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Vasculitis ANCA

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https://www.readbyqxmd.com/read/28816948/medial-medullary-infarction-caused-by-antineutrophil-cytoplasmic-antibody-related-vasculitis-case-report-and-review-of-the-literature
#1
Kumi Yanagiha, Kazuhiro Ishii, Tomoyuki Ueno, Aiki Marushima, Akira Tamaoka
RATIONALE: Medial medullary infarction accounts for less than 1% of brain infarctions, and medial medullary infarctions is very rarely caused by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. PATIENT CONCERNS: We report the case of a 76-year-old man at low risk of arteriosclerosis who presented with disorders on the left side including gaze-evoked nystagmus, paralysis of the extremities, pyramidal signs, sensory disturbance, and dysesthesia. Brain magnetic resonance imaging also showed right medial medullary infarction...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28807260/anca-in-anti-gbm-disease-moving-beyond-a-one-dimensional-clinical-phenotype
#2
Mark Canney, Mark Alan Little
McAdoo et al. propose that patients with both circulating antiglomerular basement membrane antibody and antineutrophil cytoplasm antibody demonstrate a phenotype that lies between that of single-positive antiglomerular basement membrane disease and antineutrophil cytoplasm antibody-associated vasculitis. Specifically, there may be a subset of "double-positives" that have a more favorable response to therapy. These observations, along with reports of "atypical" antiglomerular basement membrane disease, challenge us to look beyond antiglomerular basement membrane disease as a 1-dimensional entity, and better characterize its clinical spectrum...
September 2017: Kidney International
https://www.readbyqxmd.com/read/28781854/skin-nodule-reveals-lung-cancer-in-a-patient-with-granulomatosis-with-polyangiitis
#3
Taylor Doberstein, Brian L Swick, Namrata Singh
A 69-year-old male with granulomatosis with polyangiitis presented with new skin nodules. Skin biopsy showed metastatic poorly differentiated lung adenocarcinoma. The skin nodule was the initial presentation of his lung cancer. There is need for increased vigilance for cancer risk in ANCA-associated vasculitis patients.
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28780080/-how-i-treat-autoimmune-diseases-state-of-the-art-on-the-management-of-rare-rheumatic-diseases-and-anca-associated-systemic-idiopathic-vasculitis
#4
EDITORIAL
Dario Roccatello
This Special Issue of Autoimmunity Reviews constitutes summaries of presentations at the 20th International Meeting on Immunopathology and Orphan Diseases, held in Torino, Italy, 25-28th January 2017. As such, these presentations represent the state of the art on the pathophysiology of autoimmune diseases as well as the most recent insights into the management of these pathologic conditions. The latter includes both the optimal use of established drugs and approaches as well as novel knowledge on the means and consequences of targeted blocking of molecules or cellular mechanisms...
August 2, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28780079/antineutrophil-cytoplasmic-antibody-associated-vasculitides-and-igg4-related-disease-a-new-overlap-syndrome
#5
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/28766857/birmingham-vasculitis-activity-score-of-more-than-9-5-at-diagnosis-is-an-independent-predictor-of-refractory-disease-in-granulomatosis-with-polyangiitis
#6
Juyoung Yoo, Ho Jae Kim, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
AIM: We investigated whether clinical manifestations, anti-neutrophil cytoplasmic antibodies (ANCAs), Birmingham vasculitis activity score (BVAS) for granulomatosis with polyangiitis (GPA) and five factor score (FFS) at diagnosis can predict relapse or refractory disease in 30 histology-proven GPA patients with follow-up duration ≥ 12 weeks. METHODS: We reviewed the medical records of 30 GPA patients. We collected clinical data, ANCAs, BVAS for GPA, FFSs at diagnosis, and we compared variables between the two groups based on relapse or refractory disease...
August 1, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28766066/outcome-of-kidney-transplantation-in-pediatric-patients-with-anca-associated-glomerulonephritis-a-single-center-experience
#7
Damien Noone, Rae S M Yeung, Diane Hebert
OBJECTIVES: Data on kidney transplant outcomes for pediatric patients with end-stage renal disease (ESRD) secondary to anti-neutrophil cytoplasmic antibody glomerulonephritis (ANCA GN), particularly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), is limited. We describe our experience of kidney transplantation in pediatric ANCA GN patients. METHODS: We performed a retrospective review of patients with ANCA GN who developed ESRD and were transplanted at a single center between the years 2000 and 2014...
August 1, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28765251/severe-infection-in-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#8
Aladdin J Mohammad, Mårten Segelmark, Rona Smith, Martin Englund, Jan-Åke Nilsson, Kerstin Westman, Peter A Merkel, David R W Jayne
OBJECTIVE: To compare the rate of severe infections after the onset of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with the rate in the background population, and to identify predictors of severe infections among patients with AAV. METHODS: The study cohort was 186 patients with AAV diagnosed from 1998 to 2010, consisting of all known cases in a defined population in southern Sweden. For each patient, 4 age- and sex-matched reference subjects were randomly chosen from the background population...
