderma Health Assessment Questionnaire (SHAQ) and Short Form-36 (SF-36) we explored how patient-reported physical health, mental health, and functional status related to these clinical characteristics and to cytokine levels utilizing the Hospital for Special Surgery Scleroderma Registry...
October 2016: HSS Journal: the Musculoskeletal Journal of Hospital for Special Surgery
https://www.readbyqxmd.com/read/27683612/versican-accumulates-in-vascular-lesions-in-pulmonary-arterial-hypertension
#15
Ya-Ting Chang, Christina K Chan, Inger Eriksson, Pamela Y Johnson, Xiaofang Cao, Christian Westöö, Christian Norvik, Annika Andersson-Sjöland, Gunilla Westergren-Thorsson, Staffan Johansson, Ulf Hedin, Lena Kjellén, Thomas N Wight, Karin Tran-Lundmark
Pulmonary arterial hypertension (PAH) is a lethal condition for which there is no effective curative pharmacotherapy. PAH is characterized by vasoconstriction, wall thickening of pulmonary arteries, and increased vascular resistance. Versican is a chondroitin sulfate proteoglycan in the vascular extracellular matrix that accumulates following vascular injury and promotes smooth-muscle cell proliferation in systemic arteries. Here, we investigated whether versican may play a similar role in PAH. Paraffin-embedded lung sections from patients who underwent lung transplantation to treat PAH were used for immunohistochemistry...
September 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/27677579/risk-factors-for-development-of-pulmonary-arterial-hypertension-in-australian-systemic-sclerosis-patients-results-from-a-large-multicenter-cohort-study
#16
Kathleen Morrisroe, Molla Huq, Wendy Stevens, Candice Rabusa, Susanna M Proudman, Mandana Nikpour
BACKGROUND: Pulmonary arterial hypertension (PAH) is the leading cause of mortality in patients with systemic sclerosis (SSc). We sought to determine the incidence, prevalence and risk factors for PAH development in a large Australian SSc cohort. METHODS: PAH was diagnosed on right heart catheterisation (mPAP >25 and PAWP <15 mmHg at rest). Patients with PH secondary to interstitial lung disease (ILD; defined as abnormal HRCT scan and FVC < 60 %) were excluded...
September 27, 2016: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/27635465/prevalence-and-risk-factors-for-left-ventricular-diastolic-dysfunction-in-a-scleroderma-cohort
#17
S Vemulapalli, L Cohen, V Hsu
OBJECTIVES: Left ventricular diastolic dysfunction (LVDD) is more common in systemic sclerosis (SSc) compared to the general population. Focal myocardial ischaemia and fibrosis may be important in its pathogenesis. LVDD is associated with increased mortality and little is known about the risk factors. Advanced SSc lung complications may accompany LVDD. METHOD: We conducted a cross-sectional study of 300 SSc outpatients with and without LVDD, seen between May 2012 and May 2014, and performed univariate and multivariate regression analyses to determine clinical factors associated with LVDD...
September 16, 2016: Scandinavian Journal of Rheumatology
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
#18
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/27613260/esophageal-motor-abnormalities-in-patients-with-scleroderma-%C3%A2-heterogeneity-risk-factors-and-effects-on-quality%C3%A2-of-life
#19
Michael D Crowell, Sarah B Umar, W Leroy Griffing, John K DiBaise, Brian E Lacy, Marcelo F Vela
BACKGROUND & AIMS: Systemic scleroderma (SSc) is associated with esophageal aperistalsis and hypotensive esophagogastric junction pressure, although there could be a gradation in esophageal motor dysfunction. We characterized esophageal motor function by high-resolution esophageal manometry (HRM) and assessed associations between SSc severity, health-related quality of life (HRQOL), and HRM findings in patients. METHODS: We performed a prospective study of 200 patients with SSc and 102 patients without SSc (controls) who underwent HRM at Mayo Clinic Arizona from May 2006 through January 2015...
February 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/27609773/plexin-c1-deficiency-permits-synaptotagmin-7-mediated-macrophage-migration-and-enhances-mammalian-lung-fibrosis
#20
Xueyan Peng, Meagan Moore, Aditi Mathur, Yang Zhou, Huanxing Sun, Ye Gan, Jose D Herazo-Maya, Naftali Kaminski, Xinyuan Hu, Hongyi Pan, Changwan Ryu, Awo Osafo-Addo, Robert J Homer, Carol Feghali-Bostwick, Wassim H Fares, Mridu Gulati, Buqu Hu, Chun-Geun Lee, Jack A Elias, Erica L Herzog
Pulmonary fibrosis is a progressive and often fatal condition that is believed to be partially orchestrated by macrophages. Mechanisms that control migration of these cells into and within the lung remain undefined. We evaluated the contributions of the semaphorin receptor, plexin C1 (PLXNC1), and the exocytic calcium sensor, synaptotagmin 7 (Syt7), in these processes. We evaluated the role of PLXNC1 in macrophage migration by using Boyden chambers and scratch tests, characterized its contribution to experimentally induced lung fibrosis in mice, and defined the mechanism for our observations...
December 2016: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
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