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eosinophilic granulomatosis with polyangiitis

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https://www.readbyqxmd.com/read/29037900/autoimmune-comorbidity-in-chronic-spontaneous-urticaria-a-systematic-review
#1
REVIEW
Pavel Kolkhir, Elena Borzova, Clive Grattan, Riccardo Asero, Dmitry Pogorelov, Marcus Maurer
Background and Objective Numerous autoimmune diseases (AIDs) have been linked to chronic spontaneous urticaria (CSU). Here, we provide the first extensive and comprehensive evaluation of the prevalence of AIDs in patients with CSU and vice versa. METHODS: A Pubmed and Google Scholar search was performed to identify studies reporting the prevalence of various AIDs in CSU and vice versa published before April 2017. RESULTS: The prevalence of individual AIDs in CSU is increased (≥1% in most studies vs ≤1% in general population)...
October 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29022232/a-case-of-fatal-perimyocarditis-due-to-a-rare-disease
#2
Stefanie Plenzig, Sara Heinbuch, Hannelore Held, Marcel A Verhoff, Constantin Lux
Although myocarditis is caused by viral infections in about 50% of cases in European countries, various other causative agents are known. We report the case of a 51-year-old man who died several months after being diagnosed with asthma by his general practitioner. This diagnosis had been confirmed by a pulmonologist approximately 6 weeks before the man's death. To rule out the possibility of medical malpractice the prosecuting authority ordered a forensic autopsy. At autopsy macroscopic indicators for perimyocarditis and pneumonia were found...
October 11, 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/29016646/methotrexate-versus-cyclophosphamide-for-remission-maintenance-in-anca-associated-vasculitis-a-randomised-trial
#3
Federica Maritati, Federico Alberici, Elena Oliva, Maria L Urban, Alessandra Palmisano, Francesca Santarsia, Simeone Andrulli, Laura Pavone, Alberto Pesci, Chiara Grasselli, Rosaria Santi, Bruno Tumiati, Lucio Manenti, Carlo Buzio, Augusto Vaglio
OBJECTIVES: The treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is based on remission-induction and remission-maintenance. Methotrexate is a widely used immunosuppressant but only a few studies explored its role for maintenance in AAV. This trial investigated the efficacy and safety of methotrexate as maintenance therapy for AAV. METHODS: In this single-centre, open-label, randomised trial we compared methotrexate and cyclophosphamide for maintenance in AAV...
2017: PloS One
https://www.readbyqxmd.com/read/28988280/echocardiographic-features-in-patients-with-anca-associated-vasculitis-within-3%C3%A2-months-before-and-after-diagnosis
#4
Sung Soo Ahn, Eun Seong Park, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
We investigated the all-inclusive echocardiographic features in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) within 3 months before and after diagnosis. We reviewed the medical records of 89 AAV patients taking echocardiography and 35 age- and gender-matched controls. We collected clinical and laboratory data and echocardiographic results. We compared the variables between patients with AAV of each variant and controls and among those with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA)...
October 8, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28983650/-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome
#5
REVIEW
A Jerrentrup, T Müller, J Mariss, S Dylla
This article presents the case of a patient with dyspnea, a history of poorly controlled asthma, sinonasal polyposis, blood eosinophilia and transient pulmonary infiltrates. The autoantibodies antinuclear antibodies, cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were negative. These symptoms are typical for eosinophilic granulomatosis with polyangiitis (EGPA, alternatively known as Churg-Strauss syndrome). The delay between onset and diagnosis in this case was several years...
October 5, 2017: Der Internist
https://www.readbyqxmd.com/read/28960271/alterations-in-circulating-lymphoid-cell-populations-in-systemic-small-vessel-vasculitis-are-non-specific-manifestations-of-renal-injury
#6
Barbara Fazekas, Ana Moreno-Olivera, Yvelynne Kelly, Paul O'Hara, Susan Murray, Alan Kennedy, Niall Conlon, Jennifer Scott, Ashanty M Melo, Fionnuala B Hickey, Dearbhaile Dooley, Eoin C O'Brien, Sarah Moran, Derek G Doherty, Mark A Little
Innate lymphocyte populations, such as innate lymphoid cells (ILCs), γδ T cells, invariant natural killer T (iNKT) cells and mucosal associated invariant T (MAIT) cells are emerging as important effectors of innate immunity and are involved in various inflammatory and autoimmune diseases. The aim of this study was to assess the frequencies and absolute numbers of innate lymphocytes as well as conventional lymphocytes and monocytes in peripheral blood from a cohort of ANCA associated vasculitis (AAV) patients...
