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Antineutrophil cytoplasmic antibodies

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https://www.readbyqxmd.com/read/28229991/a-novel-mutation-in-the-pyrin-domain-of-the-nod-like-receptor-family-pyrin-domain-containing-protein-3-in-muckle-wells-syndrome
#1
Jian Hu, Yun Zhu, Jian-Zhong Zhang, Rong-Guang Zhang, Hou-Min Li
BACKGROUND: Cryopyrin-associated periodic syndrome (CAPS) is a group of rare, heterogeneous autoinflammatory disease characterized by interleukin (IL)-1β-mediated systemic inflammation and clinical symptoms involving skin, joints, central nervous system, and eyes. It encompasses a spectrum of three clinically overlapping autoinflammatory syndromes including familial cold autoinflammatory syndrome, Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease. CAPS is associated with gain-of-function missense mutations in NOD-like receptor family pyrin domain-containing protein 3 (NLRP3), the gene encoding NLRP3...
2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28229830/neutrophil-extracellular-traps-formation-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis-association-with-eosinophilic-inflammation
#2
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/28217616/cocaine-induced-vasculitis-with-cutaneous-manifestation-a-recurrent-episode-after-2-years
#3
Thein Swe, Mona Pervil-Ulysse, Aam A Baqui
Cocaine is a popular recreational drug in the United States, and up to 70% of the seized cocaine contains levamisole which is an antihelminthic that can cause cutaneous vasculitis with necrosis and positive antineutrophil cytoplasmic antibodies (ANCAs). Here, we report a unique case of recurrent cocaine-induced vasculitis in a patient who smokes cocaine for more than 20 years. A 38-year-old woman complained of painful erythematous rash in her right arm and right thigh which appeared some hours after smoking cocaine...
July 2016: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/28214498/vasculitic-and-autoimmune-wounds
#4
REVIEW
Victoria K Shanmugam, Divya Angra, Hamza Rahimi, Sean McNish
OBJECTIVE: Chronic wounds are a major cause of morbidity and mortality. Approximately 20% to 23% of nonhealing wounds that are refractory to vascular intervention have other causes, including vasculitis, pyoderma gangrenosum, and other autoimmune diseases. The purpose of this article was to review the literature across medical and surgical specialties with regard to refractory chronic wounds associated with vasculitis and autoimmune diseases and to delineate clinical outcomes of these wounds in response to vascular and other interventions...
March 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28202741/seasonal-influence-on-the-risk-of-relapse-at-a-rise-of-antineutrophil-cytoplasmic-antibodies-in-vasculitis-patients-with-renal-involvement
#5
Michael J Kemna, Jan Willem Cohen Tervaert, Kelly Broen, Sjoerd A M E G Timmermans, Pieter van Paassen, Jan G M C Damoiseaux
OBJECTIVE: The objective of this study was to identify risk factors for a relapse at the time of an increase in antineutrophil cytoplasmic antibodies (ANCA) in patients with renal ANCA-associated vasculitis (AAV). METHODS: All patients between January 2000 and November 2011 with renal AAV having an ANCA rise during remission were included. Differences in time to relapse since the ANCA rise were assessed using a Cox regression model. The level of 25-hydroxy Vitamin D (25(OH)D) was assessed at the ANCA rise and at a subsequent relapse or time-matched during remission...
February 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28181422/hmgb1-contributes-to-glomerular-endothelial-cell-injury-in-anca-associated-vasculitis-through-enhancing-endothelium-neutrophil-interactions
#6
Chen Wang, Dong-Yuan Chang, Min Chen, Ming-Hui Zhao
Our previous studies demonstrated that high mobility group box-1 (HMGB1), a typical damage-associated molecular pattern (DAMP) protein, is associated with the disease activity of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Moreover, HMGB1 participates in ANCA-induced neutrophil activation. The current study aimed to investigate whether HMGB1 regulated the interaction between neutrophils and glomerular endothelial cells (GEnC) in the presence of ANCA. Correlation analysis on HMGB1 levels in AAV patients and soluble intercellular cell adhesion molecule-1 (sICAM-1) levels or vascular endothelial growth factor (VEGF) levels, which are markers of endothelial cell activation, was performed...
February 9, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28172776/p291-proteinase-3-antineutrophil-cytoplasmic-antibodies-pr3-anca-would-be-a-predictive-biomarker-for-the-clinical-course-of-ulcerative-colitis
#7
M Sono, T Yoshino, S Yazumi
No abstract text is available yet for this article.
February 1, 2017: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/28157661/eosinophilic-endomyocardial-disease
#8
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.
January 23, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28148583/anca-associated-vasculitis
#9
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/28134583/pathology-of-antineutrophil-cytoplasmic-antibody-associated-pulmonary-and-renal-disease
#10
Maxwell L Smith
CONTEXT: -Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis commonly presents with pulmonary and renal involvement that may present diagnostic challenges. OBJECTIVES: -To highlight the updates in the classification of small vessel vasculitis, present the patterns of pulmonary and renal pathology in which ANCA-associated vasculitis is included in the differential diagnosis, analyze the screening and specific antineutrophil cytoplasmic antibody testing methods in the clinical laboratory, compare and contrast the 3 major ANCA-associated vasculitis diseases, and review the pathophysiologic mechanisms of tissue injury in this setting...
