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lung disease scleroderma

L L Ji, H Wang, X H Zhang, Z L Zhang
OBJECTIVE: To evaluate the correlation between nailfold capillaroscopic (NC) findings and the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc) patients. METHODS: We retrospectively involved 71 SSc patients, 45 patients with ILD. NC was performed in all the patients according to the standard method. The NC findings were semi-quantitatively scored, including enlarged and giant capillaries, hemorrhages, loss of capillaries, avascular areas, ramified/bushy capillaries and disorganization of the vascular array...
June 18, 2018: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
Roger Diegel, Brad Black, Jean C Pfau, Tracy McNew, Curtis Noonan, Raja Flores
An increased risk for Systemic Autoimmune Diseases (SAID) has been reported in Libby, Montana, where extensive exposures to fibrous amphiboles occurred due to mining and use of asbestos-laden vermiculite. In addition, positive antinuclear autoantibody tests are associated with exposure to Libby Asbestiform Amphiboles (LAA) in both humans and mice. Among 6603 subjects who underwent health screening at the Center for Asbestos Related Diseases (CARD, Libby MT), 13.8% were diagnosed with an autoimmune disease, with prevalence values for the most common SAID being significantly higher than expected in the United States...
June 21, 2018: Journal of Toxicology and Environmental Health. Part A
Dane H Dougherty, Linda Kwakkenbos, Marie-Eve Carrier, Gloria Salazar, Shervin Assassi, Murray Baron, Susan J Bartlett, Daniel E Furst, Karen Gottesman, Frank van den Hoogen, Vanessa L Malcarne, Luc Mouthon, Warren R Nielson, Serge Poiraudeau, Maureen Sauvé, Gilles Boire, Alessandra Bruns, Lorinda Chung, Christopher Denton, James V Dunne, Paul Fortin, Tracy Frech, Anna Gill, Jessica Gordon, Ariane L Herrick, Monique Hinchcliff, Marie Hudson, Sindhu R Johnson, Niall Jones, Suzanne Kafaja, Maggie Larché, Joanne Manning, Janet Pope, Robert Spiera, Virginia Steen, Evelyn Sutton, Carter Thorne, Pearce Wilcox, Brett D Thombs, Maureen D Mayes
Objectives: The Scleroderma Patient-centered Intervention Network (SPIN) Cohort is a web-based cohort designed to collect patient-reported outcomes at regular intervals as a framework for conducting trials of psychosocial, educational, self-management and rehabilitation interventions for patients with SSc. The aim of this study was to present baseline demographic, medical and patient-reported outcome data of the SPIN Cohort and to compare it with other large SSc cohorts. Methods: Descriptive statistics were used to summarize SPIN Cohort characteristics; these were compared with published data of the European Scleroderma Trials and Research (EUSTAR) and Canadian Scleroderma Research Group (CSRG) cohorts...
June 2, 2018: Rheumatology
S Shenavandeh, S B Hashemi, MahmoodReza Masoudi, M A Nazarinia, A Zare
Systemic sclerosis is a multi-systemic disease with widespread small-vessel vasculopathy and fibrosis. Involvement of the middle and inner ear and hearing loss has been reported as an uncommon manifestation of scleroderma in some studies. In this study, we evaluated hearing problems in scleroderma patients and determined its association with clinical manifestations and capillaroscopy. We evaluated 54 patients with scleroderma referred to Hafez Hospital clinic of scleroderma related to Shiraz University of Medical Science; they fulfilled the LeRoy and ACR/EULAR criteria for scleroderma...
June 2, 2018: Clinical Rheumatology
Xiaoru Xia, Caijun Dai, Hua Yu, Xiaoying Huang, Ali Chen, Yingxia Tan, Liangxing Wang
Interstitial lung disease is the most common complication of systemic sclerosis (SSc) and is associated with a high rate of mortality. Due to the complex pathogenesis of SSc, the therapies currently available remain limited. In the present study, the effect of asiatic acid (AA) on SSc-associated pulmonary fibrosis (PF) and its association with the transforming growth factor-β1 (TGF-β1)/Smad2/3 signaling pathway were evaluated. A hypochlorous acid (HOCl)-induced model of SSc was used to evaluate the therapeutic effect of AA on PF in SSc, where AA was administered to SSc mice by gavage...
June 2018: Oncology Letters
Christopher A Mecoli, Ami A Shah, Francesco Boin, Fredrick M Wigley, Laura K Hummers
Two major complications in scleroderma patients that cause substantial morbidity and mortality are ischemic digital lesions (DL) and pulmonary hypertension (PH). The clinician's ability to predict which patients will develop these complications is imperfect. We conducted a prospective observational cohort study of 300 patients with scleroderma who were followed for at least a 5-year period. At baseline, patients lacked evidence of PH and were without a current DL. At each 6-month visit, the patient was examined for signs/symptoms of PH and/or a DL...