September 28, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28955487/validation-of-the-eular-era-edta-recommendations-for-the-management-of-anca-associated-vasculitis-by-disease-content-experts
#7
Max Yates, Richard Watts, Ingeborg Bajema, Maria Cid, Bruno Crestani, Thomas Hauser, Bernhard Hellmich, Julia Holle, Martin Laudien, Mark A Little, Raashid Ahmed Luqmani, Alfred Mahr, Peter Merkel, John Mills, Janice Mooney, Mårten Segelmark, Vladimir Tesar, Kerstin W A Westman, Augusto Vaglio, Nilüfer Yalçındağ, David R Jayne, Chetan Mukhtyar
The European League Against Rheumatism recommendations for the management of antineutrophil cytoplasmic antibody-associated vasculitis have been recently published. Unique to recommendation development, they were also voted on by members of a learned society. This paper explores the wider validity of the recommendations among people who self-identify as clinicians caring for patients with vasculitis. In addition to the task force, a learned society (European Vasculitis Society-EUVAS) was invited, through online survey, to rate independently the strength of evidence of each recommendation to obtain an indication of the agreement among the final target audience and ultimate end-users of the recommendations...
2017: RMD Open
https://www.readbyqxmd.com/read/28950436/eosinophilic-granulomatosis-with-polyangiitis-toward-personalized-treatment
#8
Xavier Puéchal, Christian Pagnoux, Loïc Guillevin
Dr. Smitienko and colleagues commented on our trial on the interest of azathioprine (AZA) adjunction to glucocorticoids to induce remission of systemic necrotizing vasculitides (SNVs) without poor-prognosis factors, more specifically on eosinophilic granulomatosis with polyangiitis (EGPA). We fully agree that EGPA is a heterogenous disease. Indeed, we (1) and others (2) demonstrated that antineutrophil cytoplasm antibody (ANCA)-positive and ANCA-negative EGPA differ phenotypically. Among the CHUSPAN2 trial's 95 patients, 51 had new-onset EGPA, 19 (37%) of which were anti-myeloperoxidase-positive...
September 26, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28943503/basophil-and-m2-macrophage-infiltration-in-lesional-skin-of-eosinophilic-granulomatosis-with-polyangiitis
#9
Chisa Nakashima, Atsushi Otsuka, Naomi Kitayama, Tetsuya Honda, Kenji Kabashima
No abstract text is available yet for this article.
September 23, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28941467/an-atypical-presentation-of-cardiac-tamponade-and-periorbital-swelling-in-a-patient-with-eosinophilic-granulomatosis-with-polyangiitis-a-case-report
#10
Alexandra C Keefe, Joseph C Hymas, Lyska L Emerson, John J Ryan
BACKGROUND: Eosinophilic granulomatosis with polyangiitis is a rare, necrotizing systemic vasculitis associated with asthma and hypereosinophilia. Its cause and pathophysiology are still being elucidated. CASE PRESENTATION: We report a case of eosinophilic granulomatosis with polyangiitis in a 50-year-old Caucasian woman who presented with chest pain, dyspnea at rest, fever, and periorbital swelling. She was found to have significant hypereosinophilia and cardiac tamponade physiology...
