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lung disease scleroderma

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https://www.readbyqxmd.com/read/28629914/mortality-risk-prediction-in-scleroderma-related-interstitial-lung-disease-the-sadl-model
#1
Julie Morisset, Eric Vittinghoff, Brett M Elicker, Xiaowen Hu, Stephanie Le, Jay H Ryu, Kirk D Jones, Anna Haemel, Jeffrey A Golden, Francesco Boin, Brett Ley, Paul J Wolters, Talmadge E King, Harold R Collard, Joyce S Lee
RATIONALE: Interstitial lung disease (ILD) is an important cause of morbidity and mortality in scleroderma (Scl). Risk prediction and prognostication in Scl-ILD patients is challenging because of heterogeneity in the disease course. METHODS: We aimed to develop a clinical mortality risk prediction model for Scl-ILD. Patients with Scl-ILD were identified from two ongoing longitudinal cohorts: 135 patients at the University of California, San Francisco (UCSF, derivation cohort) and 90 patients at the Mayo Clinic (validation cohort)...
June 16, 2017: Chest
https://www.readbyqxmd.com/read/28621173/evaluation-and-management-approaches-for-scleroderma-lung-disease
#2
Sara R Schoenfeld, Flavia V Castelino
Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are leading causes of morbidity and mortality in systemic sclerosis (SSc). As symptoms are often under-reported in SSc, early screening of ILD and PAH is of paramount importance, and early treatment may be associated with better clinical outcomes. Serologies are particularly helpful in identifying patients at risk for pulmonary involvement. Pulmonary function testing, high-resolution computed tomography of the chest and echocardiography are important tools in the initial screening of these patients...
June 1, 2017: Therapeutic Advances in Respiratory Disease
https://www.readbyqxmd.com/read/28619061/rituximab-versus-cyclophosphamide-for-the-treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-recital-study-protocol-for-a-randomised-controlled-trial
#3
Peter Saunders, Vicky Tsipouri, Gregory J Keir, Deborah Ashby, Marcus D Flather, Helen Parfrey, Daphne Babalis, Elisabetta A Renzoni, Christopher P Denton, Athol U Wells, Toby M Maher
BACKGROUND: Interstitial lung disease (ILD) frequently complicates systemic autoimmune disorders resulting in considerable morbidity and mortality. The connective tissue diseases (CTDs) most frequently resulting in ILD include: systemic sclerosis, idiopathic inflammatory myositis (including dermatomyositis, polymyositis and anti-synthetase syndrome) and mixed connective tissue disease. Despite the development, over the last two decades, of a range of biological therapies which have resulted in significant improvements in the treatment of the systemic manifestations of CTD, the management of CTD-associated ILD has changed little...
June 15, 2017: Trials
https://www.readbyqxmd.com/read/28580628/mortality-in-the-waikato-hospital-systemic-sclerosis-cohort
#4
Clement Ooi, Kamal Solanki, Chunhuan Lao, Chris Frampton, Douglas White
OBJECTIVE: To characterize the causes of mortality and standardised mortality ratio in a cohort of patients with systemic sclerosis (SSc). METHODS: A cohort of 132 patients enrolled at the Waikato Systemic Sclerosis Clinic was prospectively followed from 2005 to 2016. Patient demographics, diagnoses and laboratory reports were used to assess risk of mortality and generate standardised mortality ratios (SMR). Survival was analyzed using Kaplan-Meier methods. RESULTS: Of the cohort of 132 patients, 20 (15%) were deceased by the end of the study period...
June 4, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28576149/survival-and-quality-of-life-in-incident-systemic-sclerosis-related-pulmonary-arterial-hypertension
#5
Kathleen Morrisroe, Wendy Stevens, Molla Huq, David Prior, Jo Sahhar, Gene-Siew Ngian, David Celermajer, Jane Zochling, Susanna Proudman, Mandana Nikpour
BACKGROUND: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). We sought to determine survival, predictors of mortality, and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH. METHODS: We studied consecutive SSc patients with newly diagnosed (incident) World Health Organization (WHO) Group 1 PAH enrolled in a prospective cohort between 2009 and 2015. Survival methods were used to determine age and sex-adjusted standardised mortality ratio (SMR) and years of life lost (YLL), and to identify predictors of mortality...
