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Wegener's granulomatosis

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https://www.readbyqxmd.com/read/28220286/characteristics-of-hypertrophic-pachymeningitis-in-patients-with-granulomatosis-with-polyangiitis
#1
Hyun Ah Choi, Mi Ji Lee, Chin-Sang Chung
Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients...
February 20, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28210182/successful-cataract-surgery-in-a-patient-with-refractory-wegener-s-granulomatosis-effectively-treated-with-rituximab-a-case-report
#2
Abdullah M Alfawaz
: Wegener's granulomatosis is a granulomatous disorder associated with systemic necrotizing vasculitis. Eye involvement occurs in approximately 50% of Wegener's granulomatosis patients and is an important cause of morbidity. Conventional treatment-related morbidity and failure have led to studies of alternative treatment modalities. In this case of a 35-year-old man with severe Wegener's granulomatosis, conventional therapy failed to induce remission. Despite the standard immunosuppressive therapy, progression of the disease was observed, mainly with ocular manifestations and renal impairment...
July 2016: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
https://www.readbyqxmd.com/read/28207017/healing-of-leg-ulcers-associated-with-granulomatosis-with-polyangiitis-wegener-granulomatosis-after-rituximab-therapy
#3
Scott Kindle, Joseph Fanciullo
No abstract text is available yet for this article.
January 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28185705/cardiovascular-magnetic-resonance-imaging-pattern-at-the-time-of-diagnosis-of-treatment-na%C3%A3-ve-patients-with-connective-tissue-diseases
#4
Sophie Mavrogeni, George Markousis-Mavrogenis, Loukia Koutsogeorgopoulou, Theodoros Dimitroulas, Konstantinos Bratis, George D Kitas, Petros Sfikakis, Maria Tektonidou, Georgia Karabela, Efthymios Stavropoulos, Gikas Katsifis, Kyriaki A Boki, Anastasia Kitsiou, Vasiliki Filaditaki, Elias Gialafos, Sotiris Plastiras, Vasiliki Vartela, Genovefa Kolovou
BACKGROUND-AIM: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis. PATIENTS-METHODS: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]...
February 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28163812/acute-urinary-retention-secondary-to-urethral-involvement-of-granulomatosis-with-polyangiitis
#5
Patrick T Anderson, Stephanie Gottheil, Manal Gabril, Lillian Barra, Nicholas Power
Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a systemic necrotizing vasculitis of small- and medium-sized blood vessels, primarily affecting the upper and lower respiratory tracts, as well as the kidneys. Urogenital manifestations of GPA are exceedingly rare and usually respond well to systemic immunosuppressive therapy. Here, we present a case of a 36-year-old female presenting with acute urinary obstruction secondary to urethral GPA involvement in the immediate postpartum period...
January 2017: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/28162219/surgical-treatment-of-nasal-septal-perforations-sir-italian-society-of-rhinology-experts-opinion
#6
Desiderio Passali, Maria Carla Spinosi, Lorenzo Salerni, Michele Cassano, Hugo Rodriguez, Francesco Maria Passali, Luisa Maria Bellussi
BACKGROUND AND AIM: The aim of our study has been to investigate the perception of aspects related to nasal perforation among experts in Rhinology and ENT surgeons. Our aim was reporting the situations in different Countries to improve the knowledge of colleagues interested in this topic. METHODS: A panel of experts prepared a 20-question questionnaire regarding nasal perforations and their surgical repair, that were emailed to all the members of SIR (Società Italiana di Rnologia - Italian Society of Rhinology)...
February 2, 2017: Acta Otorrinolaringológica Española
https://www.readbyqxmd.com/read/28148583/anca-associated-vasculitis
#7
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/28129481/fatal-infection-of-pneumocystis-jiroveci-pneumonia-in-a-pemphigus-patient-treated-with-rituximab
#8
LETTER
K C Wei, Y H Wang, W H Wang, W Chen
No abstract text is available yet for this article.
January 27, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28121720/the-use-of-integra%C3%A2-dermal-regeneration-template-for-the-orbital-exenteration-socket-a-novel-technique
#9
Cem Ozgonul, Ana Beatriz Diniz Grisolia, Hakan Demirci
PURPOSE: Integra® dermal regeneration template is a bilayer membrane system that acts as a scaffold for regenerating dermal skin cells. It is used for wound reconstruction following burns, extensive injuries, and a large tumor excision in multiple parts of the body. The dermal layer is made of porous matrix of bovine tendon collagen and glycosaminoglycan. The epidermal layer is made of polysiloxane layer. In this study, the authors evaluated the use of Integra® dermal regeneration template for the immediate reconstruction of the orbital exenteration socket...
January 23, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28119348/a-pragmatic-approach-to-vasculitis-in-the-gastrointestinal-tract
#10
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/28097250/spinal-cord-compression-from-wegener-s-granulomatosis-an-unusual-presentation
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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