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Scleroderma

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https://www.readbyqxmd.com/read/29782295/diagnostic-and-treatment-strategies-of-dermatologists-for-treating-morphea-in-hungary
#1
Gábor Bali, Bernadett Hidvégi
Localized scleroderma is an uncommon disease, only infrequently encountered by dermatologists in private practices or even in larger academic centers. Because of its rarity, current treatment guidelines are mostly based on low-level clinical evidence and expert opinions. The aim of this study was to evaluate treatment strategies to treat localized scleroderma. A questionnaire was developed and sent to dermatologists in Hungary. 101 returned questionnaires were eligible for evaluation. 87.12% of clinicians employed local steroids...
April 2018: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/29781588/smoking-in-systemic-sclerosis-a-longitudinal-european-scleroderma-trials-and-research-group-study
#2
Veronika K Jaeger, Gabriele Valentini, Eric Hachulla, Franco Cozzi, Oliver Distler, Paolo Airó, Laszlo Czirják, Yannick Allanore, Elise Siegert, Edoardo Rosato, Marco Matucci-Cerinic, Cristian Caimmi, Jörg Henes, Patricia E Carreira, Vanessa Smith, Francesco Del Galdo, Christopher P Denton, Susanne Ullman, Ellen De Langhe, Valeria Riccieri, Juan J Alegre-Sancho, Simona Rednic, Ulf Müller-Ladner, Ulrich A Walker
OBJECTIVES: Data on the role of tobacco exposure in systemic sclerosis (SSc) severity and progression are scarce. We aimed to assess the effects of smoking on the evolution of pulmonary and skin manifestations in the EUSTAR database. METHODS: Adult SSc patients with data on smoking history and a 12-24 months follow-up visit were included. Associations of severity and progression of organ involvement with smoking history and the comprehensive smoking index (CSI) were assessed using multivariable regression analyses...
May 21, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29781586/treatment-algorithms-for-systemic-sclerosis-according-to-experts
#3
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
https://www.readbyqxmd.com/read/29781576/ultrasonography-for-the-assessment-of-skin-in-systemic-sclerosis-a-systematic-review
#4
Tânia Santiago, Mariana Santiago, Barbara Ruaro, Maria João Salvador, Maurizio Cutolo, J A P da Silva
OBJECTIVE: To identify and synthesise the best available evidence on the use of ultrasound to assess skin involvement in systemic sclerosis (SSc). METHODS: We conducted a systemic review of the literature on Pubmed MEDLINE and Embase, using the vocabulary terms: ('systemic sclerosis OR scleroderma') AND ('ultrasonography' OR 'Elasticity Imaging Techniques') AND ('skin' OR 'dermis'). Two independent reviewers selected articles, collected data from studies and carried out a manual search of the references from the studies included...
May 21, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29777655/long-term-outcomes-in-systemic-sclerosis-associated-pulmonary-arterial-hypertension-from-the-pulmonary-hypertension-assessment-and-recognition-of-outcomes-in-scleroderma-registry-pharos
#5
Kathleen D Kolstad, Shufeng Li, Virginia Steen, Lorinda Chung
BACKGROUND: Pulmonary arterial hypertension (PAH) is a leading cause of death in patients with systemic sclerosis (SSc). The purpose of this study was to assess long-term outcomes in patients with SSc-PAH. METHODS: Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma is a prospective registry of SSc patients at high risk for or with incident pulmonary hypertension based on right heart catheterization (RHC). Incident World Health Organization Group I PAH patients were analyzed...
May 16, 2018: Chest
https://www.readbyqxmd.com/read/29774953/dermoscopic-patterns-of-filiform-papillae-of-the-tongue-in-patients-with-and-without-connective-tissue-autoimmune-diseases
#6
José Manuel Díaz-González, María E Vega-Memije, Adalberto Mosqueda-Taylor, Víctor Noé García-Edgar, Juan Carlos Cuevas-González
BACKGROUND: Connective tissue autoimmune diseases (CTADs) constitute a group of conditions, including rheumatoid arthritis; systemic lupus erythematosus; mixed connective tissue disease; calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome; scleroderma; dermatomyositis; and Sjögren syndrome. There are few studies on the alterations in filiform papillae in CTAD. Thus, the objective of this work was to determine whether there are changes in the macroscopic and dermoscopic patterns of filiform papillae...
