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wegeners granulomatous

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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/28393729/granulomatous-polyangitis-wegener-granulomatosis-clinical-findings-and-results-of-long-term-follow-up
#2
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/28366156/-granulomatous-polyangitis-wegener-granulomatosis-clinical-findings-and-results-of-long-term-follow-up
#3
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/28318198/-subglottic-stenosis-as-the-initial-manifestation-of-wegener-s-granulomatosis-in-a-teenager-case-report
#4
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
#5
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/28295009/-refractory-form-of-orbital-granulomatosis-with-polyangiitis-a-clinical-and-morphological-study
#6
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/28220286/characteristics-of-hypertrophic-pachymeningitis-in-patients-with-granulomatosis-with-polyangiitis
#7
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
#8
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/27672552/retroperitoneal-fibrosis-with-periaortitis-a-case-report-of-an-unusual-form-of-presentation-of-granulomatosis-with-polyangiitis
#9
Elizabeth Manuely González Revilla, Araceli Abad Fernandez, María Teresa Río Ramirez, Sara Calero Pardo, María Antonia Juretschke Moragues
Granulomatosis with polyangiitis (GPA) is the name that has been used in recent years for Wegener's granulomatosis. This condition is a systemic inflammatory disease characterised by necrotizing vasculitis that affects small and medium-sized blood vessels (capillaries, arterioles, venules and arteries). The granulomatous inflammation affects the respiratory system; it also commonly affects the kidney and can very rarely affect large vessels such as the aorta and the surrounding retroperitoneal tissue. Early diagnosis and treatment is of vital importance because of the high risk of dissection and of obstruction of retroperitoneal structures...
2016: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27521731/sellar-wegener-granulomatosis-masquerading-as-cabergoline-resistant-prolactinoma
#10
Ilyas M Eli, Amol Raheja, Heather J Corn, Debra L Simmons, Cheryl Ann Palmer, William T Couldwell
BACKGROUND: Pituitary manifestation of Wegener granulomatosis (WG) is extremely rare. When there is pituitary involvement, the granulomatous inflammatory lesions involving the pituitary gland may appear several months to years after the primary diagnosis. CASE DESCRIPTION: We present a case of a 32-year-old woman who presented with galactorrhea, amenorrhea, and elevated serum prolactin levels. Imaging demonstrated a sellar lesion with characteristics of a pituitary macroadenoma...
November 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27468098/-intraocular-inflammation-autoimmune-or-infectious
#11
C Auw-Hädrich, S Heinzelmann, S Coupland
Presentation of 3 cases of intraocular inflammation: 1. 47-year old female patient with severe necrotising scleritis and uveitis with underlying granulomatous polyangiitis (formerly known as Wegener granulomatosis, in honour of the German pathologist Friedrich Wegener), known for 10 years. 2. 48-year old male patient with longstanding bilateral uveitis and granulomatous polyangiitis for 2 years. In the histopathological examination of the enucleation specimen, a retrolental tumour turned out to be a granuloma...
July 2016: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/27366530/airway-surgery-in-tracheostomised-patients-with-wegener-granulomatosis-leading-to-subglottic-stenosis
#12
Demet Altun, Nükhet Sivrikoz, Emre Çamcı
Wegener granulomatosis (WG) is a multisystemic disorder characterised by granulomatous inflammation of the respiratory system. The growing of proliferative tissue towards the larynx and trachea may cause airway obstruction on account of subglottic stenosis. In this situation, the surgical goal is to eliminate the airway obstruction by providing natural airway anatomy. While mild lesions do not require surgical intervention, in fixed lesions, surgical intervention is required, such as tracheostomy, laser resection and dilatation...
October 2015: Turkish Journal of Anaesthesiology and Reanimation
https://www.readbyqxmd.com/read/27294654/palisaded-neutrophilic-and-granulomatous-dermatitis-cutaneous-manifestation-of-lyme-disease-or-connected-with-ctd-case-report
#13
Magdalena Pirowska, Aleksander Obtułowicz, Grzegorz Dyduch, Sylwia Lipko-Godlewska, Anna Wojas-Pelc
INTRODUCTION: Palisaded neutrophilic and granulomatous dermatitis (PNGD) are terms which include such diseases as rheumatoid nodules, Churg-Strauss granuloma, and interstitial granulomatous dermatitis with arthritis. This heterogeneous group was first described in 1965. It is associated with immunological diseases. There are reported cases of coexistence with systemic lupus erythematosus, rheumatoid arthritis, Wegener's granuloma, inflammatory bowel disease, generalized vascular inflammation, and lymphoproliferative disorders...
June 2, 2016: Annals of Agricultural and Environmental Medicine: AAEM
https://www.readbyqxmd.com/read/27290815/-granulomatosis-with-polyangiitis-gpa-limited-to-upper-respiratory-tract-a-case-report
#14
Ivan Marković, Silva Puksić, Ana Gudelj-Gracanin, Ivan Ozegović, Melanie-Ivana Culo, Josko Mitrović, Jadranka Morović-Vergles
Granulomatosis with polyangiitis (Wegener's granulomatosis) is one of the anti-neutrophil cytoplasmic anti-body-associated small vessel vasculitides. Upper and lower respiratory system and kidneys are most commonly affected. The disease is characterized by granulomatous inflammation of the respiratory tract and necrotizing vasculitis of small to medium-sized blood vessels. Most patients show involvement of more than one organ systems at the time of diagnosis, and constitutional symptoms may be present. In around a quarter of patients the disease is initially localised, with involvement of upper respiratory tract or lungs...