September 24, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28924115/neurological-complications-in-eosinophilic-granulomatosis-with-polyangiitis-egpa-the-roles-of-history-and-the-results-of-physical-examinations-in-the-diagnosis-of-egpa
#11
Hiroshi Oiwa, Sho Mokuda, Tomoyasu Matsubara, Masamoto Funaki, Ikuko Takeda, Takemori Yamawaki, Kazuhiko Kumagai, Eiji Sugiyama
Objective To investigate the clinical symptoms, the physical and neurological findings, and the clinical course of neurological complications in eosinophilic granulomatosis with polyangiitis (EGPA). Methods A retrospective chart review of EGPA cases managed by two referral hospitals was performed, with a focus on the neurological findings. The study analyzed the symptoms at the onset of EGPA and investigated their chronological relationship. The patient delay (the delay between the onset of symptoms and the initial consultation), and the physician delay (the delay from consultation to the initiation of therapy) were determined and compared...
September 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28916432/rituximab-as-induction-therapy-in-eosinophilic-granulomatosis-with-polyangiitis-refractory-to-conventional-immunosuppressive-treatment-a-36-month-follow-up-analysis
#12
Jens Thiel, Arianna Troilo, Ulrich Salzer, Theresa Schleyer, Kirsten Halmschlag, Marta Rizzi, Natalie Frede, Ana Venhoff, Reinhard E Voll, Nils Venhoff
BACKGROUND: Rituximab (RTX) is approved for induction therapy of granulomatosis with polyangiitis and microscopic polyangiitis. In eosinophilic granulomatosis with polyangiitis (EGPA), organ-threatening manifestations are mainly treated with cyclophosphamide (CYC). RTX as treatment in EGPA has been described in small case series; however long-term data and the efficacy of RTX in EGPA refractory to CYC have not been reported yet. OBJECTIVES: To investigate the efficacy and safety of RTX and conventional immunosuppressive therapy with CYC in EGPA as induction therapy and during long-term follow-up...
September 12, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28895041/unmet-needs-in-the-pathogenesis-and-treatment-of-vasculitides
#13
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/28881714/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-years-observation-study
#14
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/28881446/the-epidemiology-of-anca-associated-vasculitis-in-olmsted-county-minnesota-usa-a-20-year-population-based-study
#15
Alvise Berti, Divi Cornec, Cynthia S Crowson, Ulrich Specks, Eric L Matteson
OBJECTIVE: To estimate the incidence, prevalence and mortality of ANCA-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA), in a U.S. based adult population. METHODS: All medical records of patients with a diagnosis or suspicion of AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed. Incidence rates were age- and sex-adjusted to the 2010 US white population...
September 7, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28842398/anca-glomerulonephritis-and-vasculitis
#16
J Charles Jennette, Patrick H Nachman
ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). A diagnosis of ANCA vasculitis should always specify the serotype as MPO-ANCA positive, PR3-ANCA positive, or ANCA-negative. To fully characterize a patient, the serotype also should be accompanied by the clinicopathologic variant if this can be determined: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener), eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or renal-limited vasculitis...
October 6, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28816552/the-role-of-biopsy-in-lacrimal-gland-inflammation-a-clinicopathologic-study
#17
Panitee Luemsamran, Jack Rootman, Valerie A White, Nariman Nassiri, Manraj K S Heran
PURPOSE: To determine the causes of lacrimal gland inflammation based on histopathology and systemic evaluation. METHODS: This is a retrospective case series study. From the University of British Columbia Orbit Clinic between January 1976 and December 2008, we reviewed the medical records of 60 patients who presented with inflammatory features of the lacrimal gland (i.e., erythema, edema, or tenderness) in which the diagnoses were not possible clinically and on imaging alone...
August 17, 2017: Orbit
https://www.readbyqxmd.com/read/28794143/differential-diagnosis-of-granulomatous-lung-disease-clues-and-pitfalls-number-4-in-the-series-pathology-for-the-clinician-edited-by-peter-dorfm%C3%A3-ller-and-alberto-cavazza
#18
Shinichiro Ohshimo, Josune Guzman, Ulrich Costabel, Francesco Bonella
Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis)...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
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...
October 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28769112/vasculitis-mepolizumab-for-eosinophilic-granulomatosis-with-polyangiitis
#20
Loïc Guillevin
No abstract text is available yet for this article.
September 2017: Nature Reviews. Rheumatology
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