June 2, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28572686/use-of-patterned-collagen-coated-slides-to-study-normal-and-scleroderma-lung-fibroblast-migration
#6
Bahja Ahmed Abdi, Henry Lopez, Sarah Karrar, Elisabetta Renzoni, Athol Wells, Angela Tam, Oseme Etomi, J Justin Hsuan, George R Martin, Xu Shiwen, Christopher P Denton, David Abraham, Richard Stratton
Systemic sclerosis (SSc) is a spreading fibrotic disease affecting the skin and internal organs. We aimed to model pathogenic fibroblast migration in SSc in order to identify enhancing factors, measure the effect of migrating cells on underlying extracellular matrix (ECM) and test possible therapeutic inhibitors. Novel patterned collagen substrates were used to investigate alignment and migration of skin and lung fibroblasts from SSc patients and healthy controls. Normal lung but not skin fibroblasts consistently elongated and aligned with underlying collagen and migrated dependent on PDGF or serum...
June 1, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28555642/tgf-%C3%AE-activation-by-bone-marrow-derived-thrombospondin-1-causes-schistosoma-and-hypoxia-induced-pulmonary-hypertension
#7
Rahul Kumar, Claudia Mickael, Biruk Kassa, Liya Gebreab, Jeffrey C Robinson, Daniel E Koyanagi, Linda Sanders, Lea Barthel, Christina Meadows, Daniel Fox, David Irwin, Min Li, B Alexandre McKeon, Suzette Riddle, R Dale Brown, Leslie E Morgan, Christopher M Evans, Daniel Hernandez-Saavedra, Angela Bandeira, James P Maloney, Todd M Bull, William J Janssen, Kurt R Stenmark, Rubin M Tuder, Brian B Graham
Pulmonary arterial hypertension (PAH) is an obstructive disease of the precapillary pulmonary arteries. Schistosomiasis-associated PAH shares altered vascular TGF-β signalling with idiopathic, heritable and autoimmune-associated etiologies; moreover, TGF-β blockade can prevent experimental pulmonary hypertension (PH) in pre-clinical models. TGF-β is regulated at the level of activation, but how TGF-β is activated in this disease is unknown. Here we show TGF-β activation by thrombospondin-1 (TSP-1) is both required and sufficient for the development of PH in Schistosoma-exposed mice...
May 30, 2017: Nature Communications
https://www.readbyqxmd.com/read/28542177/mycophenolate-mofetil-for-scleroderma-related-interstitial-lung-disease-a-real-world-experience
#8
Misbah Baqir, Ashima Makol, Thomas G Osborn, Brian J Bartholmai, Jay H Ryu
BACKGROUND AND OBJECTIVE: Interstitial lung disease (ILD) remains the number one cause of mortality in scleroderma (SSc). Our goal was to determine the effectiveness of mycophenolate mofetil (MMF) in treating SSc-ILD in a retrospective study. METHODS: A retrospective, computer-assisted search was performed to identify patients with SSc-ILD treated with MMF from 1997 through 2014. We used a novel software tool, Computer-Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER), to quantify parenchymal lung abnormalities on high-resolution computed tomography...
2017: PloS One
https://www.readbyqxmd.com/read/28456874/pulmonary-artery-dimensions-as-a-prognosticator-of-transplant-free-survival-in-scleroderma-interstitial-lung-disease
#9
James Benjamin Gleason, Krunal B Patel, Felix Hernandez, Anas Hadeh, Kristin B Highland, Franck Rahaghi, Jinesh P Mehta
BACKGROUND: Systemic sclerosis is a chronic debilitating autoimmune disease characterized by endothelial dysfunction and multi-organ fibrosis. Interstitial lung disease, a common manifestation of SSc, is termed scleroderma-related interstitial lung disease (SSc-ILD) and along with pulmonary hypertension contributes to a majority of deaths in SSc. SSc-ILD patients frequently develop pulmonary hypertension, which prognosticates a poorer outcome. We investigated pulmonary artery dimensions as an outcome predictor in patients with SSc-ILD...
