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https://www.readbyqxmd.com/read/28816552/the-role-of-biopsy-in-lacrimal-gland-inflammation-a-clinicopathologic-study
#1
Panitee Luemsamran, Jack Rootman, Valerie A White, Nariman Nassiri, Manraj K S Heran
PURPOSE: To determine the causes of lacrimal gland inflammation based on histopathology and systemic evaluation. METHODS: This is a retrospective case series study. From the University of British Columbia Orbit Clinic between January 1976 and December 2008, we reviewed the medical records of 60 patients who presented with inflammatory features of the lacrimal gland (i.e., erythema, edema, or tenderness) in which the diagnoses were not possible clinically and on imaging alone...
August 17, 2017: Orbit
https://www.readbyqxmd.com/read/28811951/granulomatosis-with-polyangiitis-presenting-as-pyrexia-of-unknown-origin-leukocytosis-and-microangiopathic-haemolytic-anemia
#2
Sima Terebelo, Iona Chen
A 66-year-old woman presented to the Emergency Department with a florid sepsis-like picture, a two-week history of fever, relative hypotension with end organ ischemia (unexplained liver enzyme and troponin elevations), and nonspecific constitutional symptoms. She was initially found to have a urinary tract infection but, despite appropriate treatment, her fever persisted and her white blood cell count continued to rise. During her hospitalization the patient manifested leukocytosis to 47,000 WBC/μL, ESR 67 mm/hr (normal range 0-42 mm/hr), CRP 17...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28794143/differential-diagnosis-of-granulomatous-lung-disease-clues-and-pitfalls-number-4-in-the-series-pathology-for-the-clinician-edited-by-peter-dorfm%C3%A3-ller-and-alberto-cavazza
#3
Shinichiro Ohshimo, Josune Guzman, Ulrich Costabel, Francesco Bonella
Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis)...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28791214/cavitary-lung-lesions-melioidosis-and-pulmonary-embolism-causing-necrotizing-pneumonia
#4
N Mohammad, W S Wan Ghazali
Cavitary lung lesions of various etiologies may be encountered in patients with respiratory symptoms associated with fever. Non-malignant cavitary lesions may mimic malignant lung lesions on most of radiographic modalities including chest radiographs or thoracic computed tomography (CT). Primary lung malignancy can be detected in as high as one-fifths of CT thorax as cavitary lesions and the remaining aetiologies may be due to bacterial, parasitic, and invasive fungal infections, as well as Granulomatosis with polyangiitis (GPA), sarcoidosis, septic thrombo-embolism, and lung metastasis from extra-pulmonary primaries...
2017: IDCases
https://www.readbyqxmd.com/read/28781854/skin-nodule-reveals-lung-cancer-in-a-patient-with-granulomatosis-with-polyangiitis
#5
Taylor Doberstein, Brian L Swick, Namrata Singh
A 69-year-old male with granulomatosis with polyangiitis presented with new skin nodules. Skin biopsy showed metastatic poorly differentiated lung adenocarcinoma. The skin nodule was the initial presentation of his lung cancer. There is need for increased vigilance for cancer risk in ANCA-associated vasculitis patients.
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28780079/antineutrophil-cytoplasmic-antibody-associated-vasculitides-and-igg4-related-disease-a-new-overlap-syndrome
#6
REVIEW
François-Xavier Danlos, Giovanni Maria Rossi, Daniel Blockmans, Giacomo Emmi, Andreas Kronbichler, Stéphane Durupt, Claire Maynard, Luminita Luca, Cyril Garrouste, Bertrand Lioger, Rachel Mourot-Cottet, Robin Dhote, Jean-Benoit Arlet, Thomas Hanslik, Philippe Rouvier, Mikael Ebbo, Xavier Puéchal, Dominique Nochy, Agnès Carlotti, Luc Mouthon, Loïc Guillevin, Augusto Vaglio, Benjamin Terrier
OBJECTIVE: Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. METHODS: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria...
August 2, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28775083/rare-cause-of-respiratory-failure-in-a-young-woman-isolated-diffuse-alveolar-haemorrhage-requiring-extracorporeal-membrane-oxygenation
#7
David Kelly, Damodar Makkuni, Dhiraj Ail
A previously healthy 21-year-old young woman presented with worsening dyspnoea and haemoptysis. Imaging was suggestive of widespread pulmonary haemorrhage. There was no other organ system involvement in particular no evidence of renal involvement. Raised antimyeloperoxidase titres allowed diagnosis of isolated diffuse alveolar haemorrhage (DAH) secondary to microscopic polyangiitis (MPA). The patient rapidly deteriorated with worsening respiratory failure despite invasive mechanical ventilation and required extracorporeal membrane oxygenation (ECMO)...