May 26, 2018: Clinical Rheumatology
Andreu Fernández-Codina, Kyle M Walker, Janet E Pope
INTRODUCTION: Treatment for many aspects of systemic sclerosis (SSc) lacks agreement. OBJECTIVES: To generate SSc treatment algorithms endorsed by high percentage of SSc experts. METHODS: Experts from the Scleroderma Clinical Trials Consortium and the Canadian Scleroderma Research group (N=170) were asked whether they agreed with SSc algorithms (from 2012). Two consensus rounds refined agreement; 62 (36%), 54 (32%) and 48 (28%) experts completed surveys...
May 21, 2018: Arthritis & Rheumatology
Harry Karmouty-Quintana, Jose G Molina, Kemly Philip, Chiara Bellocchi, Brent Gudenkauf, Minghua Wu, Ning-Yuan Chen, Scott D Collum, Junsuk Ko, Sandeep K Agarwal, Shervin Assassi, Hongyan Zhong, Michael R Blackburn, Tingting Weng
OBJECTIVE: Systemic sclerosis (SSc - scleroderma) is a chronic disease that affects the skin and various internal organs. Dermal fibrosis is a major component of this disease. The mechanisms that promote dermal fibrosis remain elusive. Elevations in tissue adenosine levels and the subsequent engagement of the pro-fibrotic A2B adenosine receptor (ADORA2B) have been shown to regulate fibrosis in multiple organs including the lung, kidney and penis; however, the role of the ADORA2B in dermal fibrosis has not been investigated...
May 17, 2018: Arthritis & Rheumatology
Małgorzata Michalska-Jakubus, Małgorzata Kowal, Michał Adamczyk, Dorota Krasowska
Introduction: Anti-endothelial cell antibodies (AECA) recognize endothelial cell proteins and are thought to play an important role in vascular damage observed in systemic scleroderma (SSc) and many other autoimmune diseases. In SSc, AECA were found to be more common in patients with pulmonary hypertension, digital ulcers and nailfold capillaroscopic changes. Until now, there have been no studies examining the association between AECA positivity with the activity and duration of the disease...
April 2018: Postȩpy Dermatologii i Alergologii
Ankur Kumar Jindal, Sandesh Guleria, Rakesh Kumar Pilania, Anuradha Bishnoi, Keshavamurthy Vinay, Sunil Dogra, Deepti Suri, Amit Rawat, Surjit Singh
The objective of this study was to report four children having juvenile dermatomyositis (JDM) with inverse Gottron sign along with a brief review of literature of similarly published cases. This is a retrospective review of case records of all children diagnosed with JDM at a single tertiary care centre in North India. Children who were found to have Gottron papules over palmar surface were identified. A total of 127 children were diagnosed with JDM in the last 24 years (1994-2017). Out of these 127 patients, 4 were found to have Gottron papules over palmar surface...
May 2, 2018: Rheumatology International
Silke Meiners, John Evankovich, Rama K Mallampalli
The present review aims to summarize available knowledge on the role of the ubiquitin-proteasome system (UPS) in the pathogenesis of scleroderma and scleroderma-related disease mechanisms. This will provide the reader with a more mechanistic understanding of disease pathogenesis and help to identify putative novel targets within the UPS for potential therapeutic intervention. Because of the heterogenous manifestations of scleroderma, we will primarily focus on conserved mechanisms that are involved in the development of lung scleroderma phenotypes...
March 29, 2018: Translational Research: the Journal of Laboratory and Clinical Medicine
A Zabara, S Selezneva, S Dubina, O Syniachenko
Systemic sclerosis (SSc) is accompanied by severe disorders of serum component of blood rheological properties (BRP), the study indicators of which performed as the monitoring of the therapeutic interventions effectiveness. BRP changes have not been established at different forms of SSc, the connection between physicochemical serum rheological properties and morphological signs of vascular skin lesions and extracardiac implications of the disease remains unknown. The purpose and objectives of the study - to evaluate the changes of BRP serum component with clinical and morphological variants of limited and diffuse form of SSc...
March 2018: Georgian Medical News
Mohammadali Nazarinia, Elmira Esmaeilzadeh
INTRODUCTION: Gauzoma is an iatrogenic complication, which occurs rarely due to surgical team negligence. Depending on the sterility of the retained tissue, it can lead to life threatening surgical complications or may remain asymptomatic for many years and be detected incidentally in imaging studies. It may be mistaken as tumors or aneurysms. Thus, high clinical suspicion is needed to diagnose them in patients with past history of operation. Reporting Case: A 35 years old woman, a known case of scleroderma underwent open-heart surgery 20 years before being diagnosed as scleroderma, presented by dyspnea especially on activity...
April 22, 2018: Current Rheumatology Reviews
Vivek Thakkar, Karen A Patterson, Wendy Stevens, Michelle Wilson, Janet Roddy, Joanne Sahhar, Susanna Proudman, Pravin Hissaria, Mandana Nikpour
Studies suggest elevated serum intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) levels may be markers of pulmonary arterial hypertension in systemic sclerosis (SSc-PAH). We sought to evaluate whether ICAM-1 and VCAM-1 levels are useful screening biomarkers for incident SSc-PAH. In this cross-sectional study, four groups were selected from the Australian Scleroderma Cohort Study: group 1 (n = 15) had definite PAH; group 2 (n = 19) had interstitial lung disease (ILD); group 3 (n = 30) were SSc-controls; and group 4 (n = 34) were healthy controls...