April 29, 2017: Lung
https://www.readbyqxmd.com/read/28446601/diffuse-parenchymal-lung-disease
#10
REVIEW
Sara Tomassetti, Claudia Ravaglia, Venerino Poletti
Between September 2015 and August 2016 there were >1500 publications in the field of diffuse parenchymal lung diseases (DPLDs). For the Clinical Year in Review session at the European Respiratory Society Congress that was held in London, UK, in September 2016, we selected only five articles. This selection, made from the enormous number of published papers, does not include all the relevant studies that will significantly impact our knowledge in the field of DPLDs in the near future. This review article provides our personal view on the following topics: early diagnosis of idiopathic pulmonary fibrosis, current knowledge on the multidisciplinary team diagnosis of DPLDs and the diagnostic role of transbronchial cryobiopsy in this diagnostic setting, insights on the new entity of interstitial pneumonia with autoimmune features, and new therapeutic approaches for scleroderma-related interstitial lung disease...
June 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28413064/systemic-sclerosis
#11
REVIEW
Christopher P Denton, Dinesh Khanna
Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis...
April 13, 2017: Lancet
https://www.readbyqxmd.com/read/28386760/lung-transplantation-in-patients-with-systemic-sclerosis
#12
REVIEW
Rupal J Shah, Francesco Boin
PURPOSE OF REVIEW: The purpose of this study was to highlight recent findings on evaluation for lung transplantation and outcomes after transplantation in patients with systemic sclerosis (scleroderma). RECENT FINDINGS: The recognition that extra-pulmonary disease manifestations can seriously compromise post-transplant outcomes has changed the way patients are screened for lung transplant candidacy. Reluctance to transplant subjects affected by scleroderma has been driven by the complexity and multisystem nature of this disease...
May 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28376288/mycophenolate-mofetil-versus-placebo-for-systemic-sclerosis-related-interstitial-lung-disease-an-analysis-of-scleroderma-lung-studies-i-and-ii
#13
Elizabeth R Volkmann, Donald P Tashkin, Ning Li, Michael D Roth, Dinesh Khanna, Anna-Maria Hoffmann-Vold, Grace Kim, Jonathan Goldin, Philip J Clements, Daniel E Furst, Robert M Elashoff
OBJECTIVE: To compare mycophenolate mofetil (MMF) with placebo for the treatment of systemic sclerosis (SSc)-related interstitial lung disease (ILD). METHODS: We included participants enrolled in the placebo arm of Scleroderma Lung Study (SLS) I and the MMF arm of SLS II. SLS I randomized participants to receive either oral cyclophosphamide (CYC) or placebo for 1 year, while SLS II randomized participants to receive either MMF for 2 years or oral CYC for 1 year followed by 1 year of placebo...
April 4, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28365359/leflunomide-prevents-ros-induced-systemic-fibrosis-in-mice
#14
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/28340090/predictive-value-of-european-scleroderma-group-activity-index-in-an-early-scleroderma-cohort
#15
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/28331982/autoantibodies-in-children-with-juvenile-dermatomyositis-a-single-centre-experience-from-north-west-india
#16
Altaf Hussain, Amit Rawat, Ankur Kumar Jindal, Anju Gupta, Surjit Singh
The objective of this study is to determine autoantibody profile in children with juvenile dermatomyositis (JDM). Children who were diagnosed with JDM (either recently diagnosed during the study period or follow-up patients) were included in the study. Autoantibodies were detected with commercially available Immunodot kit. Thirty patients were included in the study. Nine out of thirty patients (30%) were positive for one of the 12 autoantibodies tested. Anti-SRP antibody was most common antibody detected in 3 patients followed by anti-MDA-5 antibody in 2 patients; while anti-Jo1 antibody, anti-TIF1-γ antibody, anti-Mi-2 antibody, and anti-PM-Scl antibody were positive in 1 patient each...
