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Carlo Selmi, Jobert G Barin, Noel R Rose
In the broad field of autoimmunity and clinical immunology, experimental evidence over the past few years have demonstrated several connections between the immune system and the nervous system, both central and peripheral, leading to the definition of neuroimmunology and of an immune-brain axis. Indeed, the central nervous system as an immune-privileged site, thanks to the blood-brain barrier, is no longer a dogma as the barrier may be altered during chronic inflammation with disruptive changes of endothelial cells and tight junctions, largely mediated by adenosine receptors and the expression of CD39/CD73...
December 2016: Journal of Autoimmunity
A Filosa, A Verdelli, B Bianchi, E Del Bianco, L Bugatti, G Filosa, M Caproni
Cutaneous vasculitis comprises a wide spectrum of diseases that involve predominantly the blood vessels and surrounding tissues of the skin. Few vasculitic syndromes have pathognomonic clinical, radiographic and/or laboratory findings; thus, confident and accurate diagnosis of vasculitis requires histological confirmation. Skin biopsy should be done, optimally within 24 to 48 hours after vasculitic lesions appear. Deep excision biopsy must be preferred. Direct immunofluorescence of lesional skin is helpful in the diagnosis of vasculitides in the light of a proper clinico-pathological setting and diagnostic in some peculiarly forms...
April 2015: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
J Antonio Aviña-Zubieta, Vidula M Bhole, Neda Amiri, Eric C Sayre, Hyon K Choi
IMPORTANCE: Patients with giant cell arteritis (GCA) may have an increased risk of pulmonary embolism (PE), similar to other systemic vasculitidies; however, no relevant population data are available to date. OBJECTIVE: To evaluate the future risk and time trends of new venous thromboembolism (VTE) in individuals with incident GCA at the general population level. DESIGN: Observational cohort study. SETTING: General population of British Columbia...
January 2016: Annals of the Rheumatic Diseases
Eoin F McKinney, Lisa C Willcocks, Verena Broecker, Kenneth G C Smith
The small-vessel vasculitides are a group of disorders characterised by variable patterns of small blood vessel inflammation producing a markedly heterogeneous clinical phenotype. While any vessel in any organ may be involved, distinct but often overlapping sets of clinical features have allowed the description of three subtypes associated with the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCA), namely granulomatosis with polyangiitis (GPA, formerly known as Wegener's Granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (eGPA, formerly known as Churg-Strauss syndrome)...
July 2014: Seminars in Immunopathology
S Pérez-Esteban, M A González-Gay, S Castañeda
The term large vessel vasculitidis includes two distinct clinical entities: Takayasu's arteritis and giant cell arteritis. Takayasu's arteritis mainly affects children and women under 40 years, affecting the aorta and its major branches. Its incidence in Southern Europe is low. Giant cell arteritis is the most common vasculitis in those 60. Its incidence in the European population is greater than that of Takayasu's arteritis. It mainly affects the extracranial vessels, especially those derived from the carotid artery...
October 2013: Revista Clínica Espanõla
Odul Egritas Gurkan, Buket Dalgic
BACKGROUND AND OBJECTIVES: Familial Mediterranean fever (FMF) and inflammatory bowel disease togetherness is well described in the literature. Abdominal pain and various gastrointestinal manifestations may arise directly from FMF or secondary to FMF-associated diseases such as inflammatory bowel disease, vasculitidies, or amyloidosis. The aim of the study was to document gastrointestinal involvement in familial Mediterranean fever. METHODS: The medical files of the patients who were diagnosed as having FMF at the Department of Pediatric Gastroenterology, Gazi University School of Medicine between 2007 and 2012 were examined retrospectively...
September 2013: Journal of Pediatric Gastroenterology and Nutrition
Luis Felipe Flores-Suárez
The primary vasculitidies are complex diseases with varied clinical manifestations, which may be common to those present in multiple diseases. The antineutrophil cytoplasm autoantibodies (ANCA) led to a revolution in the diagnosis and research of these diseases, being the first and so far, the only biomarkers for three of these diseases, which affect small caliber vessels. From their description, much progress has been made, but there are still gray or misunderstood areas regarding their best use in the clinic...
