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Scleroderma

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https://www.readbyqxmd.com/read/28631695/-chronic-stress-and-mental-disorders-in-patients-with-systemic-scleroderma-results-of-an-interdisciplinary-study
#1
O F Seravina, T A Lisitsyna, M N Starovoytova, O V Desinova, O B Kovalevskaya, D Yu Veltishchev
AIM: To analyze of the prevalence of stressful factors and mental disorders (MDs), as well as their clinical psychopathological and clinical psychological characteristics to improve the comprehensive diagnosis and treatment of systemic scleroderma (SSD). SUBJECTS AND METHODS: Examinations were performed in 110 patients (predominantly women (n=97 (88.2%); mean age, 49.9±2.47 years) with a documented diagnosis of SSD (its mean duration, 7.25±0.42 years). 62 (56...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28629914/mortality-risk-prediction-in-scleroderma-related-interstitial-lung-disease-the-sadl-model
#2
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/28628466/evaluation-of-cancer-associated-myositis-and-scleroderma-autoantibodies-in-breast-cancer-patients-without-rheumatic-disease
#3
Ami A Shah, Antony Rosen, Laura K Hummers, Betty J May, Alpana Kaushiva, Richard B S Roden, Deborah K Armstrong, Fredrick M Wigley, Livia Casciola-Rosen, Kala Visvanathan
OBJECTIVES: Systemic sclerosis (scleroderma) and dermatomyositis are two prototypic autoimmune diseases that are strongly associated with malignancy. While specific autoantibodies in these diseases are markers of an increased risk of cancer at scleroderma and dermatomyositis onset, it is not known whether these autoantibodies are biomarkers of cancer risk in patients without rheumatic disease. METHODS: In a matched case-control study of women without rheumatic disease, identified from a familial breast cancer cohort, 50 breast cancer cases and 50 controls were assayed for 3 autoantibodies that are known markers of cancer-associated scleroderma and dermatomyositis: anti-RNA polymerase III, anti-NXP2, and anti-TIF1γ...
June 19, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28624173/high-resolution-manometry-compared-with-the-university-of-california-los-angeles-scleroderma-clinical-trials-consortium-git-2-0-in-systemic-sclerosis
#4
Hanan Sayed M Abozaid, Hala M K Imam, Marwa Mahmoud Abdelaziz, Dina H El-Hammady, Nihal A Fathi, Daniel E Furst
OBJECTIVES: To study esophageal high resolution manometry (HRM) in systemic sclerosis (SSc) patients and the correlation of findings to The University of California, Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA SCTC_GIT 2.0). METHODS: Forty SSc patients were administered to the UCLA SCTC GIT 2.0. Patients underwent HRM study (Solar GI MMS). HRM data were compared with 15 healthy volunteers. RESULTS: Forty patients with mean age 46 ± 7 years and disease duration 9...
May 19, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28621173/evaluation-and-management-approaches-for-scleroderma-lung-disease
#5
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
#6
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/28610600/the-cumulative-number-of-micro-haemorrhages-and-micro-thromboses-in-nailfold-videocapillaroscopy-is-a-good-indicator-of-disease-activity-in-systemic-sclerosis-a-validation-study-of-the-nemo-score
#7
Romina Andracco, Rosaria Irace, Eleonora Zaccara, Serena Vettori, Wanda Maglione, Antonella Riccardi, Francesca Pignataro, Roberta Ferrara, Domenico Sambataro, Gianluca Sambataro, Claudio Vitali, Gabriele Valentini, Nicoletta Del Papa
BACKGROUND: Some abnormalities in nailfold videocapillaroscopy (NVC), such as the presence of micro-haemorrhages (MHEs), micro-thromboses (MTs), giant capillaries (GCs) and reduction in the number of capillaries (nCs), suggest a disease activity (DA) phase in systemic sclerosis (SSc). In a previous paper, we showed that the number of micro-haemorrhages and micro-thromboses (the so-called NEMO score) was the NVC feature more closely associated with DA. The present study was aimed at validating the NEMO score as a measure of DA in patients with SSc...