May 2017: Rheumatology International
https://www.readbyqxmd.com/read/28282243/differences-in-right-ventricular-functional-changes-during-treatment-between-systemic-sclerosis-associated-pulmonary-arterial-hypertension-and-idiopathic-pulmonary-arterial-hypertension
#17
Rahul G Argula, Abhijit Karwa, Abigail Lauer, David Gregg, Richard M Silver, Carol Feghali-Bostwick, Lynn M Schanpp, Kim Egbert, Bruce W Usher, Viswanathan Ramakrishnan, Paul M Hassoun, Charlie Strange
RATIONALE: Patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) continue to have an unacceptably high mortality rate despite the progress achieved with pulmonary arterial vasodilator therapies. OBJECTIVES: We sought to determine whether SSc-PAH is a clinically distinct pulmonary vascular disease phenotype when compared with idiopathic pulmonary arterial hypertension (IPAH) on the basis of progression of echocardiographic right ventricular (RV) dysfunction...
May 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28263294/increased-expression-of-latent-tgf-%C3%AE-binding-protein-4-affects-the-fibrotic-process-in-scleroderma-by-tgf-%C3%AE-smad-signaling
#18
Jiaying Lu, Qingmei Liu, Lei Wang, Wenzhen Tu, Haiyan Chu, Weifeng Ding, Shuai Jiang, Yanyun Ma, Xiangguang Shi, Weilin Pu, Xiaodong Zhou, Li Jin, Jiucun Wang, Wenyu Wu
Scleroderma is a fibrosis-related disorder characterized by cutaneous and internal organ fibrosis, and excessive collagen deposition in extracellular matrix (ECM) is a major cause of fibrosis. Transforming growth factor-β (TGF-β)/SMAD signaling has a central role in the pathogenesis of fibrosis by inducing abnormal collagen accumulation in ECM, and latent TGF-β-binding protein 4 (LTBP-4) affects the secretion of latent TGF-β to ECM. A previous study indicated that bleomycin (BLM) treatment increased LTBP-4 expression in lung fibroblasts of Thy-1 knockout mice with lung fibrosis, and LTBP-4 further promoted TGF-β bioavailability as well as SMAD3 phosphorylation...
March 6, 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/28254742/pirfenidone-ameliorates-murine-chronic-gvhd-through-inhibition-of-macrophage-infiltration-and-tgf-%C3%AE-production
#19
Jing Du, Katelyn Paz, Ryan Flynn, Ante Vulic, Tara M Robinson, Katie E Lineburg, Kylie A Alexander, Jingjing Meng, Sabita Roy, Angela Panoskaltsis-Mortari, Michael Loschi, Geoffrey R Hill, Jonathan S Serody, Ivan Maillard, David Miklos, John Koreth, Corey S Cutler, Joseph H Antin, Jerome Ritz, Kelli P MacDonald, Timothy W Schacker, Leo Luznik, Bruce R Blazar
Allogeneic hematopoietic stem cell transplantation is hampered by chronic graft-versus-host disease (cGVHD), resulting in multiorgan fibrosis and diminished function. Fibrosis in lung and skin leads to progressive bronchiolitis obliterans (BO) and scleroderma, respectively, for which new treatments are needed. We evaluated pirfenidone, a Food and Drug Administration (FDA)-approved drug for idiopathic pulmonary fibrosis, for its therapeutic effect in cGVHD mouse models with distinct pathophysiology. In a full major histocompatibility complex (MHC)-mismatched, multiorgan system model with BO, donor T-cell responses that support pathogenic antibody production are required for cGVHD development...
May 4, 2017: Blood
https://www.readbyqxmd.com/read/28217959/brief-report-anti-rnpc-3-antibodies-as-a-marker-of-cancer-associated-scleroderma
#20
Ami A Shah, George Xu, Antony Rosen, Laura K Hummers, Fredrick M Wigley, Stephen J Elledge, Livia Casciola-Rosen
OBJECTIVE: Prior studies have demonstrated an increased risk of cancer-associated scleroderma in patients with anti-RNA polymerase III (anti-RNAP III) autoantibodies as well as in patients who are triple-negative for anticentromere (anti-CENP), anti-topoisomerase I (anti-topo I), and anti-RNAP III (also known as anti-POL) autoantibodies (referred to as CTP negative). In a recent study of 16 CTP-negative scleroderma patients with coincident cancer, 25% of the patients were found to have autoantibodies to RNPC-3, a member of the minor spliceosome complex...
June 2017: Arthritis & Rheumatology
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