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https://www.readbyqxmd.com/read/28205373/pulmonary-hypertension-in-connective-tissue-diseases-an-update
#1
REVIEW
Ramya Aithala, Anoop G Alex, Debashish Danda
Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. CTD-associated PH belongs to group 1 PH, also known as pulmonary arterial hypertension (PAH). Around 30% of scleroderma-related deaths are due to PAH. Underlying pathogenesis is related to pulmonary vasculopathy involving small vessels...
February 16, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28089973/malignancies-in-patients-with-anti-rna-polymerase-iii-antibodies-and-systemic-sclerosis-analysis-of-the-eular-scleroderma-trials-and-research-cohort-and-possible-recommendations-for-screening
#2
Maria-Grazia Lazzaroni, Ilaria Cavazzana, Enrico Colombo, Rucsandra Dobrota, Jasmin Hernandez, Roger Hesselstrand, Cecilia Varju, Gabriella Nagy, Vanessa Smith, Paola Caramaschi, Valeria Riccieri, Eric Hachulla, Alexandra Balbir-Gurman, Emmanuel Chatelus, Katarzyna Romanowska-Próchnicka, Ana Carolina Araújo, Oliver Distler, Yannick Allanore, Paolo Airò
OBJECTIVE: To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)- positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients. METHODS: (1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28088339/evidence-based-management-of-systemic-sclerosis-navigating-recommendations-and-guidelines
#3
REVIEW
Russell Edward Pellar, Janet Elizabeth Pope
OBJECTIVES: Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed. METHODS: The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted...
December 9, 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/27931056/-guideline-s2k-awmf-of-the-deutsche-gesellschaft-f%C3%A3-r-pneumologie-und-beatmungsmedizin-and-the-deutsche-gesellschaft-f%C3%A3-r-arbeitsmedizin-und-umweltmedizin-diagnostics-and-expert-opinion-in-the-occupational-disease-no-4101-silicosis-including-coal-worker-s-pneumoconiosis
#4
X Baur, M Heger, R M Bohle, K G Hering, K Hofmann-Preiß, D Nowak, A Tannapfel, H Teschler, T Voshaar, T Kraus
During the last 1.5 years an update of the guideline on silicosis was made by an interdisciplinary working group. New medical and scientific knowledge and the experience in expert opinion practice were taken into account.By preparing the initial guideline in 2010 standardization of diagnostics and adaption of the "Moers convention" which was not based on medical knowledge was in the focus, whereas the current update deals with fine emendation and extension, especially of the compensation rate (adaption with the Reichenhall recommendation)...
December 2016: Pneumologie
https://www.readbyqxmd.com/read/27833730/general-diseases-influence-on-peri-implantitis-development-a-systematic-review
#5
REVIEW
Zygimantas Guobis, Ingrida Pacauskiene, Inesa Astramskaite
OBJECTIVES: To systematically review the influence of systemic diseases or medications used in their treatment on the dental implant therapy success. MATERIAL AND METHODS: The search strategy was implemented on the National Library of Medicine database (MEDLINE) (Ovid) and EMBASE electronic databases between January 2006 and January 2016. Human studies with available English articles analysing the relationship between dental implant therapy success and systemic diseases, such as diabetes mellitus, AIDS/HIV, rheumatoid arthritis, osteoporosis, Crohn's disease, cardiovascular diseases, scleroderma, Sjögren's syndrome, lichen planus, ectodermal dysplasia, post-transplantation status, were included in present review according to the PRISMA guidelines...
July 2016: Journal of Oral & Maxillofacial Research
https://www.readbyqxmd.com/read/27749244/uk-scleroderma-study-group-ukssg-guidelines-on-the-diagnosis-and-management-of-scleroderma-renal-crisis
#6
REVIEW
Bernadette M Lynch, Edward P Stern, Voon Ong, Mark Harber, Aine Burns, Christopher P Denton
The UK Scleroderma Study Group developed guidelines on the diagnosis and management of scleroderma renal crisis (SRC) based on best available evidence and clinical experience. SRC is characterised by the acute onset of severe hypertension and acute kidney injury. Current strategies to reduce the associated morbidity and mortality include identifying at risk patients to aid early diagnosis. ACE inhibitor therapy should be lifelong in all patients, regardless of whether they require renal replacement therapy...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27514197/-rheumatology-progress-in-diagnosis-and-treatments-topics-iv-collagen-diseases-except-for-rheumatoid-arthritis-and-hot-topics-3-systemic-sclerosis-scleroderma
#7
REVIEW
Hidekata Yasuoka
No abstract text is available yet for this article.
