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Vasculitides

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https://www.readbyqxmd.com/read/28929493/pediatric-vasculitis-a-single-center-experience
#1
Alexios Alexopoulos, Maria Dakoutrou, Kalliopi Stefanaki, George Chrousos, Talia Kakourou
BACKGROUND: Existing studies of children with vasculitis are limited. The aim of this study was to assess the epidemiology, clinical manifestations, laboratory findings, course, and outcome of Greek children presenting with vasculitic rash. METHODS: The relevant data included in the study were collected retrospectively using a standardized form from children who were admitted into our department between 2003 and 2013, with the provisional diagnosis of vasculitis of the skin...
September 19, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28918161/clinical-and-pathological-significance-of-cutaneous-manifestations-in-anca-associated-vasculitides
#2
REVIEW
Laure Frumholtz, Sara Laurent-Roussel, Olivier Aumaître, François Maurier, Guillaume Le Guenno, Agnes Carlotti, Alexiane Dallot, Jean Louis Kemeny, Laurent Antunes, Nicolas Froment, Sylvie Fraitag, Jonathan London, Alice Berezne, Benoît Terris, Claire Le Jeunne, Luc Mouthon, Selim Aractingi, Loïc Guillevin, Nicolas Dupin, Benjamin Terrier
OBJECTIVES: Cutaneous manifestations (CM) in ANCA-associated vasculitides (AAV) are frequent, but data on clinical significance and clinical-pathological correlations are lacking. METHODS: We conducted a multicenter, retrospective study including 1553 AAV patients. Clinical, biological and pathological features have been analyzed, and tissue samples from 46 biopsies were reviewed in a blind manner. RESULTS: CM were more frequent in EGPA (53...
September 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28916114/-scleritis-and-episcleritis
#3
REVIEW
E Héron, T Bourcier
Episcleritis is generally a benign disease with a self-limited course, while scleritis is a severe ocular condition due to a risk of impaired vision in one-fifth of patients and its association with systemic diseases in one third of them. Infectious scleritis, representing 8 % of the etiologies, is mainly of herpetic origin (varicella zoster and herpes simplex viruses). A systemic autoimmune disease is observed in roughly 30 % of scleritis patients: inflammatory rheumatisms (15 %), firstly rheumatoid polyarthritis, systemic vasculitides (8 %), mainly granulomatosis with polyangiitis (Wegener's) and polychondritis, and less often inflammatory bowel disease and systemic lupus erythematosus...
September 12, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/28895041/unmet-needs-in-the-pathogenesis-and-treatment-of-vasculitides
#4
REVIEW
Francesco Muratore, Giulia Pazzola, Alessandra Soriano, Nicolò Pipitone, Stefania Croci, Martina Bonacini, Luigi Boiardi, Carlo Salvarani
Despite the progress in the last years on the field of vasculitides, there are several unmet needs regarding classification, disease activity assessment, predictors of flares and complications, and type of treatment for the different forms. The 1990 American College of Rheumatology (ACR) classification criteria currently used to define giant cell arteritis and Takayasu arteritis were designed to discriminate between different types of vasculitides but not to differentiate vasculitis from other disorders. Recently, efforts have been made to overcome the shortcomings of the ACR criteria...
September 11, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28889234/peripheral-arterial-tonometry-in-pulmonary-vasculitis
#5
A Falkowski, K A Wardyn, K Życińska
Vascular wall inflammation in primary vasculitides results in diminished vessel dilation and finally impaired blood flow, causing multiple organs dysfunction and ultimate damage. In granulomatosis with polyangiitis (GPA), the inflammatory process concerns small and medium sized vessels and its pulmonary location is often predominant. The pivotal role in the development of that pathology plays vascular endothelium. Endothelial vasodilatory function strongly depends on the instant production and release of nitrogen oxide (NO), a potent local factor controlling vascular tonus...