August 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28765242/pulmonary-involvement-in-antineutrophil-cytoplasmic-antibodies-anca-associated-vasculitis-the-influence-of-anca-subtype
#9
Aladdin J Mohammad, Kristian H Mortensen, Judith Babar, Rona Smith, Rachel B Jones, Daiki Nakagomi, Pasupathy Sivasothy, David R W Jayne
OBJECTIVE: To describe pulmonary involvement at time of diagnosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), as defined by computed tomography (CT). METHODS: Patients with thoracic CT performed on or after the onset of AAV (n = 140; 75 women; granulomatosis with polyangiitis, n = 79; microscopic polyangiitis MPA, n = 61) followed at a tertiary referral center vasculitis clinic were studied. Radiological patterns of pulmonary involvement were evaluated from the CT studies using a predefined protocol, and compared to proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA specificity...
August 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28758504/investigational-drugs-in-systemic-vasculitis
#10
Adrien Mirouse, Patrice Cacoub, Anne Claire Desbois, Cloé Comarmond, Christian Pagnoux, David Saadoun
Treatment of systemic vasculitis is based on glucocorticoids (GC) in association with immunosuppressive therapy. There are still unmet needs, including earlier onset of response, more targeted therapies, reduction of relapse-risk and decrease of long-term GC and classic immunosuppressants toxicities. Areas covered: In this review, we discuss investigational drugs in early phase clinical trials for induction of remission in vasculitis. We focus on ANCA-associated vasculitis, Behçet's disease, giant cell arteritis, Takayasu arteritis, and cryoglobulinemic vasculitis...
August 4, 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28754431/recommendations-of-the-brazilian-society-of-rheumatology-for-the-induction-therapy-of-anca-associated-vasculitis
#11
REVIEW
Alexandre Wagner Silva de Souza, Ana Luisa Calich, Henrique de Ataíde Mariz, Manuella Lima Gomes Ochtrop, Ana Beatriz Santos Bacchiega, Gilda Aparecida Ferreira, Jozelia Rêgo, Mariana Ortega Perez, Rosa Maria Rodrigues Pereira, Wanderley Marques Bernardo, Roger Abramino Levy
The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed), EMBASE and Cochrane database to retrieve articles until October 2016...
July 25, 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28752492/pulmonary-involvement-in-systemic-vasculitis
#12
REVIEW
Luis Felipe Flores-Suárez, Marco A Alba, Heidegger Mateos-Toledo, Natllely Ruiz
PURPOSE OF REVIEW: The purpose of this study is to describe the most relevant advances concerning lung involvement in the ANCA-associated vasculitides (excluding eosinophilic granulomatosis with polyangiitis which may have different disease mechanisms). Focus is on pathophysiology, recent important imagenological procedures, treatment, and outcome. RECENT FINDINGS: Emerging information exists on potential newly investigated diagnostic procedures (v.g. transbronchial cryobiopsies), detailed tomographic abnormalities, the potential favorable role of rituximab and the still uncertain one of plasma exchange in the treatment, and the increasing description of interstitial lung disease...
September 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28750930/positive-antineutrophil-cytoplasmic-antibody-serology-in-patients-with-lupus-nephritis-is-associated-with-distinct-histopathologic-features-on%C3%A2-renal-biopsy
#13
Tabitha Turner-Stokes, Hannah R Wilson, Massimiliano Morreale, Ana Nunes, Tom Cairns, H Terence Cook, Charles D Pusey, Ruth M Tarzi, Liz Lightstone
Class IV-S lupus nephritis is often associated with more necrosis and fewer subendothelial immune deposits compared to class IV-G lupus nephritis, suggestive of necrotising glomerular inflammation found in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. ANCAs are present in a significant proportion of patients with lupus nephritis. Here we determine whether ANCAs are associated with distinct clinical and histopathologic features of lupus nephritis. Thirty-two ANCA-positive biopsies were compared to 222 ANCA-negative biopsies from patients with lupus nephritis...
