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Wegeners granulomatosis

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https://www.readbyqxmd.com/read/28097250/spinal-cord-compression-from-wegener-s-granulomatosis-an-unusual-presentation
#1
Deb Roy, Prashanth J Rao, Kevin Phan, Ralph J Mobbs, Michael Selby
Wegener's granulomatosis (WG) causing spinal cord compression is very rare with only few cases reported in literature. We present a case report with review of literature. A 55-year-old lady with known WG presented with acute on chronic spinal cord compression. MRI scan revealed spinal cord compression anteriorly and posteriorly at T2-T5 level. Patient underwent urgent surgical decompression with excision of the posterior dural lesion with synthetic duraplasty. Patient made good neurological recovery. Histopathology revealed features consistent with WG...
December 2016: Journal of Spine Surgery (Hong Kong)
https://www.readbyqxmd.com/read/28096986/extracorporeal-membrane-oxygenation-rescue-in-adolescent-with-bronchiolitis-obliterans-organizing-pneumonia-like-wegener-s-granulomatosis
#2
Lars Falk, Lars Mikael Broman
We report a 17-year-old woman with bronchiolitis obliterans-organizing pneumonia (BOOP)-like granulomatosis with polyangiitis developing severe airway obliterations. Pending age, phase and grade of autoimmune treatment, and offering ECMO treatment may be crucial for survival but occasionally preface futility. ECMO-treated patient with BOOP-like GPA has never been described before.
January 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28067125/value-of-non-identified-anca-non-pr3-non-mpo-in-the-diagnosis-of-granulomatosis-with-polyangiitis-wegener-s-granulomatosis
#3
Mélanie Givaudan, Frédéric Vandergheynst, Patrick Stordeur, Annick Ocmant, Christian Melot, Valérie Gangji, Muhammad S Soyfoo
OBJECTIVE: Determine the frequency of granulomatosis with polyangiitis (GPA) associated with non-identified ANCA (non-MPO, non-PR3 ANCA) and secondarily compare their clinic with GPA associated with MPO-positive or PR3-positive ANCA. METHODS: In a monocentric retrospective observational study, clinical data of 398 patients with non-identified ANCA (titer of ANCA at least 1/80 by immunofluorescence on ethanol fixed PMN) was gathered over a period of 6 years. GPA patients from this population were compared with GPA patients with identified ANCA on the basis of clinical, biological, immunological and histological features...
January 9, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28062909/paediatric-anti-neutrophil-cytoplasmic-antibody-anca-associated-vasculitis-an-update-on-renal-management
#4
REVIEW
Lucy A Plumb, Louise Oni, Stephen D Marks, Kjell Tullus
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of disorders characterized by necrotizing inflammation of the small to medium vessels in association with autoantibodies against the cytoplasmic region of the neutrophil. Included in this definition are granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). AAV are chronic, often relapsing diseases that can be organ or life threatening...
January 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28025926/clinical-and-economic-burden-of-elevated-blood-eosinophils-in-patients-with-and-without-uncontrolled-asthma
#5
Julian Casciano, Jerry Krishnan, Zenobia Dotiwala, Chenghui Li, Shawn X Sun
BACKGROUND: The European Respiratory Society and American Thoracic Society (ERS/ATS) published guidelines in 2014 for the evaluation and treatment of asthma. These guidelines draw attention to management of patients with asthma that remains uncontrolled despite therapy. One phenotypic characteristic of therapy-resistant asthma is eosinophil elevation. It is important to better understand the burden of care gaps in this patient subgroup in order to support improved treatment strategies in the future...
January 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28012056/pachymeningitis-in-granulomatosis-with-polyangiitis-case-series-with-earlier-onset-in-younger-patients-and-literature-review
#6
Violeta Higuera-Ortiz, Abraham Reynoso, Natllely Ruiz, Rosa Delia Delgado-Hernández, Gilberto Gómez-Garza, Luis Felipe Flores-Suárez
The objective of this study is to describe the characteristics of patients with pachymeningitis (PM) in granulomatosis with polyangiitis (GPA) from Latin America, including three young patients. This is a retrospective case series. Patients were classified according to the ACR criteria, the 2012 Chapel Hill Consensus Conference Nomenclature and the EMA algorithm. Demographic, clinical, serological, and neuroimaging characteristics are described. Thirteen patients (nine females, four males) were identified. Mean age ± SD of PM diagnosis was 35...
December 23, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27982217/bilateral-acute-angle-closure-glaucoma-as-a-first-presentation-of-granulomatosis-with-polyangiitis-wegener-s
#7
Alper Mete, Sabit Kimyon, Oguzhan Saygili, Can Pamukcu, Kıvanç Güngör
We report a case of bilateral acute angle-closure glaucoma in a patient with undiagnosed granulomatosis with polyangiitis (Wegener's). A 59-year-old man presented with a severe headache, ocular pain, blurred vision, shortness of breath, and mild fever. Clinical examination revealed conjunctival chemosis, corneal edema, and shallow anterior chambers. Closed angles were observed bilaterally on gonioscopy. The patient was treated with intravenous mannitol, oral acetazolamide, and anti-glaucomatous eye drops. Over the following two days, his vision improved and intraocular pressures decreased...