February 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28134075/cyclophosphamide-followed-by-rituximab-for-aggressive-multiple-relapsing-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#11
Sophia Lionaki, George E Fragoulis, Aliki Venetsanopoulou, Panagiotis Vlachoyiannopoulos, John N Boletis, Athanasios G Tzioufas
OBJECTIVES: To evaluate the long term outcomes of patients with multi-relapsing antineutrophil cytoplasmic antibody-associated vasculitis (AAV), who received sequentially therapy with cyclophosphamide and rituximab, upon new onset of aggressive vasculitis. METHODS: We retrospectively studied patients with multiple-relapsing AAV, who were treated with the standard regimen plus rituximab, given in sequence, upon a major relapse, compared to historical patients, who had been treated with the standard regimen alone in the same setting...
January 25, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28134074/chest-and-renal-involvements-birmingham-vascular-activity-score-more-than-13-5-and-five-factor-score-1996-more-than-1-at-diagnosis-are-significant-predictors-of-relapse-of-microscopic-polyangiitis
#12
Yoon-Jeong Oh, Sung Soo Ahn, Eun Seong Park, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
OBJECTIVES: We investigated whether specified organ involvements, antineutrophil cytoplasmic antibody (ANCA) positivity, Birmingham vasculitis activity score (BVAS) and five factor scores (FFS) at diagnosis could predict relapse of microscopic polyangiitis (MPA). METHODS: We reviewed the medical records of 90 patients with MPA. We collected clinical and prognostic data, (MPO)-ANCA and proteinase 3 (PR3)-ANCA, BVAS and FFS at diagnosis, and we compared them between the two groups...
January 19, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28131439/-antineutrophil-cytoplasmic-antibodies-associated-with-infective-endocarditis-literature-review
#13
V Langlois, I Marie
Antineutrophil cytoplasmic antibodies (ANCA) associated with infective endocarditis are a rare disorder. The condition can mimic primary systemic vasculitis (i.e. granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis). Thus, a wrong diagnosis of valvular involvement related to primary systemic vasculitis can be made in patients exhibiting ANCA associated with infective endocarditis. Because treatment of both conditions is different, this wrong diagnosis will lead to dramatic consequences in these latter patients...
January 25, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28124664/screening-for-renal-involvement-in-anca-associated-vasculitis-room-for-improvement
#14
E Houben, J W van der Heijden, B van Dam, W A Bax, A E Voskuyl, E L Penne
BACKGROUND: Renal involvement in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) requires prompt and aggressive immunosuppressive therapy. The aim of this study was to evaluate screening practice for renal involvement in AAV and its potential effect on renal outcomes. METHODS: Between 2005 and 2015, ANCA-positive AAV patients in a teaching hospital in the Netherlands were retrospectively included. Complete screening for renal involvement was defined as: assessment of erythrocyturia, proteinuria and serum creatinine within two weeks of the diagnosis of AAV...
January 2017: Netherlands Journal of Medicine
https://www.readbyqxmd.com/read/28119348/a-pragmatic-approach-to-vasculitis-in-the-gastrointestinal-tract
#15
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/28110970/-plasma-exchange-in-nephrology-indications-and-technique
#16
Christophe Ridel, Sébastien Kissling, Laurent Mesnard, Alexandre Hertig, Éric Rondeau
Plasma exchange is a non-selective apheresis technique that can be performed by filtration or centrifugation allowing rapid purification of high molecular weight pathogens. An immunosuppressive treatment is generally associated to reduce the rebound effect of the purified substance. Substitution solutes such as human albumin and macromolecules are needed to compensate for plasma extraction. Compensation by viro-attenuated plasma is reserved solely for the treatment of thrombotic microangiopathies or when there is a risk of bleeding, because this product is very allergenic and expensive...
January 16, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28109377/antineutrophil-cytoplasmic-antibody-associated-multiple-giant-saccular-aortic-aneurysms
#17
Ryoji Kinoshita, Tomohiro Mizuno, Tsuyoshi Hachimaru, Keiji Oi, Masafumi Yashima, Eiki Nagaoka, Tatsuki Fujiwara, Hidehito Kuroki, Dai Tasaki, Hirokuni Arai
We describe a very rare case of a 67-year-old man with multiple saccular aortic aneurysms throughout the entire aorta due to antineutrophil cytoplasmic antibody-associated vasculitis (AAV). The patient underwent staged aortic surgical procedures, including stent-graft insertion for a left iliac artery aneurysm, thoracic endovascular aortic repair for a descending aortic aneurysm, and total replacement of the ascending aorta and aortic arch with the use of high-dose steroids to control inflammation. The histologic findings demonstrated that the damage to the vasa vasorum of the adventitia resulting from AAV caused ischemia of the media, resulting in the formation of saccular aneurysmal changes...
February 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28089971/incidence-of-malignancy-prior-to-antineutrophil-cytoplasmic-antibody-associated-vasculitis-compared-to-the-general-population
#18
Emma E van Daalen, Chinar Rahmattulla, Ron Wolterbeek, Jan A Bruijn, Ingeborg M Bajema
OBJECTIVE: Previous studies have reported an increased malignancy risk preceding antineutrophil cytoplasmic antibody-associated vasculitis (AAV), suggesting common pathogenic pathways in these 2 entities. However, the study results were conflicting and often limited to patients with granulomatosis with polyangiitis (GPA). Here, we study the malignancy risk prior to AAV diagnosis [either GPA or microscopic polyangiitis (MPA)] to elaborate on the putative association between malignancy and AAV...
January 15, 2017: Journal of Rheumatology
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
#19
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/28077133/t1-and-t2-mapping-for-evaluation-of-myocardial-involvement-in-patients-with-anca-associated-vasculitides
#20
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
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