August 3, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28772006/accuracy-of-magnetic-resonance-imaging-for-grading-of-subglottic-stenosis-in-patients-with-granulomatosis-with-polyangiitis-correlation-with-pulmonary-function-tests-and-laryngoscopy
#8
Frank O Henes, Martin Laudien, Laura Linsenhoff, Jan P Bremer, Tim Oqueka, Gerhard Adam, Gerhard Schön, Peter Bannas
OBJECTIVES: To compare magnetic resonance imaging (MRI)-based and laryngoscopy-based subglottic stenosis (SGS) grading with pulmonary function tests (PFT) in patients with granulomatosis with polyangiitis (GPA). METHODS: In this retrospective study we included 118 examinations of 44 patients with GPA and suspected SGS. All patients underwent MRI, laryngoscopy, and PFT. Stenosis was graded on a 4-point scale by endoscopy and MRI using the Meyer-Cotton (MC)-Score (score1: ≤50%; 2: 51-70%; 3: 71-99%; 4: 100%) and as percentage by MRI...
August 3, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28771641/prospective-monitoring-of-in-vitro-produced-pr3-anca-does-not-improve-relapse-prediction-in-granulomatosis-with-polyangiitis
#9
Judith Land, Wayel H Abdulahad, Suzanne Arends, Jan-Stephan F Sanders, Coen A Stegeman, Peter Heeringa, Abraham Rutgers
OBJECTIVES: Patients with granulomatosis with polyangiitis (GPA) are prone to disease relapse. Currently, no good biomarkers are available to predict relapses in individual patients. This study aimed to determine whether patients at risk for relapse can be distinguished based on increased in vitro autoantibody production. METHODS: Eighty-four proteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) positive GPA outpatients were prospectively monitored for up to two years and 32 healthy controls were included...
2017: PloS One
https://www.readbyqxmd.com/read/28769139/three-atypical-manifestations-of-granulomatosis-with-polyangiitis-lateral-medullary-syndrome-anterior-cheek-mass-and-melting-scleritis-of-eye
#10
Saeedeh Shenavandeh, Peyman Petramfar
Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis) is a vasculitis with various organ involvement. There have been a few cases of CNS stroke and rare cases of lateral medullary infarction (LMI) as a manifestation of GPA. Also there have been reports of sinuses, nose and laryngeal masses mistakenly referred as carcinomas and subsequently GPA was diagnosed in their pathological reports. Another severe fulminant manifestation can be necrotizing scleritis leading to perforation of sclera. Therefore, here we present some rare and fulminant manifestations of GPA in 3 separate cases for further emphasis of the unusual manifestations of GPA that should always be kept in mind...
2017: Reumatologia
https://www.readbyqxmd.com/read/28769112/vasculitis-mepolizumab-for-eosinophilic-granulomatosis-with-polyangiitis
#11
Loïc Guillevin
No abstract text is available yet for this article.
August 3, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28766857/birmingham-vasculitis-activity-score-of-more-than-9-5-at-diagnosis-is-an-independent-predictor-of-refractory-disease-in-granulomatosis-with-polyangiitis
#12
Juyoung Yoo, Ho Jae Kim, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
AIM: We investigated whether clinical manifestations, anti-neutrophil cytoplasmic antibodies (ANCAs), Birmingham vasculitis activity score (BVAS) for granulomatosis with polyangiitis (GPA) and five factor score (FFS) at diagnosis can predict relapse or refractory disease in 30 histology-proven GPA patients with follow-up duration ≥ 12 weeks. METHODS: We reviewed the medical records of 30 GPA patients. We collected clinical data, ANCAs, BVAS for GPA, FFSs at diagnosis, and we compared variables between the two groups based on relapse or refractory disease...
August 1, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28766511/unusual-cardiac-involvement-in-granulomatosis-with-polyangiitis-manifesting-as-acute-congestive-heart-failure
#13
Htoo Kyaw, Deepika Misra, Malary M Mani, Won J Park, Meir Shinnar
No abstract text is available yet for this article.