September 10, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28881714/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-years-observation-study
#6
Dario Roccatello, Savino Sciascia, Daniela Rossi, Mirella Alpa, Carla Naretto, Massimo Radin, Roberta Fenoglio, Simone Baldovino, Elisa Menegatti
BACKGROUND: ANCA associated vasculitides (AAV) often present with a chronic relapsing course. Relapse leads to increased immunosuppressive exposure and consequent toxicity. While two randomized controlled trials have shown rituximab (RTX) to be the most effective induction treatment in patients with relapsing disease, the optimal treatment duration and RTX dose remain debated. Whether to administer a maintenance dose to every patient, at a fixed time interval or on the basis of B cell count and ANCA titre or only when disease manifestations do occur is still debated as well...
August 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28868297/a-toddler-presenting-with-pulmonary-renal-syndrome
#7
Florence A Aeschlimann, Rae S M Yeung, Ronald M Laxer, Diane Hebert, Ashley Cooper, Rose Chami, Damien Noone
Pulmonary renal syndrome refers to an association of pulmonary and glomerular disease and includes disorders, such as the ANCA-associated vasculitides, anti-glomerular basement membrane antibody disease, systemic lupus erythematosus, and IgA vasculitis (Henoch-Schönlein purpura). We present the medical history of a 26-month-old boy with an extensive purpuric rash, involving the limbs, trunk, and face, who developed clinically significant pulmonary hemorrhage and renal involvement. Rapid recognition of this rare but potentially life-threatening condition is crucial...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28864650/omeract-endorsement-of-patient-reported-outcome-instruments-in-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#8
Joanna C Robson, Gunnar Tomasson, Nataliya Milman, Sue Ashdown, Annelies Boonen, George C Casey, Peter F Cronholm, David Cuthbertson, Jill Dawson, Haner Direskeneli, Ebony Easley, Tanaz A Kermani, John T Farrar, Don Gebhart, Georgia Lanier, Raashid A Luqmani, Alfred Mahr, Carol A McAlear, Jacqueline Peck, Beverley Shea, Judy A Shea, Antoine G Sreih, Peter S Tugwell, Peter A Merkel
OBJECTIVE: The antineutrophil cytoplasmic antibody-associated vasculitides (AAV) are multiorgan diseases. Patients with AAV report impairment in their health-related quality of life (HRQOL) and have different priorities regarding disease assessment compared with physicians. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group previously received endorsement for a core set of domains in AAV. Two approaches to measure patient-reported outcomes (PRO) were presented at OMERACT 2016...
September 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28864202/pathogenesis-of-thromboangiitis-obliterans-gene-polymorphism-and-immunoregulation-of-human-vascular-endothelial-cells
#9
REVIEW
Xiao-Lei Sun, Betty Yuen-Kwan Law, Ivo Ricardo de Seabra Rodrigues Dias, Simon Wing Fai Mok, Yan-Zheng He, Vincent Kam-Wai Wong
Thromboangiitis obliterans (TAO) is a nonatherosclerotic, segmental, inflammatory vasculitis, which commonly affects the small- and medium-sized arteries of the upper and lower extremities. Despite its discovery more than a century ago, little progress has been made in its treatment. Unless the pathogenesis is elucidated, therapeutic approaches will be limited. The purpose of this review article is to collate current knowledge of mechanisms for the pathogenesis of thromboangiitis obliterans and to propose potential mechanisms from a genetic and immunoreactive point of view for its inception...
August 18, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28861674/-ocular-involvement-in-rheumatoid-arthritis-connective-tissue-diseases-and-vasculitis
#10
REVIEW
I Kötter, N Stübiger, C Deuter
There are many interfaces between ophthalmologists and rheumatologists. On the one hand ophthalmologists face the question if an inflammation of the eye is caused by systemic inflammatory rheumatic diseases and on the other hand rheumatologists have to consider that ocular manifestations are relatively common in some inflammatory rheumatic diseases. Furthermore, these ocular manifestations may influence therapeutic decisions of the rheumatologist. This article summarizes which ocular inflammations can be associated with rheumatoid arthritis, connective tissue diseases and vasculitides...
