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https://www.readbyqxmd.com/read/28527170/oculo-otological-manifestations-in-a-case-of-granulomatosis-with-polyangiitis
#1
Archana Sonawale, Anjali Rajadhyaksha, Shreepriya Mangalgi
A 44 year old lady presented with acute onset of loss of vision in the right eye and cough with mucopurulent expectoration for two months. Ophthalmic examination revealed central retinal artery occlusion (CRAO). Chest radiograph showed multiple cavitatory nodules with fluid levels. Sputum was negative for AFB and ANCA was strongly positive suggestive of a diagnosis of Granulomatosis with Polyangiitis (GPA). Within the next few weeks the patient had rapid detenoration due to left eye CRAO, progressive bilateral hearing loss, facial palsy and retro orbital mass...
April 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28524146/-combination-therapy-of-refractory-localized-granulomatosis-with-polyangiitis-and-orbital-involvement
#2
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/28521808/b-cell-repopulation-kinetics-after-rituximab-treatment-in-anca-associated-vasculitides-compared-to-rheumatoid-arthritis-and-connective-tissue-diseases-a-longitudinal-observational-study-on-120-patients
#3
Jens Thiel, Marta Rizzi, Marie Engesser, Ann-Kathrin Dufner, Arianna Troilo, Raquel Lorenzetti, Reinhard E Voll, Nils Venhoff
BACKGROUND: B cell depletion with rituximab (RTX) is approved for treatment of rheumatoid arthritis (RA) and ANCA-associated vasculitides (AAV). Recently, RTX has been shown to be effective in AAV maintenance therapy, but an optimal RTX treatment schedule is unknown and the time to B cell repopulation after RTX has not been studied. METHODS: Retrospective single-center analysis of B cell repopulation in patients with AAV, RA or connective tissue disease (CTD) treated with RTX...
May 18, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28521792/m2-macrophage-is-the-predominant-phenotype-in-airways-inflammatory-lesions-in-patients-with-granulomatosis-with-polyangiitis
#4
Alexandre Wagner Silva de Souza, Mirjan van Timmeren, Jan-Stephan Sanders, Coen Stegeman, Peter Heeringa, Cees G M Kallenberg, Johanna Westra
BACKGROUND: Macrophages may present two distinct phenotypes indicated as M1 and M2 under different stimuli. M1 and M2 macrophages have divergent functions that range from enhancement of inflammation for M1 to tissue repair and remodeling for M2 macrophages. The objective of this study was to evaluate the distribution of M1 and M2 macrophage phenotypes in biopsies from the airways of patients with active granulomatosis with polyangiitis (GPA) and to analyze their associations with T and B cells in those biopsies, and with nasal carriage of Staphylococcus aureus, disease parameters and therapy...
May 18, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28521688/vasculitis-following-influenza-vaccination-a-review-of-the-literature
#5
Toru Watanabe
BACKGROUND: Influenza vaccine is safe and effective for the general population as well as for patients with autoimmune diseases. However, although rare, vasculitis has been reported as an adverse event following influenza vaccination. OBJECT: The aims of this literature review were to identify patients who developed vasculitis following influenza vaccination and to clarify the clinical manifestations of vasculitis in these patients. METHODS: Using the PubMed database and search engine, we performed a search of the English-language literature by combining the term influenza vaccination with each term for a specific form of vasculitis from January 1966 through April 2016...
May 17, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28514619/targeting-eosinophils-in-eosinophilic-granulomatosis-with-polyangiitis
#6
EDITORIAL
Ratko Djukanovic, Paul M O'Byrne
New England Journal of Medicine, Volume 376, Issue 20, Page 1985-1986, May 2017.
May 18, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28514601/mepolizumab-or-placebo-for-eosinophilic-granulomatosis-with-polyangiitis
#7
Michael E Wechsler, Praveen Akuthota, David Jayne, Paneez Khoury, Amy Klion, Carol A Langford, Peter A Merkel, Frank Moosig, Ulrich Specks, Maria C Cid, Raashid Luqmani, Judith Brown, Stephen Mallett, Richard Philipson, Steve W Yancey, Jonathan Steinfeld, Peter F Weller, Gerald J Gleich
BACKGROUND: Eosinophilic granulomatosis with polyangiitis is an eosinophilic vasculitis. Mepolizumab, an anti-interleukin-5 monoclonal antibody, reduces blood eosinophil counts and may have value in the treatment of eosinophilic granulomatosis with polyangiitis. METHODS: In this multicenter, double-blind, parallel-group, phase 3 trial, we randomly assigned participants with relapsing or refractory eosinophilic granulomatosis with polyangiitis who had received treatment for at least 4 weeks and were taking a stable prednisolone or prednisone dose to receive 300 mg of mepolizumab or placebo, administered subcutaneously every 4 weeks, plus standard care, for 52 weeks...