June 2018: Clinical Rheumatology
Takeru Igusa, Laura K Hummers, Kala Visvanathan, Carrie Richardson, Fredrick M Wigley, Livia Casciola-Rosen, Antony Rosen, Ami A Shah
OBJECTIVES: Recent studies demonstrate autoantibodies are powerful tools to interrogate molecular events linking cancer and the development of autoimmunity in scleroderma. Investigating cancer risk in these biologically relevant subsets may provide an opportunity to develop personalised cancer screening guidelines. In this study, we examined cancer risk in distinct serologic and phenotypic scleroderma subsets and compared estimates with the general population. METHODS: Patients in the Johns Hopkins Scleroderma Center observational cohort were studied...
April 20, 2018: Annals of the Rheumatic Diseases
Pauline Pradère, Igor Tudorache, Jesper Magnusson, Laurent Savale, Olivier Brugiere, Benoît Douvry, Martine Reynaud-Gaubert, Johanna Claustre, Aurélie Le Borgne, Are M Holm, Hans Henrik Schultz, Christiane Knoop, Laurent Godinas, Andrew J Fisher, Sandrine Hirschi, Jens Gottlieb, Jérôme Le Pavec
BACKGROUND: Due to its multisystemic nature, scleroderma is considered a relative contraindication to lung transplantation at many centers. However, recent studies suggest similar post-transplant outcomes in patients with scleroderma compared to those with other causes of interstitial lung disease (ILD). Furthermore, it remains unknown whether scleroderma-associated pulmonary arterial hypertension (PAH) influences post-transplant outcomes. Our objective in this study was to assess the indications, survival, and prognostic factors of lung or heart-lung transplantation for scleroderma lung disease...
March 15, 2018: Journal of Heart and Lung Transplantation
Andreas Recke, Ann-Katrin Regensburger, Florian Weigold, Antje Müller, Harald Heidecke, Gabriele Marschner, Christoph M Hammers, Ralf J Ludwig, Gabriela Riemekasten
Systemic sclerosis (SSc) is a severe chronic autoimmune disease with high morbidity and mortality. Sera of patients with SSc contain a large variety of autoantibody (aab) reactivities. Among these are functionally active aab that bind to G protein-coupled receptors (GPCR) such as C-X-C motif chemokine receptor 3 (CXCR3) and 4 (CXCR4). Aab binding to the N-terminal portion of these two GPCRs have been shown to be associated with slower disease progression in SSc, especially deterioration of lung function. Aabs binding to GPCRs exhibit functional activities by stimulating or inhibiting GPCR signaling...
2018: Frontiers in Immunology
Tomáš Soukup, Tomáš Veleta
Systemic sclerosis is classed as a diffuse (systemic) disease of connective tissue. It is a heterogeneous disease significantly shortening life expectancy. Its etiology is unknown. Pathogenetic interplay is assumed to involve a triad of pathological autoimmune inflammation, vasculopathy and fibrosis. Clinical manifestations can be classed based on the preponderant pathogenetic process. Vasculopathy is manifested by secondary Raynauds phenomenon with abnormal findings on the nailfold capillaroscopy, skin telangiectasias, gastric antral vascular ectasia, life threatening scleroderma renal crisis, digital ulcerations and prognostically severe pulmonary arterial hypertension...
2018: Vnitr̆ní Lékar̆ství
Marzia Dolcino, Andrea Pelosi, Piera Filomena Fiore, Giuseppe Patuzzo, Elisa Tinazzi, Claudio Lunardi, Antonio Puccetti
Systemic sclerosis (SSc) is a rare connective tissue disease characterized by three pathogenetic hallmarks: vasculopathy, dysregulation of the immune system, and fibrosis. A particular feature of SSc is the increased frequency of some types of malignancies, namely breast, lung, and hematological malignancies. Moreover, SSc may also be a paraneoplastic disease, again indicating a strong link between cancer and scleroderma. The reason of this association is still unknown; therefore, we aimed at investigating whether particular genetic or epigenetic factors may play a role in promoting cancer development in patients with SSc and whether some features are shared by the two conditions...
2018: Frontiers in Immunology
Anne E Wyman, Sergei P Atamas
PURPOSE OF REVIEW: Premature activation of aging-associated molecular mechanisms is emerging as an important contributor to many diseases, including scleroderma. Among central regulators of the aging process are a group of histone deacetylases called sirtuins (SIRTs). Recent findings implicate these molecules as pathophysiological players in scleroderma skin and lung fibrosis. The goal of this article is to review recent studies on the involvement of SIRTs in scleroderma from the perspective of aging-related molecular mechanisms...
March 17, 2018: Current Rheumatology Reports
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