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lung activity disease systemic sclerosis

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https://www.readbyqxmd.com/read/29771006/the-anti-fibrotic-effect-of-adenosine-a2b-receptor-antagonism-in-a-mouse-model-of-dermal-fibrosis
#1
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
https://www.readbyqxmd.com/read/29760620/anti-endothelial-cell-antibodies-do-not-correlate-with-disease-activity-in-systemic-sclerosis
#2
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
https://www.readbyqxmd.com/read/29697389/-rheological-properties-of-blood-serum-at-systemic-sclerosis
#3
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
https://www.readbyqxmd.com/read/29623076/autoantibodies-in-serum-of-systemic-scleroderma-patients-peptide-based-epitope-mapping-indicates-increased-binding-to-cytoplasmic-domains-of-cxcr3
#4
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
https://www.readbyqxmd.com/read/29616048/tnfsf14-light-exhibits-inflammatory-activities-in-lung-fibroblasts-complementary-to-il-13-and-tgf-%C3%AE
#5
Ricardo da Silva Antunes, Amit K Mehta, Lisa Madge, Joel Tocker, Michael Croft
The cytokine TNFSF14 [homologous to Lymphotoxin, exhibits Inducible expression and competes with HSV Glycoprotein D for binding to HVEM, a receptor expressed on T lymphocytes (LIGHT)] has been shown in mouse models to be important for development of lung tissue remodeling that is characteristic of asthma, idiopathic pulmonary fibrosis (IPF), and systemic sclerosis (SSc). However, its cellular targets are not fully delineated. In the present report, we show that LTβR and HVEM, the receptors for LIGHT, are constitutively expressed in primary human lung fibroblasts (HLFs)...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29579252/epigenetic-modulation-as-a-therapy-in-systemic-sclerosis
#6
Steven O'Reilly
SSc is an autoimmune idiopathic disease in which there is an inflammatory component driving fibrosis. The chief cell involved is the myofibroblast, which when activated secretes copious amounts of extracellular matrix that forms deposits, leading to stiffness and fibrosis. The fibrosis is most prevalent in the skin and lungs. In recent years epigenetic modifications have been uncovered that positively and negatively regulate the genesis of the myofibroblasts and that can be activated and regulated by a variety of cytokines and hormones...
March 22, 2018: Rheumatology
https://www.readbyqxmd.com/read/29559981/gene-profiling-in-patients-with-systemic-sclerosis-reveals-the-presence-of-oncogenic-gene-signatures
#7
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
https://www.readbyqxmd.com/read/29507108/rgc32-promotes-bleomycin-induced-systemic-sclerosis-in-a-murine-disease-model-by-modulating-classically-activated-macrophage-function
#8
Chenming Sun, Shi-You Chen
Systemic sclerosis (SSc) is a multisystem autoimmune disorder that is characterized by inflammation and fibrosis in the skin and internal organs. Previous studies indicate that inflammatory cells and cytokines play essential roles in the pathogenesis of SSc; however, the mechanisms that underlie the inflammation-driven development of SSc are not fully understood. In this study, we show that response gene to complement 32 (RGC32) is abundantly expressed in mouse macrophages in the early stage of bleomycin-induced SSc...
April 15, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29447111/free-light-chains-of-immunoglobulins-in-patients-with-systemic-sclerosis-correlations-with-lung-involvement-and-inflammatory-milieu
#9
Silvia Bosello, Umberto Basile, Enrico De Lorenzis, Francesca Gulli, Giovanni Canestrari, Cecilia Napodano, Federico Parisi, Krizia Pocino, Clara Di Mario, Barbara Tolusso, Gianfranco Ferraccioli, Elisa Gremese
AIM: Humoral immunity and B cells are thought to play an important role in the pathophysiology of the systemic sclerosis (SSc). The production of free light chains (FLC) of immunoglobulins is abnormally high in several pathological autoimmune conditions and reflects B cell activation. Furthermore, FLCs demonstrated different biological activities including their capability to modulate the immune system, proteolytic activity and complement cascade activation. The aims of this study are to determine the FLC levels in patients with SSc compared with healthy controls (HC) and to study their possible association with organ involvement and disease characteristics...