October 10, 2014: Nihon Naika Gakkai Zasshi. the Journal of the Japanese Society of Internal Medicine
https://www.readbyqxmd.com/read/27390939/left-ventricular-diastolic-dysfunction-and-increased-left-ventricular-mass-index-related-to-pulmonary-hypertension-in-patients-with-systemic-autoimmune-disease-without-pericardial-effusion
#8
Atsushi Sugiura, Nobusada Funabashi, Koya Ozawa, Yoshio Kobayashi
PURPOSE: We investigated the relationship of left ventricular (LV) diastolic dysfunction and LV mass index (LVMI) against pulmonary hypertension (PH) in systemic autoimmune disease (SAD). METHODS: A total of 84 SAD patients (68 females; 53±17years; systemic lupus erythematosus, 27%; scleroderma, 17%; vasculitis, 16%; mixed connective tissue disease, 13% and polymyositis/dermatomyositis complex, 10%) without significant pericardial effusion (PE) on TTE (Vivid E9, GE) were analyzed...
October 1, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/26868300/emergency-situations-in-rheumatology-with-a-focus-on-systemic-autoimmune-diseases
#9
REVIEW
Jiri Vymetal, Martina Skacelova, Andrea Smrzova, Anna Klicova, Marketa Schubertova, Pavel Horak, Josef Zadrazil
BACKGROUND AND AIM: Rheumatic diseases are commonly considered chronic conditions. However, acute manifestations can be very severe and represent a diagnostic problem. Examples are systemic lupus erythematosus with acute flare, glomerulonephritis, CNS disorders and catastrophic antiphospholipid syndrome, scleroderma with interstitial lung disease, pulmonary hypertension and renal crisis and polyangiitis with alveolar haemorhage and acute respiratory failure. This aim of this paper is to overview emergency situations which can be encountered in the care of patients with autoimmune systemic diseases and vasculitides...
March 2016: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
https://www.readbyqxmd.com/read/26819124/german-guidelines-for-the-diagnosis-and-therapy-of-localized-scleroderma
#10
Alexander Kreuter, Thomas Krieg, Margitta Worm, Jörg Wenzel, Pia Moinzadeh, Annegret Kuhn, Elisabeth Aberer, Karin Scharffetter-Kochanek, Gerd Horneff, Emma Reil, Tobias Weberschock, Nicolas Hunzelmann
Localized scleroderma designates a heterogeneous group of sclerotic skin disorders. Depending on the subtype, severity, and site affected, adjacent structures such as adipose tissue, muscles, joints, and bones may be involved. This is an update of the existing German AWMF (Association of the Scientific Medical Societies in Germany) guidelines (classification: S2k). These guidelines provide an overview of the definition, epidemiology, classification, pathogenesis, laboratory workup, histopathology, clinical scoring systems, as well as imaging and device-based workup of localized scleroderma...
February 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/26718896/factors-associated-with-development-of-gastrointestinal-problems-in-patients-with-scleroderma-a-systematic-review
#11
REVIEW
Brian Younho Hong, Raymond Giang, Lawrence Mbuagbaw, Maggie Larche, Lehana Thabane
BACKGROUND: Up to 90% of people with scleroderma have gastrointestinal (GI) problems such as constipation, bloating, diarrhea, and malabsorption. These problems significantly impair quality of life. Our objective was to determine the risk factors for gastrointestinal issues in people with scleroderma. METHODS: We conducted a systematic review of observational studies that report GI problems in patients with scleroderma along with the associated risk factors. We were interested in any GI problem and any risk factor as long as the study included patients diagnosed with scleroderma according to the 1980 or 2013 American College of Rheumatology guideline...
2015: Systematic Reviews
https://www.readbyqxmd.com/read/26684622/digital-ulcers-in-scleroderma-patients-a-retrospective-observational-study
#12
A De Cata, M Inglese, F Molinaro, S De Cosmo, R Rubino, M Bernal, G Mazzoccoli
BACKGROUND: The guidelines for digital ulcers (DUs) management in systemic sclerosis (SSc) indicate the use of iloprost to induce wound healing and bosentan to prevent the onset of new DU. The aim of our study was to evaluate whether the combination treatment may surmount the effect of the single drug. METHODS: We analyzed data regarding 34 patients with SSc and at least one active DU persisting despite 6 months of iloprost therapy, and treated for other 6 months with a combination therapy, i...
June 2016: International Journal of Immunopathology and Pharmacology
https://www.readbyqxmd.com/read/26359329/serum-prealbumin-is-an-independent-predictor-of-mortality-in-systemic-sclerosis-outpatients
#13
MULTICENTER STUDY
Veronica Codullo, Emanuele Cereda, Catherine Klersy, Ilaria Cavazzana, Claudia Alpini, Chiara Bonardi, Annalisa Turri, Franco Franceschini, Riccardo Caccialanza, Carlomaurizio Montecucco, Roberto Caporali
OBJECTIVE: Serum prealbumin is a recognized marker of malnutrition, but its role in the prognosis of patients with SSc has not yet been investigated. The aim of the present multicentre prospective study was to investigate the association between prealbumin and mortality, independent of clinical features, in a cohort of SSc outpatients. METHODS: Patients were followed up according to standard clinical guidelines with visits at least every 6 months. Data collected included records of skin and internal organ involvement, survival and causes of death...
