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Vasculitides

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https://www.readbyqxmd.com/read/28095714/disease-related-and-drug-induced-skin-manifestations-in-inflammatory-bowel-disease
#1
Pieter Hindryckx, Gregor Novak, Antonio Costanzo, Silvio Danese
Skin manifestations are common in patients with inflammatory bowel diseases (IBD) and can be part of a concomitant illness with a shared genetic background, an extra-intestinal manifestation of the disease, or a drug side-effect. Areas covered: We provide a practical overview of the epidemiology, pathogenesis, diagnosis, therapeutic approach and prognosis of the most frequent disease-related and drug-induced cutaneous manifestations in IBD, illustrated by cases encountered in our clinical practice. Among the most frequently encountered IBD-related lesions are erythema nodosum, pyoderma gangrenosum and Sweet's syndrome...
January 17, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28087591/clinical-trials-in-antineutrophil-cytoplasmic-antibody-associated-vasculitis-what-we-have-learnt-so-far-and-what-we-still-have-to-learn
#2
Divi Cornec, Emilie Cornec-Le Gall, Ulrich Specks
The prognosis of the antineutrophil cytoplasmic antibody associated vasculitides (AAV), microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), has been fundamentally improved over the last five decades by the use of glucocorticoids and immunosuppressants, turning them from consistently fatal diseases into chronic conditions. The long-term course is now largely determined by the frequency of disease flares and by accruing damage caused by disease activity and treatment-related comorbidities...
January 12, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28079914/intravenous-immunoglobulin-in-pediatric-rheumatology-when-to-use-it-and-what-is-the-evidence
#3
Martha M Rodriguez, Linda Wagner-Weiner
Intravenous immunoglobulin (IVIG) is given to children with a variety of rheumatologic illnesses. The mechanism of action by which it exerts therapeutic effects is not well understood and likely differs in the medical conditions for which it is given. IVIG is approved by the US Food and Drug Administration and is the standard of care for Kawasaki disease, but most IVIG use in pediatric rheumatology is "off-label. " The literature supports the use of IVIG for juvenile dermatomyositis, although it is unclear whether its use should be limited to those children with more severe or refractory disease...
January 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28077133/t1-and-t2-mapping-for-evaluation-of-myocardial-involvement-in-patients-with-anca-associated-vasculitides
#4
Simon Greulich, Agnes Mayr, Daniel Kitterer, Joerg Latus, Joerg Henes, Hannah Steubing, Philipp Kaesemann, Alexandru Patrascu, Andreas Greiser, Stefan Groeninger, Niko Braun, M Dominik Alscher, Udo Sechtem, Heiko Mahrholdt
BACKGROUND: Myocardial involvement in AAV patients might be silent, presenting with no or nonspecific symptoms, normal ECG, and preserved left-ventricular ejection fraction (LV-EF). Since up to 50% of deaths in these patients may be due to myocardial involvement, a reliable diagnostic tool is warranted. In contrast to LGE-CMR, which has its strengths in detecting focal inflammatory or fibrotic processes, recent mapping techniques are able to detect even subtle, diffuse inflammatory or fibrotic processes...
January 6, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28067080/-recent-advances-in-the-treatment-of-large-vessel-vasculitides
#5
Melinda Zsuzsanna Szabó, Emese Kiss
Giant cell arteritis and Takayasu arteritis classified to large vessel vasculitides have similar histopathology in the vascular wall proposing that these entities can be different phenotypes on a spectrum of a single disorder. Glucocorticoids are the mainstay of therapy combined with cyclophosphamide, azatioprine and mycofenolate mofetil, when it is required. However, a significant proportion of patients are glucocorticoid-dependent despite of the conventional disease-modifying antirheumatic drugs and suffer from serious side effects of the steroids, therefore alternate options for more effective disease management are needed...
January 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28062909/paediatric-anti-neutrophil-cytoplasmic-antibody-anca-associated-vasculitis-an-update-on-renal-management
#6
REVIEW
Lucy A Plumb, Louise Oni, Stephen D Marks, Kjell Tullus
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of disorders characterized by necrotizing inflammation of the small to medium vessels in association with autoantibodies against the cytoplasmic region of the neutrophil. Included in this definition are granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). AAV are chronic, often relapsing diseases that can be organ or life threatening...
January 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28053275/clinical-associations-with-venous-thromboembolism-in-anti-neutrophil-cytoplasm-antibody-associated-vasculitides
#7
Andreas Kronbichler, Johannes Leierer, Gisela Leierer, Gert Mayer, Alina Casian, Peter Höglund, Kerstin Westman, David Jayne
OBJECTIVE: To assess potential associations for the development of venous thromboembolic events in patients with ANCA-associated vasculitides (AAV). METHODS: Four hundred and seventeen patients enrolled to participate in randomized controlled trials conducted by the European Vasculitis Society were identified. Univariate and multivariate analyses were performed to validate previously proposed and identify novel risks associated with venous thromboembolism (VTE) in AAV...