January 31, 2018: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29385710/a-review-of-the-catalytic-mechanism-of-human-manganese-superoxide-dismutase
#10
REVIEW
Jahaun Azadmanesh, Gloria E O Borgstahl
Superoxide dismutases (SODs) are necessary antioxidant enzymes that protect cells from reactive oxygen species (ROS). Decreased levels of SODs or mutations that affect their catalytic activity have serious phenotypic consequences. SODs perform their bio-protective role by converting superoxide into oxygen and hydrogen peroxide by cyclic oxidation and reduction reactions with the active site metal. Mutations of SODs can cause cancer of the lung, colon, and lymphatic system, as well as neurodegenerative diseases such as Parkinson's disease and amyotrophic lateral sclerosis...
January 30, 2018: Antioxidants (Basel, Switzerland)
https://www.readbyqxmd.com/read/29322341/interstitial-lung-disease-in-systemic-sclerosis-data-from-the-spanish-scleroderma-study-group
#11
D Sánchez-Cano, N Ortego-Centeno, J L Callejas, V Fonollosa Plá, R Ríos-Fernández, C Tolosa-Vilella, G Espinosa-Garriga, D Colunga-Argüelles, M V Egurbide-Arberas, M Rubio-Rivas, M Freire, J J Ríos-Blanco, L Trapiella-Martínez, M Rodríguez-Carballeira, A Marín-Ballvé, X Pla-Salas, C P Simeón-Aznar
OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group...
March 2018: Rheumatology International
https://www.readbyqxmd.com/read/29316328/anti-fibrotic-effects-of-pirfenidone-by-interference-with-the-hedgehog-signalling-pathway-in-patients-with-systemic-sclerosis-associated-interstitial-lung-disease
#12
Hua Xiao, Guang-Feng Zhang, Xiang-Ping Liao, Xiao-Jie Li, Jian Zhang, Haobo Lin, Zhe Chen, Xiao Zhang
AIM: To determine whether pirfenidone attenuates lung fibrosis by interfering with the hedgehog (Hh) signalling pathway in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). METHODS: Twenty-five SSc-ILD patients (20 first visit, five who underwent pirfenidone treatment for 6 months) and 10 healthy controls were recruited. Lung tissues were obtained by open-chest surgery, and primary lung fibroblasts were isolated, cultured and stimulated with pirfenidone...
February 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29246248/factors-associated-with-the-6-minute-walk-distance-in-patients-with-systemic-sclerosis
#13
Sébastien Sanges, Jonathan Giovannelli, Vincent Sobanski, Sandrine Morell-Dubois, Hélène Maillard, Marc Lambert, Céline Podevin, Nicolas Lamblin, Pascal De Groote, Jean-François Bervar, Thierry Perez, Régis Matran, Martine Rémy-Jardin, Pierre-Yves Hatron, Éric Hachulla, David Launay
BACKGROUND: There is an ongoing debate regarding the relevance of the 6-minute walking distance (6MWD) in systemic sclerosis (SSc) assessment, widely used as a usual test in these patients as well as an outcome measure in clinical trials. In this work, we aimed to assess the associations between the 6MWD and various disease parameters in patients with SSc. METHODS: Consecutive patients followed in our SSc National Reference Centre were included in this cross-sectional study if they fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism criteria for SSc...
December 15, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29196890/malnutrition-and-sarcopenia-in-a-large-cohort-of-patients-with-systemic-sclerosis
#14
C Caimmi, P Caramaschi, A Venturini, E Bertoldo, E Vantaggiato, O Viapiana, M Ferrari, G Lippi, L Frulloni, M Rossini
Systemic sclerosis (SSc) is an autoimmune disease which may lead to malnutrition. Previous studies have defined it with different criteria. No thorough evaluations of sarcopenia in SSc are available. The aim of the present study was to assess the prevalence and the potential association of malnutrition and sarcopenia in a large cohort of SSc cases. A total of 141 SSc consecutive outpatients were enrolled. Body composition was analyzed by densitometry. Malnutrition was defined according to recently published ESPEN criteria, whereas sarcopenia was diagnosed in patients with reduced skeletal muscle index...
