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Vasculitides

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https://www.readbyqxmd.com/read/28337408/eosinophilic-granulomatosis-with-polyangiitis-formerly-known-as-churg-strauss-syndrome-as-a-differential-diagnosis-of-hypereosinophilic-syndromes
#1
Yuri Albuquerque Pessoa Santos, Bruno Rangel Antunes Silva, Pollyanna Natividade Zanconato Barros Assis Lira, Luiz Carlos Aguiar Vaz, Thiago Thomaz Mafort, Leonardo Palermo Bruno, Agnaldo José Lopes
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) and hypereosinophilic syndromes (HES). Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28332442/ultrasound-imaging-in-the-diagnosis-of-large-vessel-vasculitis
#2
Michael Czihal, Christian Lottspeich, Ulrich Hoffmann
Nowadays noninvasive vascular imaging has an important role in the diagnostic work-up of the large vessel vasculitides (LVV), most importantly giant cell arteritis (GCA) and Takayasu arteritis. Among the imaging modalities available, ultrasound (US) has several important advantages, including low costs, rapid and repetitive availability without exposure to radiation, and high spatial resolution for assessment of large and medium-sized arteries. Therefore, US can be regarded the first line imaging method in suspected LVV...
March 23, 2017: VASA. Zeitschrift Für Gefässkrankheiten
https://www.readbyqxmd.com/read/28306595/update-on-maintenance-therapy-for-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#3
Ora Singer, W Joseph McCune
PURPOSE OF REVIEW: The antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitides are a group of rare systemic diseases. The past several years have seen major therapeutic advances in the treatment of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The success rate in induction of remission is high, but reducing the high incidence of relapses remains a therapeutic challenge. RECENT FINDINGS: Studies have shown no improvement in relapse rates in GPA and MPA over the past 2 decades...
March 16, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28277489/analysis-of-the-common-genetic-component-of-large-vessel-vasculitides-through-a-meta-immunochip-strategy
#4
F David Carmona, Patrick Coit, Güher Saruhan-Direskeneli, José Hernández-Rodríguez, María C Cid, Roser Solans, Santos Castañeda, Augusto Vaglio, Haner Direskeneli, Peter A Merkel, Luigi Boiardi, Carlo Salvarani, Miguel A González-Gay, Javier Martín, Amr H Sawalha
Giant cell arteritis (GCA) and Takayasu's arteritis (TAK) are major forms of large-vessel vasculitis (LVV) that share clinical features. To evaluate their genetic similarities, we analysed Immunochip genotyping data from 1,434 LVV patients and 3,814 unaffected controls. Genetic pleiotropy was also estimated. The HLA region harboured the main disease-specific associations. GCA was mostly associated with class II genes (HLA-DRB1/HLA-DQA1) whereas TAK was mostly associated with class I genes (HLA-B/MICA). Both the statistical significance and effect size of the HLA signals were considerably reduced in the cross-disease meta-analysis in comparison with the analysis of GCA and TAK separately...
March 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28242394/successful-management-of-arterial-pseudoaneurysm-caused-by-stent-fracture
#5
Maham Rahimi, Brent Robertson, Lynne M Doctor, Jonathan Bath
Pseudoaneurysm is a relatively common complication of arterial injury. Arterial pseudoaneurysms have many different etiologies, including trauma, iatrogenic injury, vascular repair, infection, and vasculitides. In this case report, we present a pseudoaneurysm created by a fracture of superficial femoral artery stent secondary to a mechanical fall. To our knowledge, this is the first report of a symptomatic pseudoaneurysm caused by a superficial femoral artery stent fracture. The large, unruptured pseudoaneurysm in this case was successfully treated with a covered stent and pseudoaneurysm exclusion...
February 24, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28229830/neutrophil-extracellular-traps-formation-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis-association-with-eosinophilic-inflammation
#6
Joanna Natorska, Michał Ząbczyk, Jakub Siudut, Piotr Krawiec, Lucyna Mastalerz, Anetta Undas
OBJECTIVES: Eosinophilic granulomatosis with polyangiitis (EGPA) is associated with an inflammation and the presence of antineutrophil cytoplasmic antibodies (ANCA). Thus, we investigated the impact of ANCAs and eosinophilic inflammation on neutrophil activation and extracellular traps (NETs) formation. METHODS: We recruited 29 patients in the remission of EGPA (17 ANCA-negative and 12 ANCA-positive, including 7 p-ANCA-positive and 5 c-ANCA-positive patients). Healthy donors' neutrophils were stimulated with EGPA patients' serum...
