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Wegeners granulomatosis

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https://www.readbyqxmd.com/read/28524146/-combination-therapy-of-refractory-localized-granulomatosis-with-polyangiitis-and-orbital-involvement
#1
Y O Grusha, D S Ismailova, P I Novikov, E A Kogan, Yu V Abramova, N M Bulanov, L V Sherstneva
Granulomatous orbital cellulitis is one of the most common ocular manifestations of granulomatosis with polyangiitis (Wegener's). The process is often refractory to conventional immunosuppressive therapy and requires a more radical treatment approach. However, surgical experience with this type of patients is limited. There have been just a few reported cases of orbital decompression in such patients and many authors have doubted the appropriateness of the procedure, since it is associated with a high risk of potentially fatal complications...
2017: Vestnik Oftalmologii
https://www.readbyqxmd.com/read/28495524/granulomatosis-with-polyangiitis-wegener-granulomatosis-a-proteinase-3-driven-disease
#2
Véronique Witko-Sarsat, Nathalie Thieblemont
Granulomatosis with polyangiitis (GPA, Wegener granulomatosis) is a systemic autoimmune vasculitis that affects small arteries, arterioles, and capillaries, most notably in the kidneys and lungs. In this disease, proteinase 3 (PR3), produced by neutrophils, is targeted by antineutrophil cytoplasmic antibodies (ANCA). Recent work by our group has shown how PR3 impairs the resolution of inflammation and deregulates the immune system. Normally, the clearance of activated neutrophils triggers an anti-inflammatory, pro-resolution process...
May 8, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28476172/anca-associated-vasculitis-in-childhood-recent-advances
#3
REVIEW
Marta Calatroni, Elena Oliva, Davide Gianfreda, Gina Gregorini, Marco Allinovi, Giuseppe A Ramirez, Enrica P Bozzolo, Sara Monti, Claudia Bracaglia, Giulia Marucci, Monica Bodria, Renato A Sinico, Federico Pieruzzi, Gabriella Moroni, Serena Pastore, Giacomo Emmi, Pasquale Esposito, Mariagrazia Catanoso, Giancarlo Barbano, Alice Bonanni, Augusto Vaglio
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in childhood but North-American and European cohort studies performed during the last decade have clarified their phenotype, patterns of renal involvement and their prognostic implications, and outcome...
May 5, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28455828/prevalence-of-hearing-impairment-in-patients-with-rheumatoid-arthritis-granulomatosis-with-polyangiitis-gpa-wegener-s-granulomatosis-or-systemic-lupus-erythematosus
#4
Torsten Rahne, Franziska Clauß, Stefan K Plontke, Gernot Keyßer
Hearing loss in patients with autoimmune diseases, such as systemic lupus erythematosus (SLE), granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), or rheumatoid arthritis (RA), is controversial. Many studies lack measurements of bone-conduction thresholds to sufficiently differentiate between sensorineural hearing loss and conductive hearing loss. In addition, many studies lack control groups or comparisons to an age-related normal hearing threshold. This study investigates hearing performance with an extended audiological battery using psychoacoustic and objective measures...
April 28, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28440995/-the-first-clinical-description-of-granulomatosis-with-polyangiitis-known-before-as-wegener-s-granulomatosis
#5
Ulises Mercado
Before 1950, cases of necrotizing vasculitis were commonly published in journals of pathology. Most of these cases were designated as polyarteritis nodosa. In 1952, the pathologist Pearl Zeek critically reviewed and summarized the literature dealing with polyarteritis nodosa and first grouped the different types of necrotizing vasculitis. But she omitted some types of not well-characterized vasculitis, among them granulomatosis with polyangiitis (Wegener's granulomatosis).
May 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28393729/granulomatous-polyangitis-wegener-granulomatosis-clinical-findings-and-results-of-long-term-follow-up
#6
Nazan Şen, Müge Aydın Tufan, Reyhan Yıldız, Emine Duygu Ersözlü Bozkırlı, Eftal Yücel
Introduction: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital. Patients and Methods: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014. Result: The mean time from the onset of symptoms to diagnosis was 7.8 ± 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72...