November 2012: Reumatología Clinica
Ignacio Villa, Mario Agudo Bilbao, Víctor M Martínez-Taboada
Depending on the geographic area and the affected age group, large vessel vasculitis (LVV) are one of the most frequent types of systemic vasculitidies. Whereas giant cell arteritis (GCA) occurs exclusively in elderly individuals and is more frequent in countries with European ancestry, Takayasús arteritis (TA) mainly affects young women from eastern countries. The diagnosis of these disorders has been based on the characteristic clinical picture, together with the typical histopathological features in the temporal artery of patients with GCA, and the angiography results in TA patients...
December 2011: Reumatología Clinica
Olga E Beltrán Rodríguez Cabo, Gabriel Tona Acedo
First manifestations of vasculitis can appear on the head and neck, still few physicians are aware of these diseases, as they are unspecific and because other conditions like infections and allergies are more frequent. Among them, signs and symptoms like epistaxis, nasal obstruction, discharge, burning pain of sinonasal region, headache, sinus polyps and crusts, the latter found in people who live in highly polluted cities with low humidity, in patients with respiratory scleroma, atrophy, those receiving local radiotherapy, and due to other conditions...
December 2011: Reumatología Clinica
Beatriz Zazueta Montiel, Luis Felipe Flores Suárez
The primary systemic vasculitidies (PSV) comprise an heterogeneous group of complex clinical entities with a common substrate: inflammation and necrosis of blood vessels. The inflammatory process involves vessels of any caliber. The location and the different size of the affected vessels, the severity of vascular damage and the different histopathological patterns which may predominate are the basic characteristics that define the different vasculitic syndromes and enable individualization. The description and progress in the knowledge of the primary vasculitidies have evolved considerably in recent decades, allowing for a better resolution of the complex problems placed by these patients...
December 2011: Reumatología Clinica
Fleur Bossi, Ellinor I Peerschke, Berhane Ghebrehiwet, Francesco Tedesco
The endothelium is a continuous physical barrier that regulates coagulation and selective passage of soluble molecules and circulating cells through the vessel wall into the tissue. Due to its anatomic localization, the endothelium may establish contact with components of the complement, the kinin and the coagulation systems which are the main, though not exclusive, inducers of vascular leakage. Although the complement and the kinin systems may act independently, increasing evidence suggest that there is a crosstalk that involve different components of both systems...
October 30, 2011: Immunology Letters
M Oz, T Hazirolan, B Turkbey, A D Karaosmanoglu, M Canyigit, B Peynircioglu
The emergence of CT angiography (CTA) has a groundbreaking impact on the evaluation of renal vessels and is gradually replacing the conventional catheter angiography as the standard imaging procedure. In this review, we aimed to describe the renal CTA technique and imaging findings of several renal arterial (i.e. atherosclerosis, fibromuscular dysplasia, aneurysms of the renal arteries, dissection, vasculitidis, follow-up of patients with renal arterial stent) and venous (i.e. nut-cracker syndrome, pelvic congestion syndrome) pathologies...
September 2010: JBR-BTR: Organe de la Société Royale Belge de Radiologie (SRBR)
Giuseppe Cocco, Armen Yuri Gasparyan
Wegener's granulomatosis (WG) is one of the most common small- and medium-sized necrotizing vasculitides that mainly affects the upper and lower respiratory tract and the kidneys. Cardiac manifestations in WG are relatively rare, and their role and place among different causes of mortality remain largely unknown. Substantially increased number of reports describing involvement of all structures of the heart, which underlie conduction disturbances, valvular disease, ischemic heart disease and other potentially serious conditions, underscores importance of comprehensive cardiovascular investigations and monitoring of patients with WG...