December 1, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29164837/serum-surfactant-protein-d-and-exhaled-nitric-oxide-as-biomarkers-of-early-lung-damage-in-systemic-sclerosis
#15
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...
April 2018: Minerva Medica
https://www.readbyqxmd.com/read/29130325/the-plasma-levels-of-adamts-13-von-willebrand-factor-vwfpp-and-fibrin-related-markers-in-patients-with-systemic-sclerosis-having-thrombosis
#16
Koji Habe, Hideo Wada, Ayaka Higashiyama, Tomoko Akeda, Kenshiro Tsuda, Ryoko Mori, Masato Kakeda, Takeshi Matsumoto, Kohshi Ohishi, Keiichi Yamanaka, Naoyuki Katayama, Hitoshi Mizutani
This study aimed to examine the hemostatic abnormalities in patients with systemic sclerosis (SSc) and the relationship between these abnormalities and thrombotic events (THEs), focusing on the difference in diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc). The plasma levels of ADAMTS-13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13), von Willebrand factor (VWF), VWF propeptide (VWFpp), d-dimer, and soluble fibrin (SF) were measured in 233 patients with SSc. The relationship between their levels and organ involvement, including THEs and interstitial lung disease (ILD), was evaluated...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29045979/-changes-of-serum-krebs-von-den-lungen-6-levels-in-interstitial-lung-disease-associated-with-dermatomyositis-and-secondary-sj%C3%A3-gren-s-syndrome-a-case-report
#17
J F Yu, Y B Jin, J He, Y An, Z G Li
Interstitial lung diseases (ILDs) are a diverse group of pulmonary disorders characterized by various patterns of inflammation and fibrosis in the interstitium of the lung. The underlying pathogenesis of ILDs is complex and associated with multiple rheumatologic conditions, such as systemic sclerosis, rheumatoid arthritis, pollymyositis and dermatomyositis, Sjögren's syndrome, and systemic lupus erythematosus. As the disease progresses, excessive pulmonary fibrosis impairs alveolar gas exchange and damages pulmonary function...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29044628/possible-pro-inflammatory-role-of-heparin-binding-epidermal-growth-factor-like-growth-factor-in-the-active-phase-of-systemic-sclerosis
#18
Megumi Hirabayashi, Yoshihide Asano, Takashi Yamashita, Shunsuke Miura, Kouki Nakamura, Takashi Taniguchi, Ryosuke Saigusa, Takehiro Takahashi, Yohei Ichimura, Takuya Miyagawa, Ayumi Yoshizaki, Tomomitsu Miyagaki, Makoto Sugaya, Shinichi Sato
Heparin-binding epidermal growth factor (EGF)-like growth factor (HB-EGF) is a member of the EGF family growth factors, which affects multiple aspects of the wound healing process such as epithelialization, wound contraction and angiogenesis. In our study, we measured the serum HB-EGF levels of 51 systemic sclerosis (SSc) patients, which showed a significant increase compared with those of 20 normal subjects. Further analysis revealed a positive correlation between the HB-EGF level and pulmonary ground-glass score but no correlation between the former and pulmonary fibrosis score...
February 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/29037314/pulmonary-magnetic-resonance-imaging-is-similar-to-chest-tomography-in-detecting-inflammation-in-patients-with-systemic-sclerosis
#19
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 (SSc) 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 SSc 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...
September 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28919455/is-there-a-role-for-il-17-in-the-pathogenesis-of-systemic-sclerosis
#20
REVIEW
Carlo Chizzolini, Aleksandra Maria Dufour, Nicolò Costantino Brembilla
In systemic sclerosis (SSc) immuno-inflammatory events are central to disease development. Amongst other mediators of inflammation, interleukin 17 (IL-17) and Th17 cells have been reported to be increased in the peripheral blood and target organs including involved skin in SSc. They participate and amplify inflammatory responses by inducing the production of cytokines such as IL-6, chemokines such as CCL2 and CXCL8 (IL-8), matrix metalloproteinases-1, -2, -9 and the expression of adhesion molecules in stromal cells including fibroblasts and endothelial cells...
March 2018: Immunology Letters
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