May 18, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29773274/scleroderma-in-children
#7
REVIEW
Francesco Zulian
Juvenile scleroderma with its two varieties, juvenile localized scleroderma and systemic sclerosis (SSc), represents the third most frequent rheumatic disease in childhood. In juvenile SSc, new developments have been recently reported in the fields of classification and monitoring. The introduction of new classification criteria for adult SSc has stimulated new ideas on how to improve the performance of the provisional 2007 PRES/ACR/EULAR pediatric criteria. The introduction of a multidimensional severity score, named "J4S," which includes parameters on growth, skin, and internal organ involvement, has improved the approach to the patients in the daily practice to guide decision-making...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29771006/the-anti-fibrotic-effect-of-adenosine-a2b-receptor-antagonism-in-a-mouse-model-of-dermal-fibrosis
#8
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/29765964/a-rare-syndrome-resembling-scleroderma-huriez-syndrome
#9
Nil Su Çelik, Şirin Yaşar, Sema Aytekin, Pembegül Güneş
Huriez syndrome, also referred to as "sclerotylosis," is a rare autosomal dominant genodermatosis characterized by the triad of congenital scleroatrophy of the distal extremities, palmoplantar keratoderma, and hypoplastic nails. The development of aggressive squamous cell carcinoma (SCC) arising in the scleroatrophic area is also a distinctive feature of the syndrome. Early diagnosis is important due to the early onset, mostly in the third to fourth decades of life, and aggressive progress of SCC, which occurs in around 15% of affected individuals...
April 2018: Skin Appendage Disorders
https://www.readbyqxmd.com/read/29763902/prognostic-factors-of-renal-involvement-in-systemic-sclerosis
#10
Edoardo Rosato, Antonietta Gigante, Biagio Barbano, Maria Ludovica Gasperini, Rosario Cianci, Maurizio Muscaritoli
BACKGROUND/AIMS: Renal involvement is common in systemic sclerosis (SSc), including asymptomatic reduction of glomerular filtration rate (GFR), increased renal resistance indices, scleroderma renal crisis (SRC) and ANCA-associated vasculitis. The aim was to evaluate type and evolution of renal involvement for a period of five years. METHODS: 121 SSc patients (100 F, 21 M) with mean age of 54.9 ± 13.8, disease duration of 9 ± 6 years, of which 62 had a diffused form and 59 limited form were enrolled...
May 10, 2018: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29762932/pulmonary-hypertension-as-a-possible-cause-of-paradoxical-low-flow-low-gradient-aortic-stenosis
#11
Yuta Watanabe, Haruhiko Higashi, Katsuji Inoue, Jun Aono, Takafumi Okura, Jitsuo Higaki, Shuntaro Ikeda
Paradoxical low-flow, low-gradient aortic stenosis (LFLG AS) is recognized as a subtype of aortic stenosis. A small left ventricular (LV) cavity with marked LV concentric remodeling leads to a reduced stroke volume in this condition. The case is reported of a paradoxical LFLG AS patient who was undergoing treatment for pulmonary hypertension (PH) and interstitial pneumonia associated with scleroderma. Echocardiography demonstrated enlargement of the right ventricle and a diminished LV cavity. Moreover, the aortic valve opening was restricted despite a preserved LV ejection fraction (61%)...
September 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/29760622/skin-and-mucous-membranes-manifestations-of-dermatological-diseases-within-the-genital-area-in-females
#12
Katarzyna Plagens-Rotman, Renata Przybylska, Zygmunt Adamski, Magdalena Czarnecka-Operacz
Introduction: Lichen sclerosus et atrophicus (LSA) and an inversed type of psoriasis belong to a group of benign dermatoses usually located within the region of female external genitalia. The most common subjective symptoms reported by patients are itching, pain and changes in the color and structure of the skin. Aim: This paper presents 3 cases of patients suffering from selected dermatoses located within the external female genitalia treated at the Department of Dermatology, Poznan University of Medical Sciences...
April 2018: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/29760620/anti-endothelial-cell-antibodies-do-not-correlate-with-disease-activity-in-systemic-sclerosis
#13
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/29760157/methyl-cpg-binding-protein-2-mediates-antifibrotic-effects-in-scleroderma-fibroblasts
#14
Ye He, Pei-Suen Tsou, Dinesh Khanna, Amr H Sawalha
OBJECTIVE: Emerging evidence supports a role for epigenetic regulation in the pathogenesis of scleroderma (SSc). We aimed to assess the role of methyl-CpG-binding protein 2 (MeCP2), a key epigenetic regulator, in fibroblast activation and fibrosis in SSc. METHODS: Dermal fibroblasts were isolated from patients with diffuse cutaneous SSc (dcSSc) and from healthy controls. MeCP2 expression was measured by qPCR and western blot. Myofibroblast differentiation was evaluated by gel contraction assay in vitro...