January 3, 2017: Rheumatology
https://www.readbyqxmd.com/read/28035582/endothelial-dysfunction-and-hypertension
#8
Dildar Konukoglu, Hafize Uzun
In the past, endothelium was thought to be only a mechanical barrier. Today, endothelium is known to be a tissue regulating vascular tone, cell growth and the interaction between the leukocytes, thrombocytes and the vessel wall. It also synthesizes growth factors and thrombo-regulatory molecules and responds to physical and chemical signals. Even though the term "endothelial dysfunction" is generally used for deterioration of endothelium-dependent vasodilatation; the term also includes the abnormalities between endothelium and leukocytes, thrombocytes and regulatory molecules and conditions resulting in aberrant endothelium activation...
December 30, 2016: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28032843/employment-work-disability-and-quality-of-life-in-patients-with-anca-associated-vasculitides-the-expovas-study
#9
Lucas Benarous, Benjamin Terrier, Hervé Laborde-Casterot, Alice Bérezné, Bertrand Dunogué, Pascal Cohen, Xavier Puéchal, Luc Mouthon, Lynda Bensefa-Colas, Loic Guillevin
OBJECTIVES: Improved therapeutic strategies for ANCA-associated vasculitis (AAV) have transformed acute and life-threatening diseases into chronic ones responsible for marked morbidity that could impact employment, work disability and quality of life (QoL). We aimed to analyse work, handicaps and QoL of AAV patients and identify their determinants. METHODS: Patients with AAV were included in a cross-sectional study assessing employment, work disability and QoL. Specific and non-specific questionnaires, including SF-36, were sent to patients, and clinical-biological data that could affect QoL and their determinants were analysed...
December 20, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27994096/paediatric-rheumatology-clinic-population-in-southeast-asia-are-we-different
#10
Thaschawee Arkachaisri, Swee-Ping Tang, Tassalapa Daengsuwan, Gun Phongsamart, Soamarat Vilaiyuk, Sirirat Charuvanij, Sook Fun Hoh, Justin Hung Tiong Tan, Lena Das, Elizabeth Ang, Wendy Lim, Yiong Huak Chan, Christine B Bernal
OBJECTIVES: To examine the descriptive epidemiology of the patient population referred to paediatric rheumatology centres (PRCs) in Southeast Asia (SEA) and to compare the frequency of conditions encountered with other PRC populations. METHODS: A web-based Registry for Childhood Onset Paediatric Rheumatic Diseases was established in 2009 and seven PRCs in four SEA countries, where paediatric rheumatologists are available, participated in a prospective 24 month data collection (43 months for Singapore)...
December 19, 2016: Rheumatology
https://www.readbyqxmd.com/read/27988436/a-scoping-review-of-the-use-of-non-biologic-disease-modifying-anti-rheumatic-drugs-in-the-management-of-large-vessel-vasculitis
#11
REVIEW
Durga Prasanna Misra, Aman Sharma, Tamilarasu Kadhiravan, Vir Singh Negi
Takayasu's arteritis (TA) and Giant cell arteritis (GCA) comprise the large vessel vasculitides (LVV). Patients with LVV are treated with disease-modifying anti-rheumatic drugs (DMARDs), both conventional (cDMARDs) and biologic (bDMARDs). We undertook a scoping review to assess the effectiveness of cDMARDs in TA and GCA. We could identify 11 studies in TA and 18 studies in GCA. There were only 3 randomized controlled trials on methotrexate, one on hydroxychloroquine and two on cyclosporine in GCA, the others being case series (including all studies on TA)...
December 15, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27935900/-thromboangiitis-obliterans-buerger-s-disease-state-of-the-art
#12
D D Zerbino, E A Zimba, N N Bagry
Thromboangiitis obliterans (Buerger's disease) is systemic vasculitis with predominant development of thrombotic occlusions of small-to-medium diameter arteries of distal portions of both upper and lower limbs. A distinctive feature of Buerger's disease from other vasculitides is the involvement of the venous bed into the pathological process in the form of migrating thrombophlebitis. The disease is encountered more often in young adult males, predominantly tobacco smokers. The clinical pattern is presented by symptoms of increasing insufficiency of blood supply of tissues of extremities...
2016: Angiologii︠a︡ i Sosudistai︠a︡ Khirurgii︠a︡, Angiology and Vascular Surgery
https://www.readbyqxmd.com/read/27933390/-de-escalation-of-therapy-in-anca-associated-vasculitides
#13
REVIEW
S Schinke, G Riemekasten, P Lamprecht
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) require a differentiated therapeutic approach depending on the degree of organ dysfunction and disease activity. In organ dysfunction and life-threatening AAV cyclophosphamide and rituximab are recommended for the induction of remission. For remission induction with a lack of organ dysfunction and non-life-threatening AAV, methotrexate or mycophenolate mofetil are recommended. For remission maintenance therapy azathioprine or methotrexate are used...
