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lung disease scleroderma

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https://www.readbyqxmd.com/read/29214548/first-clinical-symptom-as-a-prognostic-factor-in-systemic-sclerosis-results-of-a-retrospective-nationwide-cohort-study
#1
Manuel Rubio-Rivas, Xavier Corbella, Melany Pestaña-Fernández, Carles Tolosa-Vilella, Alfredo Guillen-Del Castillo, Dolores Colunga-Argüelles, Luis Trapiella-Martínez, Nerea Iniesta-Arandia, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, Jose Antonio Vargas-Hitos, Juan José Ríos-Blanco, Jose Antonio Todolí-Parra, Mónica Rodríguez-Carballeira, Adela Marín-Ballvé, Pablo Segovia-Alonso, Xavier Pla-Salas, Ana Belén Madroñero-Vuelta, Manuel Ruiz-Muñoz, Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar, E Callejas Moraga, E Calvo, C Carbonell, M J Castillo, A J Chamorro, D Colunga, X Corbella, M V Egurbide, G Espinosa, V Fonollosa, M Freire, F J García Hernández, R González León, A Guillén Del Castillo, N Iniesta, R Lorenzo, A B Madroñero, B Marí, A Marín, N Ortego-Centeno, M Pérez Conesa, M Pestaña, X Pla, J J Ríos Blanco, M Rodríguez Carballeira, M Rubio Rivas, M Ruiz Muñoz, L Sáez Comet, P Segovia, C P Simeón, A Soto, E Tarí, J A Todolí, C Tolosa, L Trapiella, J A Vargas Hitos, G Verdejo
The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited...
December 7, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29207307/systemic-sclerosis-clinical-manifestations-anesthetic-and-orthopedic-considerations-in-a-patient
#2
Obada Hasan, Muneeba Jessar, Muhammad Ashar, Shahryar Noordin, Tashfeen Ahmad
INTRODUCTION: Systemic sclerosis is a rare and progressive multisystem autoimmune disorder that is characterized pathologically by vascular abnormalities, connective tissue sclerosis and atrophy of skin and various internal organs (e.g., alimentary tract, lungs, heart, kidney, CNS), and autoantibodies. With an unknown etiology, Scleroderma is a complex polygenetic disease. A recent Genome Wide Association Study (GWAS) confirmed a strong association with the Major Histocompatibility Complex (MHC) and autoimmunity...
December 2, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29164837/serum-surfactant-protein-d-and-exhaled-nitric-oxide-as-biomarkers-of-early-lung-damage-in-systemic-sclerosis
#3
Alida Benfante, Riccardo Messina, Alessandra Paternò, Nicola Scichilone
BACKGROUND: Interstitial lung disease (ILD) complicates the course of systemic sclerosis (SSc), representing the main cause of death in these patients. The identification of parameters that can predict the early onset and progression of ILD in SSc represents an unmet need in clinical practice. The study was designed to explore whether the surfactant proteins (SP) A and D may be used as noninvasive tools for the early identification of ILD in SSc. Alveolar exhaled nitric oxide (NO) was investigated as a surrogate marker of distal inflammation...
November 21, 2017: Minerva Medica
https://www.readbyqxmd.com/read/29151520/dropped-head-syndrome-and-the-presence-of-rimmed-vacuoles-in-a-muscle-biopsy-in-scleroderma-polymyositis-overlap-syndrome-associated-with-anti-ku-antibody
#4
Yoshida Takeshi, Yoshida Mai, Mitsuyo Kinjo, Jonosono Manabu, Higuchi Itsuro
A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. A clinical examination revealed Raynaud phenomenon, arthralgia, distal skin sclerosis, and microbleeds in the nailfold capillaries. An anti-Ku antibody test was positive. A muscle biopsy revealed inflammatory myopathy with rimmed vacuoles (RVs). The diagnosis of scleroderma-polymyositis (SSc-PM) overlap syndrome was made. RVs on a muscle biopsy in a patient with inflammatory myositis involving axial muscles may be seen either in inclusion body myositis or SSc-PM overlap syndrome...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29142074/stat3-controls-col1a2-enhancer-activation-cooperatively-with-junb-regulates-type-i-collagen-synthesis-post-transcriptionally-and-is-essential-for-lung-myofibroblast-differentiation
#5
Ioannis Papaioannou, Shiwen Xu, Christopher P Denton, David J Abraham, Markella Ponticos
Fibroblast differentiation is key cellular process that underlies the process of fibrosis, a deadly complication of fibrotic diseases like Scleroderma (SSc). This transition coincides with the overproduction of Collagen type I (COL1) and other extracellular matrix proteins. High level expression of the collagen type 1α2 subunit (COL1A2), requires the engagement of a far upstream enhancer, whose activation is strongly dependent on the AP1 factor JunB. We now report that STAT3 also binds the COL1A2 enhancer and is essential for RNA polymerase recruitment, without affecting JunB binding...