2010: Open Cardiovascular Medicine Journal
Roberto Bovo, Andrea Ciorba, Alessandro Martini
Autoimmune inner ear disease probably accounts for less than 1% of all cases of balance disorders, but its incidence is often overlooked due to the absence of a specific diagnostic test. Furthermore, in several systemic autoimmune diseases the vestibulo-cochlear system may be affected. Clinical features comprise generalized imbalance, ataxia, motion intolerance, episodic vertigo and positional vertigo. An autoimmune mechanism seems to be responsible for 6% of unilateral and 16% of bilateral forms of Ménière's disease...
January 2010: European Archives of Oto-rhino-laryngology
Hilary F Ryder, Laura J Tafe, Christopher M Burns
Aortitis is typically a chronic, progressive disease manifestation associated with large vessel vasculitidies, most notably giant cell, Takayasu arteritis, and a newly described entity, isolated aortitis. The aortitis may lead to aneurysm formation and symptoms associated with branch vessel occlusion in these diseases, but aortic dissection is rare and usually a late complication of smoldering, incompletely treated disease. We present a case of aortitis in a previously healthy 39-year-old man who succumbed to aortic dissection hours after the onset of symptoms...
September 2009: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Kenneth T Calamia, Phaedon G Kaklamanis
Behçet's disease (BD) is a chronic, multisystem, inflammatory disorder that is classified among the systemic vasculitidies. Its cause has yet to be determined, but genetic and environmental factors and immune dysregulation are thought to play a role in its pathogenesis. The diagnosis of the disease and recognition and separation from disorders that may mimic BD may be difficult, especially in areas where the disease is uncommon. Certainty as to the best treatments for the various manifestations of BD is often unclear due to limited randomized controlled trials...
October 2008: Current Rheumatology Reports
Ralph Kettritz
Renal involvement in autoimmunity has many facets. Glomerular, tubular and vascular structures are targeted and damaged as a consequence of autoimmune processes. Most dramatic and life-threatening causes are observed with diseases that result in rapidly progressive glomerulonephritis (GN), frequently accompanied by involvement of additional non-renal organs. Typical diseases with these characteristics are anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitidis, anti-glomerular basement membrane (anti-GBM) GN and proliferative lupus nephritis...
2008: Scandinavian Journal of Clinical and Laboratory Investigation. Supplementum
Loïc Guillevin, Christian Pagnoux
Systemic vasculitides are characterized by different histological aspects: fibrinoid necrosis of the arterial wall, giant cell arteritis, non-necrotizing arteritides without granuloma or giant cell infiltration. Each histological form is associated with a spectrum of diseases with variable clinical expression: giant cell angeitides, such as Takayasu's arteritis and giant cell arteritis, necrotizing angeitides, such as polyarteritis nodosa, Kawasaki disease, Wegener's granulomatosis, Henoch-Schönlein purpura or Churg-Strauss syndrome...
March 15, 2008: La Revue du Praticien
Osvaldo Camilo, Larry B Goldstein
There are a large variety of non-atherosclerotic causes of ischemic stroke in the young. Arterial dissection, most commonly associated with non-traumatic causes, is among the most common. Both the carotid and vertebrobasilar circulations can be affected. The vasculitidies represent a rare, but potentially treatable series of conditions that can lead to stroke through diverse mechanisms. Moyamoya is a nonatherosclerotic, noninflammatory, nonamyloid vasculopathy characterized by chronic progressive stenosis or occlusion of the distal internal carotid arteries and/or proximal portions of the middle and/or anterior cerebral arteries...
October 2005: Journal of Thrombosis and Thrombolysis
C J Day, P Hewins, C O Savage
In recent years there have been substantial developments in the understanding of the pathogenesis of ANCA-associated vasculitidies. Animal models have now been developed that finally prove a direct pathogenic role for ANCA, a subject fiercely debated since their original identification. We are also closer to understanding how ANCA exert their effects to cause disease. Progress has been made in elucidating how ANCA activate neutrophils, from how they bind antigen and where that antigen is located, to how antigen binding is translated into intracellular activity...
November 2003: Clinical and Experimental Rheumatology
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