September 2016: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/28393058/extranodal-nk-t-cell-lymphoma-with-destruction-of-the-uvulae-a-case-report
#7
Farahzad Jabbari Azad, Zahra Delavarian, Masoud Hatami, Hosein Rahimi, Mohammad Reza Abdolvahed
INTRODUCTION: Extranodal Natural Killer (NK)/T-cell lymphoma (NKTCL) nasal type is a rare but well-known disease with poor prognosis. NKTCL is more prevalent in Asia and comprises about 7-10% of all non-Hodgkin lymphoma cases in this region. The characteristic clinical pattern of NKTCL is the destruction of the midline structures of the mid-face. CASE REPORT: The present study examines a case of NKTCL in a 23-year-old man with a destructive ulcer of the palate and uvulae...
March 2017: Iranian Journal of Otorhinolaryngology
https://www.readbyqxmd.com/read/28391344/is-there-a-role-for-tnf%C3%AE-blockade-in-anca-associated-vasculitis-and-glomerulonephritis
#8
Stephen P McAdoo, Charles D Pusey
Tumour necrosis factor alpha (TNFα) is a cytokine that is pivotal in the inflammatory response. Blockade of TNFα has been shown to be effective in a number of human autoimmune diseases, including rheumatoid arthritis, raising the question of whether this approach may be effective in inflammatory kidney disease, such as ANCA-associated vasculitis (AAV). In AAV, there is considerable evidence for the role of TNFα in the pathophysiology of disease, including increased expression of TNFα mRNA in leucocytes and in renal tissue...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28387944/th17-and-treg-lymphocytes-as-cellular-biomarkers-of-disease-activity-in-granulomatosis-with-polyangiitis
#9
Lorenzo Cosmi
Granulomatosis with Polyangiitis (GPA) (formerly known as Wegener's granulomatosis) is a vasculitis of unknown etiology affecting predominantly small- to medium-sized vessels, usually involving the upper and lower respiratory tract and kidneys. Anti-neutrophil cytoplasmic autoantibodies are probably the initial cause of the inflammatory process that leads to the typical necrotizing lesions. In this issue of the European Journal of Immunology, Szczeklik et al. [Eur. J. Immunol. 2017. 47: 724-733] report some interesting findings on the possible involvement of T-cell subsets in the pathogenesis of the disease...
April 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28366156/-granulomatous-polyangitis-wegener-granulomatosis-clinical-findings-and-results-of-long-term-follow-up
#10
Nazan Şen, Müge Aydın Tufan, Reyhan Yıldız, Emine Duygu Ersözlü Bozkırlı, Eftal Yücel
Introduction: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital. Patients and Methods: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014. Result: The mean time from the onset of symptoms to diagnosis was 7.8 ± 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72...
September 2016: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/28345423/systemic-lupus-erythematosus-and-hearing-disorders-literature-review-and-meta-analysis-of-clinical-and-temporal-bone-findings
#11
Arianna Di Stadio, Massimo Ralli
Objective This literature review and meta-analysis was performed to evaluate the correlations among hearing and vestibular clinical symptoms, temporal bone findings, and pathological mechanisms in patients with systemic lupus erythematosus (SLE). Study design Relevant papers in the literature were retrospectively reviewed. Clinical hearing aspects in patients with SLE and relevant temporal bone studies in the same field were analyzed. Methods PubMed and Google Scholar searches were performed using the following keywords: "auto-immune disease," "systemic lupus erythematosus (SLE)," "hearing loss," "temporal bone study," "vertigo," "dizziness," "tinnitus," "ear symptoms," "treatment," "diagnosis," "symptoms," "etiopathogenesis," "Wegener granulomatosis," "Sjogren," "polyarteritis nodosa," "Cogan syndrome," and "granulomatosis...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28318198/-subglottic-stenosis-as-the-initial-manifestation-of-wegener-s-granulomatosis-in-a-teenager-case-report
#12
Giselle Cuestas, Verónica Rodríguez, Flavia Doormann, Alejandra Pringe, Patricio Bellia Munzón, Gastón Bellia Munzón, Carlos Ortega, Rubén Álvarez
Wegener's granulomatosis is a necrotizing granulomatous vasculitis of autoimmune origin that primarily affects the upper and lower airways and kidneys. It is very rare in children and adolescents. When started at a young age it is often associated with subglottic stenosis. Subglottic stenosis is a potentially fatal manifestation of Wegener's granulomatosis. Its diagnosis requires a high index of suspicion since it might develop in the absence of other signs of activity. Occasionally, subglottic stenosis may present as the initial manifestation of the disease...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28306365/ulcerative-granuloma-of-the-eyelid-as-the-initial-manifestation-of-granulomatosis-with-polyangiitis-wegener-s-granulomatosis-a-case-report
#13
Helena Brosa Morros, Olaia Subirà, Montse Gomà Gàllego, Maria José Paúles Villar, Ferran Mascaró Zamora, Maravillas Abia Serrano
A 56-year-old-man presented a 2-month history of chalazion in the eyelids without response to treatment and with an inconclusive biopsy. Laboratory results confirmed the presence of Enterobacter cloacae and Streptococcus gordonii infection. Despite appropriate intravenous antibiotic treatment, clinical worsening was observed. Radical surgical excision and total tarsorrhaphy were performed. Following treatment, the patient was asymptomatic for 6 weeks until he developed acute renal failure, generalised arthralgia, acute hypertensive anterior uveitis, and dacryoadenitis...