November 15, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/29135399/-investigation-of-parameters-related-to-prognosis-in-diffuse-parenchymal-lung-diseases-prognosis-in-interstitial-lung-diseases
#6
Coşkun Canıvar, Züleyha Bingöl, Zeki Kılıçaslan, Tülin Çağatay, N Gülfer Okumuş
Introduction: Parameters related to prognosis in diffuse parenchymal lung disease (DPLD) have a decisive influence on treatment and follow-up processes. We aimed to define baseline characteristics and factors that effect the mortality of the group of patients with DPLD and to determine distinctions between subgroups. Materials and Methods: Demographic characteristics, complaints, comorbidity, treatment, pulmonary function tests, echocardiographic findings, six minute walking test (6MWT), arterial blood gases analysis, radiological findings and survival time were collected from outpatient clinics database...
September 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/29104809/mycobacterium-intracellulare-infection-mimicking-progression-of-scleroderma
#7
Simon Krabbe, Merete Engelhart, Sören Thybo, Søren Jacobsen
This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal nail fold capillaries, interstitial lung disease, Raynaud's phenomenon, esophageal dysmotility, and positivity for rheumatoid factor and anti-SSA antibodies. She developed massive inflammatory changes of the cutis, the subcutis, and the muscle fasciae of the right leg, that after several failed attempts of immunosuppressive treatments were found to be caused by Mycobacterium intracellulare...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29093152/pomalidomide-in-patients-with-interstitial-lung-disease-due-to-systemic-sclerosis-a-phase-ii-multicenter-randomized-double-blind-placebo-controlled-parallel-group-study
#8
Vivien M Hsu, Christopher P Denton, Robyn T Domsic, Daniel E Furst, Maureen Rischmueller, Marina Stanislav, Virginia D Steen, Jörg H W Distler, Shimon Korish, Alyse Cooper, Suktae Choi, Peter H Schafer, Gerald Horan, Douglas R Hough
OBJECTIVE: To evaluate the safety and efficacy of pomalidomide (POM) on forced vital capacity (FVC), modified Rodnan skin score (mRSS), and gastrointestinal (GI) symptomatology over 52 weeks of treatment in patients with interstitial lung disease due to systemic sclerosis (SSc). METHODS: Twenty-three adult patients diagnosed with SSc were randomized 1:1 POM:placebo (PBO). RESULTS: Mean change at Week 52 from baseline in predicted FVC% -5.2 and -2...
November 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/29056770/assessment-of-hospitalization-and-mortality-of-scleroderma-in-patients-a-thirteen-year-study
#9
Saeedeh Shenavandeh, Razieh Naseri
OBJECTIVE: Systemic sclerosis (SSc) is an uncommon non-hereditary sporadic disease that increases the risk of premature death, especially in diffuse type. We determined the prevalence of SSc in the last 13 years in our rheumatologic hospitals as a referral center for southern Iranian patients, the causes of hospitalization, the average length of stay (LOS), the mortality rate, and the reason for their mortality. MATERIAL AND METHODS: A cross-sectional study was performed in Shiraz University of Medical Sciences, Iran...
2017: Reumatologia
https://www.readbyqxmd.com/read/29055411/early-life-gut-dysbiosis-a-driver-of%C3%A2-later-life-fibrosis
#10
Karen J Ho, John Varga
Using a novel mouse model of scleroderma induced by immunization with topoisomerase-I peptide-loaded dendritic cells, Mehta et al. found that early-life antibiotic exposure resulted in increased later-life fibrosis in the skin and lungs. These observations advance the novel concept that gut microbiome alterations caused by early-life exposures may contribute to scleroderma pathogenesis, and warrant in-depth characterization and validation in complementary disease models.
November 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28994016/cardiopulmonary-manifestations-of-collagen-vascular-diseases
#11
REVIEW
Hamza Jawad, Sebastian R McWilliams, Sanjeev Bhalla
PURPOSE OF REVIEW: The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren's syndrome. RECENT FINDINGS: Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population...