August 31, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28860896/ct-features-of-vasculitides-based-on-the-2012-international-chapel-hill-consensus-conference-revised-classification
#11
Jee Hye Hur, Eun Ju Chun, Hyon Joo Kwag, Jin Young Yoo, Hae Young Kim, Jeong Jae Kim, Kyung Won Lee
Vasculitis, characterized by inflammation of vessel walls, is comprised of heterogeneous clinicopathological entities, and thus poses a diagnostic challenge. The most widely used approach for classifying vasculitides is based on the International Chapel Hill Consensus Conference (CHCC) nomenclature system. Based on the recently revised CHCC 2012, we propose computed tomography (CT) features of vasculitides and a differential diagnosis based on location and morphological characteristics. Finally, vasculitis mimics should be differentiated, because erroneous application of immunosuppressive drugs on vasculitis mimics may be ineffective, even deteriorating...
September 2017: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
https://www.readbyqxmd.com/read/28854963/takayasu-s-arteritis-a-case-with-relapse-after-urgent-coronary-revascularization
#12
Klaus Empen, Astrid Hummel, Daniel Beug, Stephan B Felix, Mathias C Busch, Piotr M Kaczmarek
BACKGROUND: Vasculitides are commonly unrecognized causes of coronary stenosis and myocardial ischemia. We report on a 24-year old patient with Takayasu's arteritis who underwent urgent percutaneous coronary intervention, suffered from symptomatic restenosis of the left main coronary artery during standard immunosuppressive therapy. CASE PRESENTATION: A 24-year old woman was referred for coronary angiography because of typical progressive angina pectoris. On bicycle ergometry, there were both reproducible symptoms and deep ST segment depressions on precordial leads...
July 25, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28836099/cutaneous-manifestations-of-small-vessel-leukocytoclastic-vasculitides-in-childhood
#13
REVIEW
Sebastiano A G Lava, Gregorio P Milani, Emilio F Fossali, Giacomo D Simonetti, Carlo Agostoni, Mario G Bianchetti
In childhood, cutaneous small-vessel vasculitides include Henoch-Schönlein syndrome, a systemic vasculitis, and Finkelstein-Seidlmayer syndrome, a skin-limited vasculitis. Both Henoch-Schönlein and Finkelstein-Seidlmayer syndromes are seen more frequently in white or Asian compared with black children and occur especially in winter and spring with a male-to-female ratio of approximately 2:1. In everyday clinical practice, both conditions are diagnosed on clinical grounds without histological confirmation...
August 24, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28835902/the-microbiome-in-connective-tissue-diseases-and-vasculitides-an-updated-narrative-review
#14
REVIEW
Rossella Talotta, Fabiola Atzeni, Maria Chiara Ditto, Maria Chiara Gerardi, Piercarlo Sarzi-Puttini
OBJECTIVE: To provide a narrative review of the most recent data concerning the involvement of the microbiome in the pathogenesis of connective tissue diseases (CTDs) and vasculitides. METHODS: The PubMed database was searched for articles using combinations of words or terms that included systemic lupus erythematosus, systemic sclerosis, autoimmune myositis, Sjögren's syndrome, undifferentiated and mixed CTD, vasculitis, microbiota, microbiome, and dysbiosis. Papers from the reference lists of the articles and book chapters were reviewed, and relevant publications were identified...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28817388/ophthalmologic-manifestations-of-systemic-vasculitis
#15
George N Papaliodis
PURPOSE OF REVIEW: To review the systemic vasculitides and associated ocular manifestations with emphasis on publications within the last 12 months. RECENT FINDINGS: There are multiple case reports demonstrating atypical ocular manifestations of systemic vasculitis. Often the eye findings are the initial presentation of the disorder and require a high degree of clinical suspicion to evaluate further as these conditions can compromise vision but some may also be life threatening...
