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https://www.readbyqxmd.com/read/28303689/non-healing-ischaemic-digital-ulcer-in-a-systemic-sclerosis-patient-a-challenging-clinical-case
#1
Sophie Blaise, Matthieu Roustit, Alexandra Forli, Bernard Imbert, Jean-Luc Cracowski
Ischaemic digital ulcers (DUs) are an indicator of the severity of the microangiopathy in patients with systemic sclerosis (SSc). DUs are a frequent complication, affecting about 50% of patients with SSc, and are often recurrent. In cross-sectional studies involving patients with SSc, the frequency of ischaemic DUs was 12-16% with a major impact on hand function and quality of life. Effective therapy for DUs remains elusive. Intravenous iloprost has been demonstrated to have a positive effect on healing of active DUs...
March 16, 2017: International Wound Journal
https://www.readbyqxmd.com/read/28302901/treatment-of-renal-angiomyolipoma-and-other-hamartomas-in-patients-with-tuberous-sclerosis-complex
#2
REVIEW
Joshua A Samuels
Tuberous sclerosis complex is an autosomal dominant genetic disease characterized by growth of benign tumors (hamartomas) in multiple organs, especially the kidneys, brain, heart, lungs, and skin. Tuberous sclerosis complex is usually caused by a mutation in either the tuberous sclerosis complex 1 or tuberous sclerosis complex 2 gene, resulting in constitutive activation of mammalian target of rapamycin signaling. Currently, mammalian target of rapamycin inhibitors are recommended in adult patients with tuberous sclerosis complex for the treatment of asymptomatic, growing renal angiomyolipoma that are >3 cm in diameter and pediatric or adult patients with brain lesions (subependymal giant cell astrocytoma) that either are growing or are not amenable to surgical resection...
March 16, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28270230/risk-factors-for-the-development-of-autism-spectrum-disorder-in-children-with-tuberous-sclerosis-complex-protocol-for-a-systematic-review
#3
Rebecca Mitchell, Sarah Barton, A Simon Harvey, Katrina Williams
BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominant condition, caused by mutations in either the TSC1 or TSC2 gene. It has widespread systemic manifestations and is associated with significant neurological morbidity. In addition to seizures and cerebral pathology including cortical tubers, subependymal nodules, subependymal giant cell astrocytoma and abnormal white matter, there are recognised neuropsychiatric difficulties including intellectual disability, autism spectrum disorder (ASD) and a range of learning and behaviour problems, recently conceptualised as "tuberous sclerosis-associated neuropsychiatric disorders", or "TAND"...
March 8, 2017: Systematic Reviews
https://www.readbyqxmd.com/read/28270192/epidemiology-and-disease-characteristics-of-systemic-sclerosis-related-pulmonary-arterial-hypertension-results-from-a-real-life-screening-programme
#4
Kathleen Morrisroe, Wendy Stevens, Joanne Sahhar, Candice Rabusa, Mandana Nikpour, Susanna Proudman
BACKGROUND: Pulmonary arterial hypertension (PAH) is the leading cause of death in systemic sclerosis (SSc). Annual screening with echocardiogram (ECHO) is recommended. We present the methodological aspects of a PAH screening programme in a large Australian SSc cohort, the epidemiology of SSc-PAH in this cohort, and an evaluation of factors influencing physician adherence to PAH screening guidelines. METHODS: Patient characteristics and results of PAH screening were determined in all patients enrolled in a SSc longitudinal cohort study...
March 7, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28261918/methotrexate-induced-pancytopenia-a-case-series-of-46-patients
#5
Sajal Ajmani, Yogesh Preet Singh, Shiva Prasad, Abhra Chowdhury, Amita Aggarwal, Able Lawrence, Ramnath Misra, Richa Mishra, Vikas Agarwal
AIM: Methotrexate (MTX) has the potential to cause serious adverse reactions and even mortality. We analyzed the predisposing factors and outcome in patients with MTX-induced pancytopenia admitted into our unit from 1996 to 2015. METHODS: Patients were identified by departmental database search. Pancytopenia was defined as white blood cell count (WBC) < 3500 cells/mm(3) , hemoglobin (Hb) < 11 g/dL and platelet count < 150 000 cells/mm(3) . Severe pancytopenia was defined as WBC < 2000 cells/mm(3) , Hb < 10 g/dL and platelet count < 50 000 cells/mm(3) ...