June 13, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28606036/scleroderma-like-disorders
#8
Amit Sharma
Scleroderma is a term used to describe diseases that involve hardening and tightening of the skin and the underlying subcutaneous connective tissue. It could be localized to skin and subcutaneous tissue, or may involve the internal organs too in systemic sclerosis. There are disorders that can cause hardening and tightening of skin and mimic scleroderma but are rarely associated with Raynaud phenomenon, sclerodactyly, and autoantibodies in the serum, features specific to scleroderma/systemic sclerosis. These are termed as "scleroderma variants" or "scleroderma like disorders"...
June 11, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28604345/scleroderma-lung-involvement-autoantibodies-and-outcome-prediction-the-confounding-effect-of-time
#9
EDITORIAL
Svetlana I Nihtyanova, Christopher P Denton
No abstract text is available yet for this article.
April 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28602918/angiogenic-and-angiostatic-factors-in-renal-scleroderma-associated-vasculopathy
#10
Antonietta Gigante, Navarini Luca, Margiotta Domenico, Amoroso Antonio, Barbano Biagio, Cianci Rosario, Afeltra Antonella, Rosato Edoardo
BACKGROUND: The angiogenesis in systemic sclerosis (SSc) is impaired. An imbalance of pro-angiogenic factors and angiogenesis inhibitors has been implicated in the progression of peripheral microvascular damage, defective vascular repair and fibrosis. Intrarenal resistance index are considered markers of renal vasculopathy. The aim of the study is to evaluate angiogenic and angiostatic factors (VEGF and endostatin) in SSc patients and to correlate with intrarenal hemodynamic parameters...
June 8, 2017: Microvascular Research
https://www.readbyqxmd.com/read/28599746/scleroderma-induced-by-pembrolizumab-a-case-series
#11
Naiara S Barbosa, David A Wetter, Carilyn N Wieland, Niraj K Shenoy, Svetomir N Markovic, Uma Thanarajasingam
Immune checkpoint inhibitors are approved for select cancer treatment and have shown survival benefit in patients with advanced melanoma. Adverse events, including immune-related adverse events, are common and potentially life-threatening. We describe cases of 2 patients with scleroderma (patient 1 had diffuse scleroderma, and patient 2 had limited scleroderma) that developed while they were receiving pembrolizumab therapy for metastatic melanoma. Prompt recognition and treatment of immune-related adverse events may improve tolerance to immune checkpoint inhibitors and contribute to an understanding of the manifesting autoimmune disease...
June 6, 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28597765/poor-survival-in-patients-with-scleroderma-and-pulmonary-hypertension-due-to-heart-failure-with-preserved-ejection-fraction
#12
Khalil I Bourji, Benjamin W Kelemen, Stephen C Mathai, Rachel L Damico, Todd M Kolb, Valentina Mercurio, Franco Cozzi, Ryan J Tedford, Paul M Hassoun
Pulmonary hypertension due to heart failure with preserved ejection fraction (PH-HFpEF) has been poorly studied in patients with systemic sclerosis (SSc). We sought to compare clinical characteristics and survival of SSc patients with PH-HFpEF (SSc-PH-HFpEF) versus pulmonary arterial hypertension (SSc-PAH). We hypothesized that patients with SSc-PH-HFpEF have a similar poor overall prognosis compared with patients with SSc-PAH when matched for total right ventricular load. The analysis included 117 patients with SSc-PH (93 with SSc-PAH versus 24 with SSc-PH-HFpEF) enrolled prospectively in the Johns Hopkins PH Registry...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28587630/pulmonary-arterial-hypertension-in-scleroderma-care-gaps-in-screening
#13
EDITORIAL
Janet E Pope
One in six patients with systemic sclerosis will develop pulmonary arterial hypertension (PAH). Screening with echocardiography and possibly pulmonary function testing (to determine the diffusing capacity of carbon monoxide) is recommended to detect PAH at a less severe stage. However, real-world screening programs have problems. Registries where echocardiograms are to be performed annually should have the best-case scenario of nearly perfect screening and referral for right heart catheterization of those highly suspect for PAH...