February 2016: Rheumatology
https://www.readbyqxmd.com/read/26339894/content-of-non-pharmacological-care-for-systemic-sclerosis-and-educational-needs-of-european-health-professionals-a-eushnet-survey
#14
Linda M Willems, Anthony C Redmond, Tanja A Stamm, Carina Boström, Saskia Decuman, Ann Tyrrell Kennedy, Jadranka Brozd, Sanja Roškar, Vanessa Smith, Theodora P M Vliet Vlieland, Cornelia H M van den Ende
OBJECTIVES: To describe the non-pharmacological care in systemic sclerosis (SSc) provided by European health professionals (HPs) including referrals, treatment targets, interventions, and educational needs. METHODS: In this observational study, European HPs working in SSc care were invited to complete an online survey through announcements by EUSTAR (European League Against Rheumatism (EULAR) Scleroderma Trials and Research) and FESCA (Federation of European Scleroderma Associations), the EULAR HPs' newsletter, websites of national patient and HP associations, and by personal invitation...
July 2015: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/26320140/application-of-the-detect-algorithm-for-detection-of-risk-of-pulmonary-arterial-hypertension-in-systemic-sclerosis-data-from-a-czech-tertiary-centre
#15
Tomas Soukup, Radek Pudil, Katerina Kubinova, Lucie Hromadkova, Jaroslav Dusek, Marian Tosovsky, Petr Bradna, Zbynek Hrncir, Jan Bures
OBJECTIVE: The early, simple and reliable detection of pulmonary arterial hypertension (PAH) in SSc (DETECT) study described a new algorithm for early detection of PAH in patients with SSc. The aim of this retrospective, single-centre, cross-sectional study was to apply a modified DETECT calculator in patients with SSc in the East Bohemian region, Czech Republic, to assess the risk of PAH and to compare these results with PAH screening based on the European Society of Cardiology/European Respiratory Society (ESC/ERS) 2009 guidelines...
January 2016: Rheumatology
https://www.readbyqxmd.com/read/26139334/update-on-systemic-sclerosis
#16
REVIEW
Courtney J McCray, Maureen D Mayes
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by autoantibody production, small-vessel vasculopathy, and skin and other organ fibrosis. The disease is clinically heterogeneous with most patients having some degree of skin sclerosis with varying organ system involvement. Early disease can be difficult to diagnose, especially with minimal skin sclerosis and absence of anti-nuclear antibody (ANA) positivity; however, studies have demonstrated early diagnosis is important as early treatment could potentially lead to better outcomes...
May 2015: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/26121522/a-comparison-between-general-rheumatologists-and-scleroderma-experts-with-respect-to-following-systemic-sclerosis-guidelines
#17
COMPARATIVE STUDY
Jason Reed, Janet Pope
OBJECTIVES: To determine if there are differences between expert and non-expert rheumatologists in systemic sclerosis (SSc) management. METHODS: Information relating to demographics, complications, investigations, and treatment of SSc patients was obtained from an online survey to members of the Canadian Rheumatology Association (CRA), and selected chart audits. Results were compared to data from a SSc database ('experts', Canadian Scleroderma Research Group--CSRG)...
July 2015: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/26115973/-systemic-sclerosis-current-classification-and-diagnosis-of-organ-involvement
#18
REVIEW
O D Persa, P Moinzadeh, N Hunzelmann
Systemic sclerosis (SSc) is a rare, chronic inflammatory autoimmune disease with unknown etiology, which leads to deposition of collagen and extracellular matrix proteins in the skin and affected internal organs. The diagnosis of SSc is based on clinical, serological, and paraclinical examinations. In 2013 new criteria for the classification of systemic sclerosis, which also take early forms of SSc into consideration, were developed. A complete clinical and paraclinical examination is important for the oligosymptomatic early stages and the subsequent disease course of SSc in order to diagnose and timely treat a developing organ involvement...
August 2015: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/25881068/progressive-hemifacial-atrophy-a-review
#19
REVIEW
Stanislav N Tolkachjov, Nirav G Patel, Megha M Tollefson
BACKGROUND: Progressive Hemifacial Atrophy (PHA) is an acquired, typically unilateral, facial distortion with unknown etiology. The true incidence of this disorder has not been reported, but it is often regarded as a subtype of localized scleroderma. Historically, a debate existed whether PHA is a form of linear scleroderma, called morphea en coup de sabre (ECDS), or whether these conditions are inherently different processes or appear on a spectrum (; Adv Exp Med Biol 455:101-4, 1999; J Eur Acad Dermatol Venereol 19:403-4, 2005)...
2015: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/25874345/new-acr-eular-guidelines-for-systemic-sclerosis-classification
#20
Sindhu R Johnson
The American College of Rheumatology and European League Against Rheumatism classification criteria for systemic sclerosis are a significant advancement in the field. This article describes the innovative, rigorous, criteria development strategy that was used. The new criteria build upon previous criteria by incorporating important elements (proximal scleroderma, sclerodactyly, digital pits, pulmonary fibrosis, Raynaud's phenomenon, and scleroderma specific autoantibodies). The new criteria add emphasis to the vasculopathic manifestations, and include the early manifestation of puffy fingers...
May 2015: Current Rheumatology Reports
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