January 2016: Lijec̆nic̆ki Vjesnik
https://www.readbyqxmd.com/read/27279517/an-eosinophilic-variant-granulomatosis-with-polyangiitis-involving-the-dura-bilateral-orbits-and-mastoids
#15
Hasan Al-Hakami, Abdurhman S Al-Arfaj, Mohammed Al-Sohaibani, Najma A Khalil
Granulomatosis with polyangiitis (GPA) formerly called Wegener's granulomatosis is a chronic necrotizing granulomatous inflammatory disease with systemic vasculitis involving the upper and lower respiratory tract, and kidneys. The typical histopathology is that of necrotizing granulomatous inflammation with palisading histiocytes, neutrophils, and lymphocytes. We report a case of a 57-year-old lady presenting with left eye swelling, left ear pain and discharge, but with no pulmonary or renal symptoms. Investigations revealed positive cytoplasmic antineutrophil cytoplasmic antibodies  and proteinase 3 antibodies...
June 2016: Saudi Medical Journal
https://www.readbyqxmd.com/read/27151715/mastoiditis-and-facial-paralysis-as-initial-manifestations-of-temporal-bone-systemic-diseases-the-significance-of-the-histopathological-examination
#16
Alma Aurelia Maniu, Oana Harabagiu, Laura Otilia Damian, Eugen HoraŢiu Ştefănescu, Bogdan Marius FănuŢă, Andreea Cătană, Carmen Aurelia Mogoantă
UNLABELLED: Several systemic diseases, including granulomatous and infectious processes, tumors, bone disorders, collagen-vascular and other autoimmune diseases may involve the middle ear and temporal bone. These diseases are difficult to diagnose when symptoms mimic acute otomastoiditis. CASE REPORTS: The present report describes our experience with three such cases initially misdiagnosed. Their predominating symptoms were otological with mastoiditis, hearing loss, and subsequently facial nerve palsy...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/26963309/-computed-tomography-findings-in-pulmonary-involvement-of-wegener-s-granulomatosis-pictorial-review
#17
REVIEW
Necdet Poyraz, Celalettin Korkmaz, Durdu Mehmet Yavşan, Suat Keskin, Turgut Teke, Kemal Ödev
Wegener's granulomatosis is a necrotising granulomatous vasculitis which has a variable manifestations in the chest that are best described on computed tomography. Imaging findings may include masses or nodules, which may cavitate; consolidations and ground-glass opacities. Wegener's granulomatosis can mimic pneumonia, malignancy, and noninfectious inflammatory diseases. The purpose of this pictorial essay is to demonstrate the characteristic computed tomography findings of pulmonary Wegener's granulomatosis...
2015: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/26830405/non-infectious-inflammatory-lesions-of-the-sinonasal-tract
#18
REVIEW
Timothy R Helliwell
This review covers the histopathology and pathogenesis of non-infectious inflammatory diseases of the sinonasal tract, in particular, sarcoidosis, granulomatous vasculitides Wegener, Churg-Strauss), relapsing polychondritis, eosinophilic angiocentric fibrosis, chronic rhinosinusitis and nasal perforations. Molecular associations and mechanisms are emphasised to assist pathologists to put their observations into the context of clinical, genetic and environmental influences on patients' diseases.
March 2016: Head and Neck Pathology
https://www.readbyqxmd.com/read/26684637/clinic-manifestations-in-granulomatosis-with-polyangiitis
#19
REVIEW
A Greco, C Marinelli, M Fusconi, G F Macri, A Gallo, A De Virgilio, G Zambetti, M de Vincentiis
Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is pathologically characterised by an inflammatory reaction pattern (necrosis, granulomatous inflammation and vasculitis) that occurs in the upper and lower respiratory tracts and kidneys. Although the aetiology of GPA remains largely unknown, it is believed to be autoimmune in origin and triggered by environmental events on a background of genetic susceptibility...
June 2016: International Journal of Immunopathology and Pharmacology
https://www.readbyqxmd.com/read/26513951/wegener-granulomatosis-as-possible-cause-of-vertigo-case-report-and-review
#20
REVIEW
J Goderis, S De Schepper, P Vannieuwenhuyze, B Schmelzer
OBJECTIVE: Wegener granulomatosis (WG) or granulomatosis with polyangiitis (GPA) is a multi-system necrotizing granulomatous vasculitis that classically affects the upper respiratory tract, lungs and kidneys. We report the unusual clinical course of a patient with WG, and we present a literature review on the association between WG and vertigo. RESULTS: In our case, a 56-year-old female presented with a several-month history of chronic sinusitis and otalgia that was refractory to all initiated outpatient therapies...
2015: B-ENT
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