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wegeners granulomatous

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
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
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
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
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
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
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
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
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
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
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
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
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
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
Robert Ali, Candice Baldeo, Jesse Onyenekwe, Roshan Lala, Cristian Landa, Anwer Siddiqi
Granulomatosis with polyangiitis (GPA), previously termed Wegener's Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis...
2015: Case Reports in Rheumatology
Guangyuan Du, Jiang Zhu
Wegener's granulomatosis (WG) is a multisystem disease characterized by necrotizing granulomatous inflammation and vasculitis. WG classically involves the upper airway, lung and kidneys. Otolaryngologic manifestations are often the presenting symptoms in WG, Subglottic stenosis is a less common, but a life-threatening manifestation of WG, This paper studies about the laryngeal and tracheal stenosis diagnosis and surgical treatment of Wegener's granulomatosis in the past 20 years.
May 2015: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
Zhaohui Zheng, Jin Ding, Xueyi Li, Zhenbiao Wu
Granulomatosis with polyangiitis (GPA), formerly called Wegener's Granulomatosis, is characterized by necrotizing granulomatous inflammation and belongs to the family of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. The main clinical symptoms of GPA are vasculitis primarily involving upper and lower respiratory tracts, as well as kidneys. Gastrointestinal manifestations of GPA are less common (0-20 %), with gastric presentation mimicking a gastric cancer as an initial symptom. This is a descriptive case report of one patient, together with systematic review of the literature...
November 2015: Rheumatology International
James R Stone, Patrick Bruneval, Annalisa Angelini, Giovanni Bartoloni, Cristina Basso, Lubov Batoroeva, L Maximilian Buja, Jagdish Butany, Giulia d'Amati, John T Fallon, Adriana C Gittenberger-de Groot, Rosa H Gouveia, Marc K Halushka, Karen L Kelly, Ivana Kholova, Ornella Leone, Silvio H Litovsky, Joseph J Maleszewski, Dylan V Miller, Richard N Mitchell, Stephen D Preston, Angela Pucci, Stanley J Radio, E Rene Rodriguez, Mary N Sheppard, S Kim Suvarna, Carmela D Tan, Gaetano Thiene, Allard C van der Wal, John P Veinot
Inflammatory diseases of the aorta include routine atherosclerosis, aortitis, periaortitis, and atherosclerosis with excessive inflammatory responses, such as inflammatory atherosclerotic aneurysms. The nomenclature and histologic features of these disorders are reviewed and discussed. In addition, diagnostic criteria are provided to distinguish between these disorders in surgical pathology specimens. An initial classification scheme is provided for aortitis and periaortitis based on the pattern of the inflammatory infiltrate: granulomatous/giant cell pattern, lymphoplasmacytic pattern, mixed inflammatory pattern, and the suppurative pattern...
September 2015: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Raida Ben Salah, Faten Frikha, Mouna Snoussi, Masmoudi Abderrahmen, Yosr Hentati, Zeineb Mnif, Hamida Turki, Zouhir Bahloul
Wegener's granulomatosis (WG) is a multisystemic disease of unknown etiology characterized by necrotizing vasculitis and granulomatous inflammation. Although, the gastrointestinal tract can be involved in this vasculitis, the association between WG and Crohn's disease (CD) exists and was previously described. We report the case of a 29 year-old Tunisian white patient previously diagnosed with CD, presenting 3 years later with a perforated nasal septum, mucosal ulcers and sinusitis. He had also a skin ulcerative eruption of the trunk...
December 2014: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
Serkan Guneyli, Naim Ceylan, Selen Bayraktaroglu, Sercan Gucenmez, Kenan Aksu, Kenan Kocacelebi, Turker Acar, Recep Savas, Hudaver Alper
BACKGROUND: Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis), in which pulmonary involvement often predominates, is a multisystem granulomatous, necrotizing vasculitis that affects small and medium-sized vessels. In this study we evaluated various radiological findings of pulmonary GPA and focused on spiculated pulmonary lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy. METHODS: This retrospective study included 48 patients, aged 28-73 (mean, 47...
November 2016: Wiener Klinische Wochenschrift
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