September 2016: Arquivos Brasileiros de Oftalmologia
https://www.readbyqxmd.com/read/27978568/reconstructive-techniques-for-the-saddle-nose-deformity-in-granulomatosis-with-polyangiitis-a-systematic-review
#8
Waleed H Ezzat, Rebecca A Compton, Krystyne C Basa, Jessica Levi
Importance: Repairing the saddle nose deformity in the setting of granulomatosis with polyangiitis disease is a rare but challenging situation for any surgeon. Given that the available data in the literature is based on case reports and small case series, there is little evidence available to help delineate which reconstructive techniques are optimal. Objective: To examine which techniques were most successful in reconstructive rhinoplasty for a saddle nose deformity secondary to granulomatosis with polyangiitis...
December 15, 2016: JAMA Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/27943273/granulomatosis-with-polyangiitis-associated-hypertrophic-pachymeningitis-mimicking-spontaneous-intracranial-hypotension-a-case-report
#9
Young Hee Jung, Mi Ji Lee, Chungbin Lee, Jihoon Cha, Chin-Sang Chung
BACKGROUND: Dural enhancement is a characteristic finding in both spontaneous intracranial hypotension and hypertrophic pachymeningitis. Positional headache is the most important feature that distinguishes the two diseases. We report a patient with granulomatosis with polyangiitis (formerly Wegener's granulomatosis) who initially manifested like spontaneous intracranial hypotension. CASE PRESENTATION: We report here the case of a 63-year old man who presented with severe positional headache...
December 10, 2016: Headache
https://www.readbyqxmd.com/read/27888159/transmembrane-tnf-alpha-reverse-signaling-leading-to-tgf-beta-production-is-selectively-activated-by-tnf-targeting-molecules-therapeutic-implications
#10
REVIEW
Zsuzsa Szondy, Anna Pallai
Tumor necrosis factor (TNF)-α is a potent pro-inflammatory cytokine exerting pleiotropic effects on various cell types. It is synthesized in a precursor form called transmembrane TNF-α (mTNF-α) which, after being processed by metalloproteinases, is released in a soluble form to mediate its biological activities through Type 1 and 2 TNF receptors in TNF receptor expressing cells. In addition to acting in soluble form, TNF-α also acts in the transmembrane form both as a ligand by activating TNF receptors, as well as a receptor that transmits outside-to-inside (reverse) signals back into mTNF-α bearing cells...
January 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/27804171/development-and-validation-of-case-finding-algorithms-for-the-identification-of-patients-with-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-in-large-healthcare-administrative-databases
#11
Antoine G Sreih, Narender Annapureddy, Jason Springer, George Casey, Kevin Byram, Andy Cruz, Maya Estephan, Vince Frangiosa, Michael D George, Mei Liu, Adam Parker, Sapna Sangani, Rebecca Sharim, Peter A Merkel
PURPOSE: The aim of this study was to develop and validate case-finding algorithms for granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (Churg-Strauss, EGPA). METHODS: Two hundred fifty patients per disease were randomly selected from two large healthcare systems using the International Classification of Diseases version 9 (ICD9) codes for GPA/EGPA (446.4) and MPA (446.0). Sixteen case-finding algorithms were constructed using a combination of ICD9 code, encounter type (inpatient or outpatient), physician specialty, use of immunosuppressive medications, and the anti-neutrophil cytoplasmic antibody type...
December 2016: Pharmacoepidemiology and Drug Safety
https://www.readbyqxmd.com/read/27790680/image-gallery-symmetrical-necrosis-of-the-breast-as-an-inaugural-manifestation-of-granulomatosis-with-polyangiitis-wegener-granulomatosis
#12
I Gallais Sérézal, K Jennische
No abstract text is available yet for this article.
November 2016: British Journal of Dermatology
https://www.readbyqxmd.com/read/27789156/nasal-nk-t-cell-lymphoma-a-tragic-case
#13
L Taali, M Abou-Elfadl, M Fassih, M Mahtar
INTRODUCTION: Nasal NK/T-cell lymphoma is a rare clinicopathological entity, formerly called midline lethal granuloma. Following progress in histology and the routine use of immunohistochemistry, nasal NK/T-cell lymphoma was recognized as a distinct entity by WHO in 2001. CASE REPORT: The authors report the case of a 22-year-old, insulin-dependent diabetic woman, who presented with mid-facial inflammatory swelling following facial trauma, initially diagnosed and treated as cellulitis of the face...