October 9, 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28980912/influence-of-antibody-profile-in-clinical-features-and-prognosis-in-a-cohort-of-spanish-patients-with-systemic-sclerosis
#12
Nerea Iniesta Arandia, Carmen Pilar Simeón-Aznar, Alfredo Guillén Del Castillo, Dolores Colunga Argüelles, Manuel Rubio-Rivas, Luis Trapiella Martínez, Francisco José García Hernández, Luis Sáez Comet, María Victoria Egurbide Arberas, Norberto Ortego-Centeno, Mayka Freire, Begoña Marí Alfonso, José Antonio Vargas Hitos, Juan José Ríos Blanco, José Antonio Todolí Parra, Monica Rodríguez-Carballeira, Adela Marín Ballvé, Antonio Javier Chamorro Fernández, Xavier Pla Salas, Ana Belen Madroñero Vuelta, Manuel Ruiz Muñoz, Vicent Fonollosa Pla, Gerard Espinosa
OBJECTIVES: To assess the clinical manifestations and prognosis of Spanish patients with systemic sclerosis (SSc) according to their immunological profile. METHODS: From the Spanish Scleroderma Study Group or RESCLE (Registro de ESCLErodermia as Spanish nomenclature) Registry we selected those patients in which anti-centromere (ACA), anti-topoisomerase I (ATA), and anti-RNA polymerase III (ARA) antibodies had been determined, and a single positivity for each SSc specific antibody was detected...
September 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28935460/myocardial-fibrosis-detected-by-magnetic-resonance-in-systemic-sclerosis-patients-relationship-with-biochemical-and-echocardiography-parameters
#13
Milan Hromádka, Jitka Seidlerová, David Suchý, Daniel Rajdl, Jan Lhotský, Jaroslav Ludvík, Richard Rokyta, Jan Baxa
OBJECTIVES: Systemic scleroderma (SSc) is a rare connective tissue disease presenting with fibrosis affecting skin and internal organs. Cardiovascular magnetic resonance (CMR) with quantification of extracellular volume (ECV) and T1 mapping might help to detect heart involvement. We aimed to evaluate whether myocardial involvement correlates with functional and laboratory parameters. METHODS: Thirty-three asymptomatic SSc patients (29 women, aged 56.6±12.2years) and 20 controls (10 women, 53...
December 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28913656/new-insights-into-cardiac-involvement-in-juvenile-scleroderma-a-three-dimensional-echocardiographic-assessment-unveils-subclinical-ventricle-dysfunction
#14
Reyhan Dedeoglu, Amra Adroviç, Funda Oztunç, Sezgin Sahin, Kenan Barut, Ozgur Kasapcopur
Cardiac manifestations in juvenile scleroderma or systemic sclerosis (JSSc) have poor prognosis, begin in early stages of the disease, and remain clinically asymptomatic. New echocardiography modalities, such as 2D/3D speckle tracking (STE, strain analysis for regional and global ventricular functions), can detect cardiac involvement in early stages. We assessed 21 JSSc patients and 19 controls using 2D/3D STE. The left ventricular end diastolic volume, end systolic volume, and ejection fraction of the patient and control groups were significantly different (99...
December 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28831601/endothelin-1-%C3%AE-klotho-25-oh-vit-d-levels-and-severity-of-disease-in-scleroderma-patients
#15
Mehrzad Hajialilo, Parisa Noorabadi, Sepideh Tahsini Tekantapeh, Aida Malek Mahdavi
Considering the role of endothelin-1 (ET-1) in tissue remodeling and fibrosis during the development of scleroderma as well as the effect of α-Klotho in pathogenesis of calcinosis and/or endothelial cell injury and its correlation with severity of disease, this study aimed to evaluate serum ET-1, α-Klotho and 25(OH) vitamin D levels in patients with limited and diffuse scleroderma compared to healthy subjects. In this cross-sectional study, 60 scleroderma patients according to the ACR/EULAR 2013 criteria and 60 age- and sex-matched healthy controls were included...
