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Wegeners granulomatosis

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: Eur J Rheumatol
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
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
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
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
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
Anne Laure Bernat, Etienne Lefevre, Damien Sène, Philippe Herman, Homa Adle Biassette, Sebastien Froelich
Granulomatosis with polyangitis (Wegener's disease) is a rare ANCA (anti-neutrophil cytoplasmic antibody) positive vasculitis that seldom involves brain and meninges. We herein present a case of a 35-year-old woman presenting with granulomatosis with polyangitis involving the brain and the meninges. The histological diagnosis required a complete resection of the lesion. Despite the possibility of post operative wound infection surgery was mandatory to ascertain the diagnostic and to treat locally.
September 23, 2016: World Neurosurgery
Chen-Lu Yang, Kai Shen, Qi-Bing Xie, Geng Yin
No abstract text is available yet for this article.
2016: Chinese Medical Journal
K Gleghorn, J Wilson, M Wilkerson
Rituximab is an anti-CD20 monoclonal antibody with considerable potential in dermatology due to an increase in off-label indications. Chronic graft-versus-host disease and pemphigus vulgaris are two of the most promising indications for off-label use of rituximab. It is a generally safe alternative that should be considered when traditional therapy with corticosteroids or immunosuppressants has failed or caused significant intolerance. Currently, rituximab is only FDA-approved for treatment of follicular and diffuse large B-cell non-Hodgkin's lymphoma, rheumatoid arthritis, chronic lymphocytic leukemia, granulomatosis with polyangiitis (formerly Wegener's granulomatosis) and microscopic polyangiitis...
September 2016: Skin Therapy Letter
Gabriel Horta-Baas, Esteban Meza-Zempoaltecatl, Mario Pérez-Cristóbal, Leonor Adriana Barile-Fabris
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a systemic necrotizing vasculitis, which affects small and medium sized blood vessels and is often associated with cytoplasmic anti-neutrophil cytoplasmic antibodies (ANCA). Inflammatory pseudotumor is a rare condition characterized by the appearance of a mass lesion that mimics a malignant tumor both clinically and on imaging studies, but that is thought to have an inflammatory/reactive pathogenesis. We report a patient with a GPA which was originally diagnosed as malignancy...
July 2016: Gaceta Médica de México
Akashdeep Singh, Deepinder Chhina, R K Soni, Chandan Kakkar, U S Sidhu
BACKGROUND: Pulmonary nocardiosis is a rare but a life-threatening infection caused by Nocardia spp. The diagnosis is often missed and delayed resulting in delay in appropriate treatment and thus higher mortality. AIM: In this study, we aim to evaluate the clinical spectrum and outcome of patients with pulmonary nocardiosis. METHODS: A retrospective, 5-year (2009-2014) review of demographic profile, risk factors, clinical manifestations, imaging findings, treatment, and outcome of patients with pulmonary nocardiosis admitted to a tertiary care hospital...
July 2016: Lung India: Official Organ of Indian Chest Society
Mona Mlika, Saoussen Hantous, Imen Helal, Emna Braham, Ali Mrabet, Faouzi El Mezni
No abstract text is available yet for this article.
March 2016: La Tunisie Médicale
Leena Nahata, Vidya Sivaraman, Gwendolyn P Quinn
OBJECTIVE: To assess fertility counseling and preservation practices among children, adolescents, and young adults with rheumatic diseases undergoing cyclophosphamide (CTX) treatment. DESIGN: Retrospective chart review (2006-2016). SETTING: Academic pediatric center. PATIENT(S): Male and female patients with systemic lupus erythematosus, Wegener's granulomatosis/granulomatosis with polyangiitis, or other vaculitides, receiving CTX treatment...
August 10, 2016: Fertility and Sterility
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...