October 24, 2016: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/27766271/complete-resolution-of-wegener-s-granulomatosis-lung-granuloma-after-aortic-root-replacement
#14
Sarah-Jayne Edmondson, Saina Attaran, Ulrich P Rosendahl
Wegner's granulomatosis (WG) is an autoimmune systemic vasculitis that results in necrotizing granulomas. We report a WG patient with a lung granuloma and aortic root dilatation, who underwent aortic root replacement on cardiopulmonary bypass (CPB). Intraoperatively, the patient suffered an aortic dissection, which was repaired immediately under deep hypothermic circulatory arrest (DHCA). Follow-up imaging showed complete granuloma resolution, despite absence of immunosuppressive therapy. Immune stimulation following CPB is well described; here, the opposite was observed and DHCA effects are discussed...
February 2016: Aorta (Stamford, Conn.)
https://www.readbyqxmd.com/read/27754826/clinical-significance-of-routine-lacrimal-sac-biopsy-during-dacryocystorhinostomy-a-comprehensive-review-of-literature
#15
Zoran Koturović, Miroslav Knežević, Dejan M Rašić
The main purpose of this paper is to provide the information about the incidence and types of pathology of secondary acquired obstructions of the lacrimal excretory outflow system caused by primary lacrimal sac non-neoplastic and neoplastic lesions. After a thorough literature search, 17 case-control studies were found and selected, data were extracted and categorized, to evaluate specific lacrimal sac pathology mimicking inflammation. A total of 3865 histopathologically examined lacrimal sac wall biopsy specimens from 3662 patients, taken during dacryocystorhinostomy for clinically presumed primary chronic dacryocystitis, were analyzed...
October 18, 2016: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/27733943/updates-in-anca-associated-vasculitis
#16
REVIEW
Christian Pagnoux
Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited ANCA-associated vasculitides can be considered the fourth entity. Despite their rarity and still unknown cause(s), research pertaining to ANCA-associated vasculitides has been very active over the past decades. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) has been supported using several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) has not been as strongly demonstrated...
September 2016: European Journal of Rheumatology
https://www.readbyqxmd.com/read/27717952/reversible-complete-atrioventricular-block-in-patient-with-wegener-s-granulomatosis-a-report-on-fortunate-outcome-with-long-term-follow-up
#17
Roman Steckiewicz, Marek Rosiak, Przemysław Stolarz, Elżbieta B Świętoń, Marcin Grabowski, Dariusz A Kosior
Reversible complete atrioventricular block in patient with Wegener's granulomatosis - a report on a positive outcome with long term follow-up. Atrioventricular (AV) block is a rare complication of Wegener's granulomatosis (WG), thus there are no standards of management in such cases. We present a case of a patient with a dual-chamber pacemaker (DDD) implanted due to complete AV block in the course of Wegener's granulomatosis (WG). An immunosuppressive therapy resulted in the resolution of non-cardiac and AV conduction disorders...
2016: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/27698975/upper-gastrointestinal-bleeding-as-the-first-manifestation-of-wegener-s-granulomatosis
#18
Hamid Tavakkoli, Mehdi Zobeiri, Mansour Salesi, Mohammad Hossein Sanei
Wegener's granulomatosis is an uncommon inflammatory disease that manifests as vasculitis, granulomatosis, and necrosis. It usually involves the upper and lower respiratory tracts and kidneys. Although it may essentially involve any organ, gastrointestinal (GI) involvement is notably uncommon. A 20-year-old male patient presented with epigastric pain, vomiting, hematemesis, and melena. On physical examination, he was pale. There was no abdominal tenderness or organomegaly. Upper GI endoscopy revealed dark blue-colored infiltrative lesions in prepyloric area...
July 2016: Middle East Journal of Digestive Diseases
https://www.readbyqxmd.com/read/27696762/disease-activity-glucocorticoid-exposure-and-rituximab-determine-body-composition-changes-during-induction-treatment-of-anca-associated-vasculitis
#19
Zachary S Wallace, Eli M Miloslavsky, Matthew Cascino, Sebastian H Unizony, Na Lu, Gary S Hoffman, Cees G M Kallenberg, Carol A Langford, Peter A Merkel, Paul A Monach, Philip Seo, Robert Spiera, E William St Clair, Ulrich Specks, Paul Brunetta, Hyon K Choi, John H Stone
INTRODUCTION: We investigated the relationships between glucocorticoid (GC) use, disease activity, and changes in body mass index (BMI) in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: We analyzed AAV patients enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial. GC use, BMI, and disease activity were measured regularly during the trial period. We performed mixed effects regressions to examine the associations of time-dependent cumulative average GC use and disease activity with changes in body mass index (BMI) over time, while adjusting for potential confounders...
October 1, 2016: Arthritis Care & Research
https://www.readbyqxmd.com/read/27672853/poster-85-a-unique-diagnosis-of-critical-illness-neuromyopathy-in-a-patient-with-wegener-s-granulomatosis-a%C3%A2-case-report
#20
Shweta S Duggal, Chirag Shah
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
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