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Wegener disease

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
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
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
Tim Vanbellingen, Thomas Nyffeler, Tobias Nef, Gert Kwakkel, Stephan Bohlhalter, Erwin E H van Wegen
BACKGROUND: Patients with Parkinson's disease exhibit disturbed dexterity. Validated self-reported outcomes for dexterity in Parkinson's disease are lacking. The aim of this study was to investigate the reliability, content and construct validity of a new Dexterity Questionnaire 24. METHODS: One hundred and three patients with Parkinson's disease completed the Dexterity Questionnaire 24 (score range 24-96), at baseline and four weeks later. The internal consistency was determined...
September 15, 2016: Parkinsonism & related Disorders
Ravish Kshatriya, Viral Patel, Sanjay Chaudhari, Purvesh Patel, Dhaval Prajapati, Nimit Khara, Rajiv Paliwal, Sateesh Patel
Pulmonary tuberculosis is commonly presented as cavitary lesion and infiltrations. It commonly involves upper lobe. Lower lobe involvement is less common. Various atypical presentations of tuberculosis on radiology are reported like mass, solitary nodule, multi lober involvement including lower lobes. Atypical presentations are more commo in patients with immunocompromised conditions like Diabetes Mellitus, anemia, renal failure, liver diseases, HIV infection, malignancy, patients on immunosuppressive therapy...
September 2016: Lung India: Official Organ of Indian Chest Society
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
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
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
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
Gokhan Sargin, Taskin Senturk
The first International Chapel Hill Consensus Conference (CHHC) was held in 1994. There have been suggestions about the nomenclature of systemic vasculitis. Important categories were added to the classification of vasculitis, and many changes were made for disease names in the second CHHC 2012, which were not included in the CHCC 1994. The new nomenclature was introduced instead of being referred to by many names such as Churg-Strauss and Wegener"s. New categories such as Behçet"s and Cogan etc. were also added...
June 26, 2016: Revista Brasileira de Reumatologia
Lucyna Jóźwiak, Izabela Ławnicka, Andrzej Książek
Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis of unknown aetiology, often related to the antineutrophil cytoplasmic antibody (ANCA). GPA was previously named Wegener's granulomatosis (WG). The disease frequently has multisystemic presentation, targeting mainly the respiratory tract and kidneys, but gastrointestinal involvement is uncommon. Crohn's disease (CD) is an inflammatory bowel disease (IBD) with many extraintestinal manifestations. Clinically, symptoms of WG and CD can mimic each other...
2016: Reumatologia
E Zimba, O Olkhova
Granulomatosis with polyangiitis (Wegener's disease) - systemic vasculitis, initial manifestations, the clinical picture may be present in a wide variety. This leads to difficulties in establishing a timely diagnosis. The prognosis in untreated generalized granulomatosis with polyangiitis is extremely poor. The present case report illustrates a late diagnosis of granulomatosis with polyangiitis. A 53-year-old woman was diagnosed with granulomatosis with polyangiitis only after ten months of onset of disease...
May 2016: Georgian Medical News
Brice Korkmaz, Adam Lesner, Carla Guarino, Magdalena Wysocka, Christine Kellenberger, Hervé Watier, Ulrich Specks, Francis Gauthier, Dieter E Jenne
Proteinase 3 (PR3) has received great scientific attention after its identification as the essential antigenic target of antineutrophil cytoplasm antibodies in Wegener's granulomatosis (now called granulomatosis with polyangiitis). Despite many structural and functional similarities between neutrophil elastase (NE) and PR3 during biosynthesis, storage, and extracellular release, unique properties and pathobiological functions have emerged from detailed studies in recent years. The development of highly sensitive substrates and inhibitors of human PR3 and the creation of PR3-selective single knockout mice led to the identification of nonredundant roles of PR3 in cell death induction via procaspase-3 activation in cell cultures and in mouse models...
July 2016: Pharmacological Reviews
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