August 10, 2016: World Neurosurgery
L Chaput, N Rabot, N Limousin, J-P Cottier, B Lioger, M Samimi
BACKGROUND: Reversible posterior leukoencephalopathy syndrome (RPLS) is characterised by clinical neurological features of sudden onset and brain MRI findings such as T2/Flair white matter hyperintensities. RPLS can occur in autoimmune diseases, and rarely in systemic vasculitis. We report a case of RPLS in a woman presenting granulomatosis with polyangiitis (Wegener's granulomatosis). PATIENTS AND METHODS: A 22-year-old female patient was treated with methylprednisolone pulses for granulomatosis with polyangiitis and neurological impairment...
August 5, 2016: Annales de Dermatologie et de Vénéréologie
Marcel Hanisch, Leopold F Fröhlich, Johannes Kleinheinz
BACKGROUND: Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener's granulomatosis, is a rare systemic disease of unknown etiology which can affect all areas of the body, including the oral cavity. The typical oral manifestations occur as nonspecific erosive/ulcerative lesions of the oral cavity or appear with hyperplastic gingivitis, a so called "strawberry gingivitis". CASE PRESENTATION: We report here about an extremely rare case with hyperplastic gingivitis as the first sign of recurrence of GPA in the absence of oral manifestations in the primary disease...
2016: BMC Oral Health
Fabrizio Conti, Fulvia Ceccarelli, Giancarlo Iaiani, Carlo Perricone, Alessandra Giordano, Luigino Amori, Francesca Miranda, Laura Massaro, Viviana Antonella Pacucci, Simona Truglia, Gabriella Girelli, Azis Fakeri, Gloria Taliani, Chiara Temperoni, Francesca Romana Spinelli, Cristiano Alessandri, Guido Valesini
BACKGROUND: Staphylococcus aureus (SA) is a commensal bacterium representing one of the most important components of the skin microbiome, mostly isolated in the anterior nares. A higher rate of SA nasal colonization in patients affected by Wegener's granulomatosis and rheumatoid arthritis compared with healthy subjects (HS) has been described. No studies focusing on systemic lupus erythematosus (SLE) are available. We aimed at analyzing the prevalence of SA nasal carriers in an SLE cohort and evaluating correlation between nasal colonization and clinical, laboratory and therapeutic features...
2016: Arthritis Research & Therapy
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
Felipe Paiva Fonseca, Bernar Monteiro Benites, Ana Luisa Vanalle Ferrari, Zoraida Sachetto, Glécio Vaz de Campos, Oslei Paes de Almeida, Eduardo Rodrigues Fregnani
Granulomatosis with polyangiitis (GPA) is a potentially lethal disease characterized by systemic necrotizing vasculitis, which affects small- and medium sized blood vessels and is often associated with serum cytoplasmic ANCA. Upper and lower respiratory tract and kidney are the most involved sites, but oral lesions can be identified in 6 to 13% of the cases, whereas in only 2% of the cases, oral manifestations represent the first signal of the disease usually as gingival swellings or unspecific ulcerations...
July 21, 2016: Australian Dental Journal
E M Miloslavsky, N Lu, S Unizony, H K Choi, P A Merkel, P Seo, R Spiera, C A Langford, G S Hoffman, C G M Kallenberg, E W St Clair, N K Tchao, F Fervenza, P A Monach, U Specks, J H Stone
Objective To examine the impact of antineutrophil cytoplasmic antibody (ANCA) type and ANCA-associated vasculitis (AAV) diagnosis on demographic features, disease manifestations, and clinical outcomes. We focused on patients that account for the differences between ANCA type and disease type classifications: anti-myeloperoxidase (MPO) ANCA-positive and ANCA-negative patients with granulomatosis with polyangiitis (GPA). Methods Pooled analysis of the Wegener's Granlomatosis Etanercept Trial (WGET) and the Rituximab in AAV (RAVE) trial comparing MPO-ANCA+ GPA and ANCA-negative GPA patients to proteinase 3 (PR3) ANCA+ GPA and MPO-ANCA+ microscopic polyangiitis (MPA) patients...
July 18, 2016: Arthritis & Rheumatology
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