December 8, 2016: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/27906751/an-update-on-vasculitides-lumps-and-constructs
#14
Hasan Yazici, Yusuf Yazici
No abstract text is available yet for this article.
January 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/27902615/the-prognostic-significance-of-the-birmingham-vasculitis-activity-score-bvas-with-systemic-vasculitis-patients-transferred-to-the-intensive-care-unit-icu
#15
Federico Biscetti, Angela Carbonella, Federico Parisi, Silvia Laura Bosello, Franco Schiavon, Roberto Padoan, Elisa Gremese, Gianfranco Ferraccioli
Systemic vasculitides represent a heterogeneous group of diseases that share clinical features including respiratory distress, renal dysfunction, and neurologic disorders. These diseases may often cause life-threatening complications requiring admission to an intensive care unit (ICU). The aim of the study was to evaluate the validity and responsiveness of Birmingham Vasculitis Activity Score (BVAS) score to predict survival in patients with systemic vasculitides admitted to ICU.A retrospective study was carried out from 2004 to 2014 in 18 patients with systemic vasculitis admitted to 2 different Rheumatology divisions and transferred to ICU due to clinical worsening, with a length of stay beyond 24 hours...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27894445/usual-and-unusual-manifestations-of-systemic-and-central-nervous-system-vasculitis
#16
REVIEW
James J Nocton
The idiopathic vasculitides are a group of inflammatory and immune-mediated conditions associated with inflammation of blood vessels. They affect multiple organ and body systems, and vary in their clinical manifestations, severity, prognosis, and pathology. They frequently present a diagnostic challenge for clinicians because of their complexity, overlapping features, and similar findings to other noninflammatory, genetic, or infectious conditions. This article summarizes some of the common pediatric vasculitides, emphasizing both the characteristic and unusual clinical manifestations of these diseases...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27876769/gastrointestinal-aspects-of-vasculitides
#17
REVIEW
Medha Soowamber, Adam V Weizman, Christian Pagnoux
Systemic vasculitides are caused by inflammation of blood vessels and can affect any organ and any part of the gastrointestinal tract, hepatic and biliary system, as well as the pancreas. These disorders can cause a wide array of gastrointestinal manifestations, from asymptomatic elevated transaminase levels and mild abdominal pain to potentially life-threatening bowel perforations and peritonitis. A diagnosis based solely on gastrointestinal symptoms is challenging as these manifestations are not specific...
November 23, 2016: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/27872452/how-to-treat-anca%C3%A2-associated-vasculitis-practical-messages-from-2016-eular-era%C3%A2-edta-recommendations
#18
Jan Sznajd, Chetan Mukhtyar
The European League against Rheumatism (EULAR) with the European Renal Association - European Dialysis and Transplant Association recently published an update of 2009 EULAR recommendations with a focus on the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). In this article, we discuss the following key messages for clinical practice derived from these recommendations: 1) biopsy should be performed if possible to confirm new diagnosis or relapse; 2) glucocorticoid therapy is an extremely important adjunct to the management of AAV, but it is also responsible for the majority of adverse effects; the dose should be tapered to 7...
October 28, 2016: Polskie Archiwum Medycyny Wewnętrznej
https://www.readbyqxmd.com/read/27864582/-skin-diseases-due-to-systemic-vasculitides-and-vasculopathies
#19
REVIEW
S Volc, J C Maier, M Röcken
Vasculitis and vasculopathy are two distinct disease entities. Each entity comprises a large number of heterogeneous diseases, which can occur alone or associated with autoimmune, infectious or neoplastic diseases. The terms vasculitis and vasculopathy are often falsely used synonymously. A vasculitis initially causes inflammation of the vessel walls that may result in a secondary occlusion. In contrast, a vasculopathy is a primary occlusion of the vascular lumen, which is followed by inflammation after ischemia and ulceration...
December 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/27858870/simultaneous-comprehensive-multiplex-autoantibody-analysis-for-rapidly-progressive-glomerulonephritis
#20
Mandy Sowa, Barbara Trezzi, Rico Hiemann, Peter Schierack, Kai Grossmann, Juliane Scholz, Valentina Somma, Renato Alberto Sinico, Dirk Roggenbuck, Antonella Radice
Rapidly progressive glomerulonephritis (RPGN) is mainly caused by anti-glomerular basement membrane (GBM) antibody-mediated glomerulonephritis, immune-complex or anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides and leads to rapid loss of renal function. Detection of ANCA and autoantibodies (autoAbs) to GBM and dsDNA enables early diagnosis and appropriate treatment of RPGN aiding in preventing end-stage renal disease.Determination of ANCA on neutrophils (ANCA) as well as autoAbs to myeloperoxidase (MPO-ANCA), proteinase 3 (PR3-ANCA), GBM, and dsDNA was performed by the novel multiplex CytoBead technology combining cell- and microbead-based autoAb analyses by automated indirect immunofluorescence (IIF)...
November 2016: Medicine (Baltimore)
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