March 5, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28252608/-clinical-guidelines-for-the-use-of-dimethyl-fumarate-in-relapsing-remitting-multiple-sclerosis
#6
V M Alifirova, A N Boiko, Ya V Vlasov, M V Davydovskaya, M N Zakharova, N A Malkova, E V Popova, S A Sivertseva, N N Spirin, N V Khachanova, Т Е Shmidt
Multiple sclerosis is a chronic demyelinating and neurodegenerative disease of the central nervous system, in which autoimmune inflammation and oxidative stress play essential pathogenetic roles. Activation and infiltration of immune cells in brain tissues, lipid peroxidation products, mitochondrial dysfunction, defective antioxidant protection, and many other pathological factors result in demyelination, axonal injury and death, and apoptosis of oligodendrocytes and neurons, all of which causes constant progression of the disease...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28205373/pulmonary-hypertension-in-connective-tissue-diseases-an-update
#7
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/28093711/clinical-neurophysiology-of-multiple-sclerosis
#8
Mario Habek, Ivan Adamec, Barbara Barun, Luka Crnošija, Tereza Gabelić, Magdalena Krbot Skorić
Different neurophysiological methods such as evoked potentials (EP), testing of the autonomic nervous system (ANS) or polysomnography have the potential to detect clinically silent lesions or to confirm the existence of an association between a clinical symptom and multiple sclerosis (MS); previously undetected by MRI. Therefore, in the most recent MRI criteria for the diagnosis of MS (MAGNIMS consensus guidelines), neurophysiological confirmation of optic nerve dysfunction (slowed conduction on visual EP), support dissemination in space and, in patients without concurrent visual symptoms, dissemination in time...
2017: Advances in Experimental Medicine and Biology
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
#9
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
#10
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/28069095/deficits-in-social-cognition-an-unveiled-signature-of-multiple-sclerosis
#11
Moussa A Chalah, Samar S Ayache
BACKGROUND AND OBJECTIVES: Multiple sclerosis (MS) is a chronic progressive inflammatory disease of the central nervous system, representing the primary cause of non-traumatic disability in young adults. Cognitive dysfunction can affect patients at any time during the disease process and might alter the six core functional domains. Social cognition is a multi-component construct that includes the theory of mind, empathy and social perception of emotions from facial, bodily and vocal cues...
January 10, 2017: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/27988845/an-updated-histological-classification-system-for-multiple-sclerosis-lesions
#12
Tanja Kuhlmann, Samuel Ludwin, Alexandre Prat, Jack Antel, Wolfgang Brück, Hans Lassmann
Multiple sclerosis is a complex and heterogeneous, most likely autoimmune, demyelinating disease of the central nervous system (CNS). Although a number of histological classification systems for CNS lesions have been used by different groups in recent years, no uniform classification exists. In this paper, we propose a simple and unifying classification of MS lesions incorporating many elements of earlier histological systems that aims to provide guidelines for neuropathologists and researchers studying MS lesions to allow for better comparison of different studies performed with MS tissue, and to aid in understanding the pathogenesis of the disease...
January 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/27932335/multicentre-randomised-placebo-controlled-trial-of-oral-anticoagulation-with-apixaban-in-systemic-sclerosis-related-pulmonary-arterial-hypertension-the-sphinx-study-protocol
#13
Alicia Calderone, Wendy Stevens, David Prior, Harshal Nandurkar, Eli Gabbay, Susanna M Proudman, Trevor Williams, David Celermajer, Joanne Sahhar, Peter K K Wong, Vivek Thakkar, Nathan Dwyer, Jeremy Wrobel, Weng Chin, Danny Liew, Margaret Staples, Rachelle Buchbinder, Mandana Nikpour
INTRODUCTION: Systemic sclerosis (SSc) is a severe and costly multiorgan autoimmune connective tissue disease characterised by vasculopathy and fibrosis. One of the major causes of SSc-related death is pulmonary arterial hypertension (PAH), which develops in 12-15% of patients with SSc and accounts for 30-40% of deaths. In situ thrombosis in the small calibre peripheral pulmonary vessels resulting from endothelial dysfunction and an imbalance of anticoagulant and prothrombotic mediators has been implicated in the complex pathophysiology of SSc-related PAH (SSc-PAH), with international clinical guidelines recommending the use of anticoagulants for some types of PAH, such as idiopathic PAH...
December 8, 2016: BMJ Open
https://www.readbyqxmd.com/read/27746086/radiographic-changes-of-the-distal-phalanx-tuft-of-the-hands-in-subjects-with-systemic-sclerosis-systematic-review
#14
Yojhan Edilberto Izquierdo, Enrique Calvo Páramo, Luisa María Castañeda, Sandra Viviana Gómez, Fernán Santiago Zambrano
OBJECTIVE: To determine abnormal plain radiograph findings of the distal phalanx tuft of the hand (DPTH) associated with systemic sclerosis in adults. METHODS: A systematic review was developed following the parameters of the PRISMA guidelines in databases: MEDLINE, EMBASE, BIREME, Scielo, Google Scholar and others including as primary outcomes alterations of DPTH (erosions, resorption, sclerosis and proliferation) detected by simple radiography in subjects with systemic sclerosis...