October 2017: Rheumatology International
https://www.readbyqxmd.com/read/28761806/scleroderma-related-interstitial-lung-disease
#16
Sally Suliman, Abdalhamid Al Harash, William Neil Roberts, Rafael L Perez, Jesse Roman
Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. Although certain immunosuppressive therapies may slow disease progression, current treatment strategies are not curative; consequently, SSc-ILD continues to be a major cause of morbidity and mortality. We present four cases of SSc-ILD that emphasize the importance of early screening and detection, close follow-up, and aggressive management...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28668810/an-mif-promoter-polymorphism-is-associated-with-susceptibility-to-pulmonary-arterial-hypertension-in-diffuse-cutaneous-systemic-sclerosis
#17
Lara Bossini-Castillo, Diana Campillo-Davó, Elena López-Isac, Francisco David Carmona, Carmen P Simeon, Patricia Carreira, José Luis Callejas-Rubio, Iván Castellví, Antonio Fernández-Nebro, Luis Rodríguez-Rodríguez, Manel Rubio-Rivas, Francisco J García-Hernández, Ana Belén Madroñero, Lorenzo Beretta, Alessandro Santaniello, Claudio Lunardi, Paolo Airó, Anna-Maria Hoffmann-Vold, Alexander Kreuter, Gabriela Riemekasten, Torsten Witte, Nicolas Hunzelmann, Madelon C Vonk, Alexandre E Voskuyl, J de Vries-Bouwstra, Paul Shiels, Ariane Herrick, Jane Worthington, Timothy R D J Radstake, Javier Martin
OBJECTIVE: Systemic sclerosis (SSc) is a fibrotic immune-mediated disease of unknown etiology. Among its clinical manifestations, pulmonary involvement is the leading cause of mortality in patients with SSc. However, the genetic factors involved in lung complication are not well defined. We aimed to review the association of the MIF gene, which encodes a cytokine implicated in idiopathic pulmonary hypertension among other diseases, with the susceptibility and clinical expression of SSc, in addition to testing the association of this polymorphism with SSc-related pulmonary involvement...
October 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28664832/clinical-determinants-of-elevated-systolic-pulmonary-artery-pressure-measured-by-transthoracic-doppler-echocardiography-in-early-systemic-sclerosis
#18
Patricia E Carreira, Loreto Carmona, Beatriz E Joven, Estibaliz Loza, Jose Luis Andreu, Gabriela Riemekasten, Serena Vettori, Yannick Allanore, Alexandra Balbir-Gurman, Paolo Airò, Ulrich A Walker, Nemanja Damjanov, Lidia P Ananieva, Simona Rednic, László Czirják, Oliver Distler, Dominique Farge, Roger Hesselstrand, Ada Corrado, Paola Caramaschi, Mohammed Tikly, Marco Matucci-Cerinic
OBJECTIVES: To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Doppler-echocardiography (TTE) in patients with early systemic sclerosis (SSc). METHODS: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research (EUSTAR) database was performed. SSc patients with <3 years from the first non-Raynaud's phenomenon (RP) symptom at baseline EUSTAR visit, were selected...
June 20, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28654764/deep-proteome-profiling-reveals-common-prevalence-of-mzb1-positive-plasma-b-cells-in-human-lung-and-skin-fibrosis
#19
Herbert B Schiller, Christoph H Mayr, Gabriela Leuschner, Maximilian Strunz, Claudia Staab-Weijnitz, Stefan Preisendörfer, Beate Eckes, Pia Moinzadeh, Thomas Krieg, David A Schwartz, Rudolf A Hatz, Jürgen Behr, Matthias Mann, Oliver Eickelberg
RATIONALE: Analyzing the molecular heterogeneity of different forms of organ fibrosis may reveal common and specific factors and thus identify potential future therapeutic targets. OBJECTIVES: We sought to use proteome-wide profiling of human tissue fibrosis to (1) identify common and specific signatures across endstage interstitial lung disease (ILD) cases, (2) characterize ILD subgroups in an unbiased fashion, and (3) identify common and specific features of lung and skin fibrosis...
June 27, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28645009/case-report-of-robotic-dor-fundoplication-for-scleroderma-esophagus-with-aperistalsis-on-manometry
#20
Alonso Andrade, Matthew K Folstein, Brian R Davis
Scleroderma is a systemic disease of collagen deposition resulting in fibrosis of small arteries and arterioles. It commonly affects the skin, lungs, and gastrointestinal tract. The most common site of GI tract involvement is the esophagus. We present the case report of a 44year old female with scleroderma esophagus and severe reflux which was successfully treated with robotic dor fundoplication. Because of the wide variety of symptoms with which this problem can present, a tailored approach taking into consideration the patient's symptomatology and findings during diagnostic work-up was implemented with good results...
2017: International Journal of Surgery Case Reports
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