June 6, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28586109/fyn-kinase-mediates-cortical-actin-ring-depolymerization-required-for-mast-cell-migration-in-response-to-tgf-%C3%AE-in-mice
#14
Karla A Ramírez-Valadez, Genaro Vázquez-Victorio, Marina Macías-Silva, Claudia González-Espinosa
Transforming Growth Factor-β (TGF-β) is a potent mast cell (MC) chemoattractant able to modulate local inflammatory reactions. The molecular mechanism leading to TGF-β-directed MC migration is not fully described. Here we analyzed the role of the Src family protein kinase Fyn on the main TGF-β-induced cytoskeletal changes leading to MC migration. Utilizing bone marrow-derived mast cells (BMMCs) from WT and Fyn-deficient mice we found that BMMC migration to TGF-β was impaired in the absence of the kinase...
June 6, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28580628/mortality-in-the-waikato-hospital-systemic-sclerosis-cohort
#15
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
#16
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
#17
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/28572048/the-role-of-endothelial-cells-in-the-vasculopathy-of-systemic-sclerosis-a-systematic-review
#18
REVIEW
Y Mostmans, M Cutolo, C Giddelo, S Decuman, K Melsens, H Declercq, E Vandecasteele, F De Keyser, O Distler, J Gutermuth, V Smith
INTRODUCTION: Systemic sclerosis (SSc) is an autoimmune connective tissue disorder characterized by fibroproliferative vasculopathy, immunological abnormalities and progressive fibrosis of multiple organs including the skin. In this study, all English speaking articles concerning the role of endothelial cells (ECs) in SSc vasculopathy and representing biomarkers are systematically reviewed and categorized according to endothelial cell (EC) (dys)function in SSc. METHODS: A sensitive search on behalf of the EULAR study group on microcirculation in Rheumatic Diseases was developed in Pubmed, The Cochrane Library and Web of Science to identify articles on SSc vasculopathy and the role of ECs using the following Mesh terms: (systemic sclerosis OR scleroderma) AND pathogenesis AND (endothelial cells OR marker)...
May 29, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28566901/periostin-in-mature-stage-localized-scleroderma
#19
Min-Woo Kim, Jung Tae Park, Jung Ho Kim, Seong-Joon Koh, Hyun-Sun Yoon, Soyun Cho, Hyun-Sun Park
BACKGROUND: Periostin is a novel matricellular protein expressed in many tissues, including bone, periodontal ligament, and skin. Although its expression is prominent in various fibrotic conditions, studies of periostin in localized scleroderma are rare. OBJECTIVE: To investigate the expression of periostin and other molecules in localized scleroderma. METHODS: A retrospective study of 14 patients with confirmed mature stage localized scleroderma was undertaken...
June 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28564618/very-early-and-early-systemic-sclerosis-in-the-spanish-scleroderma-registry-rescle-cohort
#20
REVIEW
Luis Trapiella-Martínez, José Bernardino Díaz-López, Luis Caminal-Montero, Carles Tolosa-Vilella, Alfredo Guilléndel Castillo, Dolores Colunga-Argüelles, Manuel Rubio-Rivas, 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, Antonio Javier Chamorro-Fernández, Xavier Pla-Salas, Ana Belén Madroñero-Vuelta, Manuel Ruiz-Muñóz, Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar
OBJECTIVES: According to the existence of subclinical organ involvement pre-scleroderma should be divided into two subsets: very early and early disease. Pre-scleroderma patients included in the Spanish Scleroderma Registry (RESCLE) Cohort were reclassified into subsets. Differences were evaluated and the risk of progression to definite systemic sclerosis was estimated. METHODS: The characteristics of very early and early SSc patients were compared. A logistic regression model was used to determine the risk factors of progression...
May 28, 2017: Autoimmunity Reviews
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