October 13, 2016: Reumatología Clinica
https://www.readbyqxmd.com/read/27635608/-the-efficacy-of-botulinum-toxin-therapy-in-patients-with-upper-limb-spasticity-due-to-traumatic-brain-injury
#15
REVIEW
M A Akulov, S E Khat'kova, O A Mokienko, O R Orlova, D Yu Usachev, V O Zakharov, A S Orlova, A A Tomsky
Spasticity is a type of muscle hyperactivity that occurs in patients after focal lesions of the Central nervous system due to various diseases: stroke, traumatic brain injury or spinal cord injury, neurosurgical intervention, as well as multiple sclerosis and other diseases of the Central nervous system and is the most disability manifestation of the syndrome of upper motor neuron (UMNS). Focal spasticity of the upper limb requires a complex treatment. Botulinum toxin therapy is an effective treatment for focal/multifocal spasticity in reducing muscle tone and improving function with the highest level of evidence according to the latest American and European guidelines for treatment of spasticity...
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/27540435/systemic-sclerosis-and-malignancy-a-review-of-current-data
#16
REVIEW
Nabil Zeineddine, Lara El Khoury, Joseph Mosak
Systemic sclerosis (SSc) is associated with increased risk of malignancy. The organ systems most commonly affected are the lungs, the breasts and the hematological system. Risk factors predisposing a SSc patient for development of malignancy are not well defined, and the pathogenic basis of the association is yet to be explained. The incidence of malignancies in SSc patients is variable from one report to another, but most importantly, questions regarding the role of immunosuppressive therapies and the effect of autoantibodies have weak or sometimes contradictory answers in most of the currently available literature and physicians have no available guidelines to screen their SSc patients for malignancies...
September 2016: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/27535277/-digital-ulcers-in-systemic-sclerosis-a-retrospective-heath-service-study-analysing-treatment-with-bosentan-and-other-vasoactive-therapies
#17
G Riemekasten, S Beissert, J H W Distler, A Kreuter, U Müller-Ladner
BACKGROUND: Digital ulcers (DU) affect up to 60 % of patients with systemic sclerosis (SSc) and have a considerable impact on quality of life and morbidity. It is unclear to what extent authorised medicines are used, and if therapy guidelines are implemented in everyday practice. METHOD: This retrospective health care study examined current standards of treatment for therapy and prevention of SSc-associated DU in an online survey with 83 physicians. Additionally, data from 161 case studies of SSc patients with DU were analysed, and the effect of DU treatment on the course of the disease determined...
August 17, 2016: Zeitschrift Für Rheumatologie
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
#18
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/27349050/-apt-guidelines-for-applying-immunomodulation-with-interferons-in-the-multiple-sclerosis-at-children
#19
REVIEW
Małgorzata Steczkowska, Barbara Skowronek-Bała, Teresa Bawół
Multiple sclerosis (MS) is a chronic demyelinating disease of the central nervous system. The disease usually affects young people, although it may also occur in early childhood. As well as the availability of diagnostic methods rises also the possibility of disease-modifying treatment increases. With constant development of diagnostic methods also the possibility of disease-modifying treatment (DMT) increases. Since 2012 in Poland a therapeutic program of the National Health Fund gives the possibility of using first-line therapy treatment in children/adolescents with multiple sclerosis (over 12 years of age), and since June 2015 using the interferon beta in children of 7 years old and body mass below 30 kg has been approved...
2016: Przegla̧d Lekarski
https://www.readbyqxmd.com/read/27306631/trigeminal-neuralgia-new-classification-and-diagnostic-grading-for-practice-and-research
#20
REVIEW
Giorgio Cruccu, Nanna B Finnerup, Troels S Jensen, Joachim Scholz, Marc Sindou, Peter Svensson, Rolf-Detlef Treede, Joanna M Zakrzewska, Turo Nurmikko
Trigeminal neuralgia (TN) is an exemplary condition of neuropathic facial pain. However, formally classifying TN as neuropathic pain based on the grading system of the International Association for the Study of Pain is complicated by the requirement of objective signs confirming an underlying lesion or disease of the somatosensory system. The latest version of the International Classification of Headache Disorders created similar difficulties by abandoning the term symptomatic TN for manifestations caused by major neurologic disease, such as tumors or multiple sclerosis...
July 12, 2016: Neurology
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