March 17, 2017: Orbit
https://www.readbyqxmd.com/read/28303064/accessory-left-gastric-artery-aneurysms-in-granulomatosis-with-polyangiitis-a-case-report-and-literature-review
#14
Toshihide Tomosugi, Takuji Takahashi, Yoshihisa Kawase, Koichi Yoshida, Shogo Hayashi, Takefumi Sugiyama, Mitsuya Shimizu, Michita Shoka, Kohichi Sawaki, Eiji Onishi, Naomi Hayashi, Hidenobu Matsushita, Osamu Okochi
Aneurysm formation is a potential complication of granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis. It is a very rare complication, but immediate diagnosis and therapy should be performed because an aneurysm can be life-threatening if it ruptures. An accessory left gastric artery (ALGA) is also a rare variant gastric artery that may obtain its blood supply from the left hepatic artery and left gastric artery. We herein describe a 57-year-old Japanese man who was diagnosed with GPA complicated by aneurysm rupture in an ALGA...
February 2017: Nagoya Journal of Medical Science
https://www.readbyqxmd.com/read/28295009/-refractory-form-of-orbital-granulomatosis-with-polyangiitis-a-clinical-and-morphological-study
#15
E A Kogan, Ya O Grusha, D S Ismailova, P I Novikov, Yu V Abramova, A D Meshkov, E F Rizopulu
Orbital granulomatosis with polyangiitis (Wegener's granulomatosis, GPA), which is characterized by granulomatous inflammation with small-vessel vasculitis, can develop in local and generalized forms of the disease. The introduction of current immunosuppressive therapy regimens has improved the prognosis of the disease; however, there are immunosuppressive treatment-refractory forms of GPA, the morphology of which has been inadequately investigated. The paper describes a clinical case of refractory GPA involving the orbit, as evidenced by histological and immunohistochemical examinations...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28273603/rare-presentation-of-wegener-s-granulomatosis-in-the-pituitary-gland-case-report-and-literature-review
#16
Samantha M Baird, Upasna Pratap, Catriona McLean, Candice P Law, Nicholas Maartens
INTRODUCTION: Wegener's granulomatosis (WG) is a systemic vasculitis that can affect a variety of organs including ear, nose and throat, lungs and kidneys. However WG is unusual in the pituitary and rare in the central nervous system. PRESENTATION OF CASE: A 56-year-old male with likely WG presented with polyuria and polydipsia despite six months of conservative medical management. MRI scanning revealed an enlarging heterogeneously enhancing pituitary gland. Following endoscopic transsphenoidal pituitary biopsy and debulking, final tissue pathology was diagnostic for WG in the pituitary gland...
February 20, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28260778/granulomatosis-with-polyangiitis-wegener-granulomatosis-with-unusual-presentation
#17
M A Mannan, K Pasha, S Nahar, K Begum
We came across a 32 years old male admitted in our hospital with prolonged low-grade fever, haemoptysis, leg swelling, weight loss, purpuric rashes and malaena. He received anti-TB treatment at another hospital without any improvement. He was pale with bullous and purpuric lesions over legs and feet. He also had features of consolidation over both lung fields. His CRP was 312mg/L, Urine R/M/E showed 40-50 RBC/HPF, Chest X-ray showed features of bilateral consolidation, c-ANCA-10U/L (positive); Tracheal aspirate for AFB was found to be negative...
January 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28220286/characteristics-of-hypertrophic-pachymeningitis-in-patients-with-granulomatosis-with-polyangiitis
#18
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...
April 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
#19
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
#20
Scott Kindle, Joseph Fanciullo
No abstract text is available yet for this article.
January 2017: Cutis; Cutaneous Medicine for the Practitioner
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