May 14, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29750156/right-ventricle-remodeling-and-function-in-scleroderma-patients
#15
REVIEW
Roxana Cucuruzac, Iolanda Muntean, Imre Benedek, Andras Mester, Nora Rat, Adriana Mitre, Monica Chitu, Theodora Benedek
Scleroderma, known also as systemic sclerosis (SSc), is a severe disease associated with high mortality rates, and right ventricular (RV) remodeling and dysfunction, along with pulmonary artery hypertension (PAH), are among the most important internal organ manifestations of this disease. PAH has a higher prevalence in patients with SSc compared to the general population and represents a significant predictor of mortality in SSc. In patients with SSc, the morphological remodeling and alteration of RV function begin even before the setting of PAH and lead to development of a specific adaptive pattern of the RV which is different from the one recorded in patients with IAPH...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29741963/challenges-and-strategies-for-coping-with-scleroderma-implications-for-a-scleroderma-specific-self-management-program
#16
Katherine Milette, Brett D Thombs, Kristina Maiorino, Warren R Nielson, Annett Körner, Sandra Peláez
PURPOSE: The purpose of this study was to explore challenges faced by patients with systemic sclerosis, also called scleroderma, in coping with their disease and the strategies they used to face those challenges. METHOD: Five focus groups were held with scleroderma patients (4 groups, n = 34) and health care professionals who have experience treating scleroderma (1 group, n = 8). Participants' discussions were recorded, transcribed and analyzed using thematic analysis...
May 9, 2018: Disability and Rehabilitation
https://www.readbyqxmd.com/read/29740855/scleroderma-like-skin-changes-induced-by-checkpoint-inhibitor-therapy
#17
B Joel Tjarks, Amy M Kerkvliet, Ali D Jassim, Jonathan S Bleeker
Checkpoint inhibitors have emerged as beneficial therapies in many different types of malignancy and their use has increased dramatically in clinical practice over the past number of years. The most common and concerning toxicities of checkpoint inhibitors are immune related adverse events (irAEs). As clinical experience with these agents increases, more irAEs have been described. We report a case of scleroderma-like skin changes induced by checkpoint inhibitor therapy, namely nivolumab.
May 8, 2018: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/29738553/first-record-of-in-vitro-formation-of-ectomycorrhizae-in-psidium-cattleianum-sabine-a-native-myrtaceae-of-the-brazilian-atlantic-forest
#18
Cassio Geremia Freire, Admir José Giachini, João Peterson Pereira Gardin, Ana Claudia Rodrigues, Renato Luis Vieira, César Milton Baratto, Simone Silmara Werner, Bernardo Haas Abreu
Like many other species of trees native to the Brazilian Mata Atlântica (Atlantic Forest), the Myrtaceae, such as the Red Araza (Psidium cattleianum Sabine), are widely cited as arbuscular mycorrhizal formers. Nevertheless, recent studies show evidence that Myrtaceae from different tropical, subtropical and neotropical ecosystems can also prompt the formation of ectomycorrhizae, indicating that this species' ectomycorrhizal status should be further explored. Because of this, this research effort studied the in vitro interaction between the Red Araza and two ectomycorrhizal fungi isolates, belonging to the Pisolithus microcarpus (D17) and Scleroderma citrinum (UFSC-Sc133) species...
2018: PloS One
https://www.readbyqxmd.com/read/29738043/complement-activation-in-atypical-hemolytic-uremic-syndrome-and-scleroderma-renal-crisis-a-critical-analysis-of-pathophysiology
#19
Roman Zuckerman, Arif Asif, Eric J Costanzo, Tushar Vachharajani
Scleroderma is an autoimmune disease that affects multiple systems. While pathophysiologic mechanisms governing the development of scleroderma are relatively poorly understood, advances in our understanding of the complement system are clarifying the role of complement pathways in the development of atypical hemolytic uremic syndrome and scleroderma renal crisis. The abundant similarities in their presentation as well as the clinical course are raising the possibility of a common underlying pathogenesis. Recent reports are emphasizing that complement pathways appear to be the unifying link...
May 7, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29732959/the-incidence-of-pulmonary-hypertension-is-higher-in-systemic-lupus-and-sj%C3%A3-gren-s-patients-than-in-scleroderma-patients-in-china
#20
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