July 24, 2017: Kidney International
https://www.readbyqxmd.com/read/28746217/18f-fluoro-deoxy-glucose-positron-emission-tomography-combined-with-computed-tomography-can-reliably-rule-out-infection-and-cancer-in-patients-with-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-suspected-of-disease-relapse
#14
Evan C Frary, Søren Hess, Oke Gerke, Helle Laustrup
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases characterized by systemic inflammation in small- to medium-sized blood vessels. Although immunosuppressive therapy has greatly improved the prognosis for these patients, there are still significant comorbidities, such as cancer and infection, associated with AAV. These comorbidities are often indistinguishable from an underlying AAV disease relapse, and create a clinical conundrum, as these conditions are normally contraindications for immunosuppressive treatment...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28745689/-kidney-injury-molecules-kim-1-mcp-1-and-type-iv-collagen-in-the-assessment-of-activity-of-antineutrophil-cytoplasmic-antibody-associated-glomerulonephritis
#15
N M Bulanov, A G Serova, E I Kuznetsova, M L Bulanova, P I Novikov, L V Kozlovskaya, S V Moiseev
AIM: To assess the significance of determining the serum and urinary concentrations of monocyte chemotactic protein-1 (MCP-1), kidney injury molecule-1 (KIM-1), and type IV collagen in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to estimate the activity of renal involvement in AAV. SUBJECTS AND METHODS: 78 patients (32 men and 46 women) (median age 55 (45; 61) years) with AAV were examined. The patients were divided into 3 groups according to the AAV activity estimated using the Birmingham vasculitis activity Score (BVAS): 1) 25 patients with active ANCA-associated glomerulonephritis (GN); 2) 26 patients with active AAV without renal involvement; 3) 27 patients in sustained AAV remission...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28733791/current-clinical-evidence-of-tocilizumab-for-the-treatment-of-anca-associated-vasculitis-a-prospective-case-series-for-microscopic-polyangiitis-in-a-combination-with-corticosteroids-and-literature-review
#16
REVIEW
Ryota Sakai, Tsuneo Kondo, Takahiko Kurasawa, Eiko Nishi, Ayumi Okuyama, Kentaro Chino, Akiko Shibata, Yusuke Okada, Hirofumi Takei, Hayato Nagasawa, Koichi Amano
The purpose of this study is to report the efficacy and safety of a combination of tocilizumab (TCZ) and high-dose corticosteroid (CS) in two patients with microscopic polyangiitis (MPA) and review the published current clinical evidence on TCZ in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), except for large vessel vasculitis (LVV) and polymyalgia rheumatica (PMR). Two MPA patients were treated with TCZ at 8 mg/kg every month for 1 year and CS (prednisolone 1 mg/kg/day for 2 weeks, followed by tapering) in a prospective single-arm, single-center, cohort, open-label pilot study (UMIN clinical trials: 000012072)...
July 21, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28732837/immune-diffuse-alveolar-hemorrhage-clinical-presentation-and-outcome
#17
Silvia Quadrelli, Diana Dubinsky, Marco Solis, Demelza Yucra, Marcos Hernández, Hugo Karlen, Alejandro Brigante
BACKGROUND: Diffuse alveolar haemorrhage (DAH) is a manifestation of several immune and nonimmune diseases. OBJECTIVE: The objective of this study was to characterize the clinical characteristics and short-term outcomes of patients with immune-mediated DAH requiring hospital admission. METHODS: A retrospective study from December 2010 to December 2015, was conducted by analyzing the clinical records of 39 patients with DAH with a proven immunological origin...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28710096/response-to-mortality-in-anca-associated-vasculitis-mission-incomplete-by-moiseev-et-al
#18
Juan Antonio Avina-Zubieta, Natasha Dehghan, Ju Ann Tan
No abstract text is available yet for this article.
July 14, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28709761/central-nervous-system-involvement-in-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-report-of-26-patients-and-review-of-the-literature
#19
REVIEW
Raphaël André, Vincent Cottin, Jean-Luc Saraux, Gilles Blaison, Boris Bienvenu, Pascal Cathebras, Robin Dhote, Aurélie Foucher, Helder Gil, Joëlle Lapoirie, David Launay, Valentine Loustau, François Maurier, Edouard Pertuiset, Thierry Zénone, Jörg Seebach, Nathalie Costedoat-Chalumeau, Xavier Puéchal, Luc Mouthon, Loïc Guillevin, Benjamin Terrier
BACKGROUND: Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis (EGPA), central nervous system (CNS) manifestations are poorly described. This study aimed to describe CNS involvement in EGPA. PATIENTS AND METHODS: This retrospective, observational, multicenter study included patients with EGPA and CNS involvement affecting cranial nerves, brain and/or spinal cord. We also undertook a systematic literature review...
July 12, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28690094/-relevance-assessment-of-requests-for-antineutrophil-cytoplasmic-antibodies-detection-retrospective-study-led-in-bordeaux-hospital-france
#20
P Biscay, M Ralazamahaleo, E Lazaro, C Richez, J Seneschal, C Rigothier, C Contin-Bordes, P Duffau
INTRODUCTION: During year 2013, 5943 tests for antineutrophil cytoplasmic antibodies (ANCA) detection were performed in Bordeaux hospital, France. This seemed disproportionate, with regard to the low prevalence of ANCA-associated vasculitis (AAV). Our purpose was to evaluate the relevance of these requests. METHODS: Requests for detection of ANCA during 2013 were recorded, with their results. A sample of 501 requests was secondarily established. Relevance of requests was assessed independently by two reviewers...
July 6, 2017: La Revue de Médecine Interne
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