February 9, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28225490/clinical-characteristics-and-outcome-of-spanish-patients-with-anca-associated-vasculitides-impact-of-the-vasculitis-type-anca-specificity-and-treatment-on-mortality-and-morbidity
#7
MULTICENTER STUDY
Roser Solans-Laqué, Guadalupe Fraile, Monica Rodriguez-Carballeira, Luis Caminal, Maria J Castillo, Ferran Martínez-Valle, Luis Sáez, Juan J Rios, Xavier Solanich, Joaquim Oristrell, Francisco Pasquau, Eva Fonseca, Monica Zamora, Jose L Callejas, Begoña Frutos, Monica Abdilla, Patricia Fanlo, Isabel García-Sánchez, Miguel López-Dupla, Bernardo Sopeña, Almudena Pérez-Iglesias, Josep A Bosch
The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome.A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA)...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28184292/rituximab-therapy-in-pemphigus-and-other-autoantibody-mediated-diseases
#8
REVIEW
Nina A Ran, Aimee S Payne
Rituximab, a monoclonal antibody targeting the B cell marker CD20, was initially approved in 1997 by the United States Food and Drug Administration (FDA) for the treatment of non-Hodgkin lymphoma. Since that time, rituximab has been FDA-approved for rheumatoid arthritis and vasculitides, such as granulomatosis with polyangiitis and microscopic polyangiitis. Additionally, rituximab has been used off-label in the treatment of numerous other autoimmune diseases, with notable success in pemphigus, an autoantibody-mediated skin blistering disease...
2017: F1000Research
https://www.readbyqxmd.com/read/28157661/eosinophilic-endomyocardial-disease
#9
Victor Y Liu, Raymundo A Quintana-Quezada, L Maximilian Buja
Eosinophilic granulomatosis with polyangiitis is the rarest of the antineutrophil cytoplasmic antibody-associated vasculitides. We describe the case of a 58-year-old woman with a history of asthma found to have a left ventricular apical thrombus. Cardiac catheterization with endomyocardial biopsy was consistent with eosinophilic endomyocardial disease. The disease affects several organ systems and shares many features with more common conditions, making it a difficult diagnosis.
March 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28153130/inflammatory-and-infectious-lesions-of-the-sinonasal-tract
#10
REVIEW
Kathleen T Montone, Virginia A LiVolsi
The sinonasal tract is frequently affected by nonneoplastic inflammatory diseases. Inflammatory lesions of the sinonasal tract can be divided into 3 main categories: chronic rhinosinusitis, which encompasses a heterogeneous group of entities, all of which result in mucosal inflammation with or without polyps-eosinophils; infectious diseases; and autoimmune diseases and vasculitides, which can result in midline necrosis and facial deformities. This article reviews the common inflammatory lesions of the sinonasal tract with emphasis on infectious diseases, vasculitis, iatrogenic, and diseases of unknown cause...
March 2017: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/28148583/anca-associated-vasculitis
#11
Max Yates, Richard Watts
The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome)...
February 2017: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/28119348/a-pragmatic-approach-to-vasculitis-in-the-gastrointestinal-tract
#12
REVIEW
Runjan Chetty, Stefano Serra
Although vasculitis involving the gastrointestinal tract (GIT) is an uncommon occurrence, occasionally vasculitis can present as haemorrhagic infarction or ischaemia for which a length of bowel is removed. Invariably, the appropriate clinical history is not forthcoming, or vasculitis is not clinically suspected. The purpose of this overview is to provide the practising gastrointestinal (GI) pathologist with a framework to recognise and diagnose vasculitides within the GIT. The classification may be approached by aetiological agent or size of vessel involved; an international consensus group now favours the latter approach...
January 24, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28095714/disease-related-and-drug-induced-skin-manifestations-in-inflammatory-bowel-disease
#13
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...
March 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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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