August 2017: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/28766066/outcome-of-kidney-transplantation-in-pediatric-patients-with-anca-associated-glomerulonephritis-a-single-center-experience
#14
Damien Noone, Rae S M Yeung, Diane Hebert
OBJECTIVES: Data on kidney transplant outcomes for pediatric patients with end-stage renal disease (ESRD) secondary to anti-neutrophil cytoplasmic antibody glomerulonephritis (ANCA GN), particularly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), is limited. We describe our experience of kidney transplantation in pediatric ANCA GN patients. METHODS: We performed a retrospective review of patients with ANCA GN who developed ESRD and were transplanted at a single center between the years 2000 and 2014...
August 1, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28765242/pulmonary-involvement-in-antineutrophil-cytoplasmic-antibodies-anca-associated-vasculitis-the-influence-of-anca-subtype
#15
Aladdin J Mohammad, Kristian H Mortensen, Judith Babar, Rona Smith, Rachel B Jones, Daiki Nakagomi, Pasupathy Sivasothy, David R W Jayne
OBJECTIVE: To describe pulmonary involvement at time of diagnosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), as defined by computed tomography (CT). METHODS: Patients with thoracic CT performed on or after the onset of AAV (n = 140; 75 women; granulomatosis with polyangiitis, n = 79; microscopic polyangiitis MPA, n = 61) followed at a tertiary referral center vasculitis clinic were studied. Radiological patterns of pulmonary involvement were evaluated from the CT studies using a predefined protocol, and compared to proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA specificity...
August 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28762062/sequential-rituximab-and-omalizumab-for-the-treatment-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome
#16
REVIEW
David Aguirre-Valencia, Iván Posso-Osorio, Juan-Carlos Bravo, Fabio Bonilla-Abadía, Gabriel J Tobón, Carlos A Cañas
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Rituximab is associated with a good response in the treatment of vasculitis, but a variable response for the control of allergic symptoms...
July 31, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28756866/tuberculosis-granulomatosis-with-polyangiitis-or-both-a-case-report
#17
Á Nava-Castañeda, F Martín, S Voorduin, F Zuazo
INTRODUCTION: Tuberculosis (TB) is a chronic granulomatose infection, and granulomatosis with polyangiitis (GP) is a small vessel vasculitis, both of which affect the lungs. The combination of these diseases is rare. Both have similar clinical features, making the differential diagnosis difficult. CASE REPORT: It concerns a 37 year-old female undergoing treatment for pulmonary TB, who presented with left ocular proptosis, eyelid and conjunctival edema and erythema...
July 27, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28754431/recommendations-of-the-brazilian-society-of-rheumatology-for-the-induction-therapy-of-anca-associated-vasculitis
#18
REVIEW
Alexandre Wagner Silva de Souza, Ana Luisa Calich, Henrique de Ataíde Mariz, Manuella Lima Gomes Ochtrop, Ana Beatriz Santos Bacchiega, Gilda Aparecida Ferreira, Jozelia Rêgo, Mariana Ortega Perez, Rosa Maria Rodrigues Pereira, Wanderley Marques Bernardo, Roger Abramino Levy
The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed), EMBASE and Cochrane database to retrieve articles until October 2016...
July 25, 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28752492/pulmonary-involvement-in-systemic-vasculitis
#19
REVIEW
Luis Felipe Flores-Suárez, Marco A Alba, Heidegger Mateos-Toledo, Natllely Ruiz
PURPOSE OF REVIEW: The purpose of this study is to describe the most relevant advances concerning lung involvement in the ANCA-associated vasculitides (excluding eosinophilic granulomatosis with polyangiitis which may have different disease mechanisms). Focus is on pathophysiology, recent important imagenological procedures, treatment, and outcome. RECENT FINDINGS: Emerging information exists on potential newly investigated diagnostic procedures (v.g. transbronchial cryobiopsies), detailed tomographic abnormalities, the potential favorable role of rituximab and the still uncertain one of plasma exchange in the treatment, and the increasing description of interstitial lung disease...
September 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28741986/serum-ferritin-as-an-activity-marker-for-granulamotosis-with-polyangiitis
#20
Hamit Kucuk, Ozkan Varan, Berna Goker, Berivan Bitik, Mehmet Akif Ozturk, Seminur Haznedaroglu, Abdurrahman Tufan
BACKGROUND: Serum ferritin correlates well with the activities of systemic lupus erythematosus (SLE) and dermatomyositis, but it has not been previously studied in patients with vasculitis. METHODS: Medical records of granulomatosis with polyangiitis (GPA, Wegener's granulomatosis) patients with at least six months of regular follow-up were evaluated. The activity of GPA was assessed with Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG)...
November 2017: Renal Failure
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