August 16, 2017: Current Opinion in Ophthalmology
https://www.readbyqxmd.com/read/28816740/the-value-of-direct-immunofluorescence-on-proteinase-digested-formalin-fixed-paraffin-embedded-skin-biopsies
#16
Aida Valencia-Guerrero, April Deng, Karen Dresser, Gail Bouliane, Kristine M Cornejo
Direct immunofluorescence (DIF) on frozen tissue (DIF-F) is the method of choice for the identification of immune deposits present in skin and other tissues. DIF can also be performed on formalin-fixed paraffin-embedded tissue (DIF-P) after antigen retrieval with proteases and has proven to be of value in renal pathology. However, its utility in skin biopsies has not been fully examined. In this study, we performed DIF-P on 60 skin biopsies that comprised of bullous pemphigoid (n = 18), pemphigoid gestationis (n = 1), pemphigus (n = 7), linear IgA disease (n = 7), vasculitis (n = 20), lupus erythematosus (n = 3), and dermatitis herpetiformis (n = 4) cases...
August 9, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28806184/noninfectious-aortitis-what-the-cardiologist-needs-to-know
#17
Ingolf Töpel
PURPOSE OF REVIEW: To sum up a group of noninfectious inflammatory diseases of the aorta and its branches relevant to a cardiologist's daily routine. To describe pathogenetic and clinical advances as well as modern diagnostic tools. To overview most recent treatment options and patient-tailored therapies. To provide an insight in future directions of research. RECENT FINDINGS: Pathophysiology of large vessel vasculitides (LVV) are still poorly defined. At least a certain number of patients with idiopathic periaortitis seem to part of the group of IgG4-related diseases which has implications for therapy...
August 12, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28803590/differential-protein-analysis-of-serum-exosomes-post-intravenous-immunoglobulin-therapy-in-patients-with-kawasaki-disease
#18
Li Zhang, Qi-Fang Song, Jing-Jie Jin, Ping Huang, Zhou-Ping Wang, Xiao-Fei Xie, Xiao-Qiong Gu, Xue-Juan Gao, Hong-Ling Jia
BACKGROUND: Kawasaki disease, which is characterised by systemic vasculitides accompanied by acute fever, is regularly treated by intravenous immunoglobulin to avoid lesion formation in the coronary artery; however, the mechanism of intravenous immunoglobulin therapy is unclear. Hence, we aimed to analyse the global expression profile of serum exosomal proteins before and after administering intravenous immunoglobulin. METHODS: Two-dimensional electrophoresis coupled with mass spectrometry analysis was used to identify the differentially expressed proteome of serum exosomes in patients with Kawasaki disease before and after intravenous immunoglobulin therapy...
August 14, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28780079/antineutrophil-cytoplasmic-antibody-associated-vasculitides-and-igg4-related-disease-a-new-overlap-syndrome
#19
REVIEW
François-Xavier Danlos, Giovanni Maria Rossi, Daniel Blockmans, Giacomo Emmi, Andreas Kronbichler, Stéphane Durupt, Claire Maynard, Luminita Luca, Cyril Garrouste, Bertrand Lioger, Rachel Mourot-Cottet, Robin Dhote, Jean-Benoit Arlet, Thomas Hanslik, Philippe Rouvier, Mikael Ebbo, Xavier Puéchal, Dominique Nochy, Agnès Carlotti, Luc Mouthon, Loïc Guillevin, Augusto Vaglio, Benjamin Terrier
OBJECTIVE: Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. METHODS: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria...
August 2, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28774453/imaging-of-childhood-vasculitis
#20
REVIEW
Claudio Granata, Maria Beatrice Damasio, Federico Zaottini, Sonia Airaldi, Clara Malattia, Giovanna Stefania Colafati, Paolo Tomà, Gianmichele Magnano, Carlo Martinoli
Pediatric vasculitides are rare conditions that can represent a diagnostic challenge because symptoms are usually aspecific and variable. Symptoms are related to the size of the involved vessel, extension of disease, and organs affected. The outcome is closely linked to an early diagnosis and proper treatment. Diagnostic imaging allows visualization of the involvement of large-size and medium-size vessels and assesses end-organ changes and response to therapy, thus playing a pivotal role in the diagnosis and treatment...
September 2017: Radiologic Clinics of North America
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