May 18, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28509135/renal-limited-vasculitis-with-elevated-levels-of-multiple-antibodies
#8
Noriaki Sato, Hideki Yokoi, Hirotaka Imamaki, Eiichiro Uchino, Kaoru Sakai, Takeshi Matsubara, Tatsuo Tsukamoto, Sachiko Minamiguchi, Motoko Yanagita
Renal-limited vasculitis (RLV) is a type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents with crescentic glomerulonephritis with no other organ involvement. Although several studies reported patients with crescentic glomerulonephritis who were dual positive for proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA or ANCA and anti-glomerular basement membrane (GBM) antibody, patients positive for all three antibodies, i.e., triple-positive patients, were rarely reported. We herein report the case of a male with pauci-immune type crescentic glomerulonephritis positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibody...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509134/two-cases-of-atypical-hemolytic-uremic-syndrome-ahus-and-eosinophilic-granulomatosis-with-polyangiitis-egpa-a-possible-relationship
#9
Mercedes Cao, Tamara Ferreiro, Bruna N Leite, Francisco Pita, Luis Bolaños, Francisco Valdés, Angel Alonso, Eduardo Vázquez, Juan Mosquera, María Trigás, Santiago Rodríguez
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal failure. It is related to genetic mutations of the alternative complement pathway and is difficult to differentiate from other prothrombotic microangiopathies. Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome, CSS) is a systemic ANCA-associated vasculitis and a hypereosinophilic disorder where eosinophils seem to induce cell apoptosis and necrosis and therefore, vasculitis...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508974/pauci-immune-crescentic-glomerulonephritis-associated-with-use-of-trimethoprim-sulfamethoxazole
#10
Shruti S Hegde, Vanesa Bijol, Bertrand L Jaber
Drug-induced pauci-immune crescentic glomerulonephritis has been described with several agents, including propylthiouracil, minocycline, D-penicillamine, and hydralazine. We present the case of a 60-year-old man who presented with rapidly progressive glomerulonephritis in the setting of recent use of trimethoprim-sulfamethoxazole complicated by the development of the Stevens-Johnson syndrome, and was found to have biopsy-proven pauci-immune crescentic glomerulonephritis and undetectable anti-neutrophilic cytoplasmic antibodies...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508972/development-of-intracerebral-hemorrhage-in-the-short-term-clinical-course-of-a-patient-with-microscopic-polyangiitis-without-neurological-symptoms-at-diagnosis-an-autopsy-case
#11
Yoshia Miyawaki, Takayuki Katsuyama, Ken-Ei Sada, Kohei Taniguchi, Yuki Kakio, Jun Wada
A 77-year-old man with high-grade fever, progressive renal dysfunction, high serum level of C-reactive protein and positive serum myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) was diagnosed with microscopic polyangiitis with rapidly progressive glomerulonephritis, and remission induction treatment with glucocorticoids and intravenous cyclophosphamide was initiated. Although his general condition improved in a short time, intracerebral hemorrhage occurred 12 days after the initiation of treatment and emergent hematoma evacuation was performed...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28500877/clinically-isolated-aortitis-pitfalls-progress-and-possibilities
#12
REVIEW
Ilkay Cinar, He Wang, James R Stone
Non-infectious aortitis may be caused by several distinct systemic rheumatologic diseases. In some patients, aortitis is identified either pathologically or radiologically in the absence of clinical evidence of a systemic vasculitis. By consensus nomenclature, such cases are referred to as clinically isolated aortitis (CIA). Some systemic disorders may initially present as CIA including giant cell arteritis (GCA), IgG4-related disease, infectious aortitis, and granulomatosis with polyangiitis. CIA most commonly occurs in women of European descent over the age of 50 and, thus, mirrors the gender, age, and geographic distribution of GCA...
April 23, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28498830/the-influence-of-the-great-east-japan-earthquake-on-microscopic-polyangiitis-a-retrospective-observational-study
#13
Yoichi Takeuchi, Ayako Saito, Yoshie Ojima, Saeko Kagaya, Hirotaka Fukami, Hiroyuki Sato, Ken Matsuda, Tasuku Nagasawa
BACKGROUND: Antineutrophil cytoplasmic antibody-associated vasculitis is triggered by environmental factors, including silica dust exposure. Repeated tsunami waves brought a large volume of silica-containing sludge inland after the Great East Japan earthquake in 2011. We aimed to determine if the serious disaster influenced the clinical features of the microscopic polyangiitis. METHODS: This is an observational retrospective study conducted in a single institute...
2017: PloS One
https://www.readbyqxmd.com/read/28497220/ct-p10-truxima%C3%A2-a-rituximab-biosimilar
#14
Emma D Deeks
CT-P10 (Truxima™) is the first biosimilar of the reference monoclonal anti-CD20 antibody rituximab. It is approved for use in all indications for which reference rituximab is approved, including follicular lymphoma (FL), diffuse large B-cell non-Hodgkin's lymphoma, chronic lymphocytic leukaemia, rheumatoid arthritis (RA), granulomatosis with polyangiitis and microscopic polyangiitis. CT-P10 has similar physicochemical and pharmacodynamic properties to those of reference rituximab, and the pharmacokinetic biosimilarity of the agents has been shown in patients with RA or FL...
May 11, 2017: BioDrugs: Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy
https://www.readbyqxmd.com/read/28496445/eosinophils-in-autoimmune-diseases
#15
REVIEW
Nicola L Diny, Noel R Rose, Daniela Čiháková
Eosinophils are multifunctional granulocytes that contribute to initiation and modulation of inflammation. Their role in asthma and parasitic infections has long been recognized. Growing evidence now reveals a role for eosinophils in autoimmune diseases. In this review, we summarize the function of eosinophils in inflammatory bowel diseases, neuromyelitis optica, bullous pemphigoid, autoimmune myocarditis, primary biliary cirrhosis, eosinophilic granulomatosis with polyangiitis, and other autoimmune diseases...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28495524/granulomatosis-with-polyangiitis-wegener-granulomatosis-a-proteinase-3-driven-disease
#16
Véronique Witko-Sarsat, Nathalie Thieblemont
Granulomatosis with polyangiitis (GPA, Wegener granulomatosis) is a systemic autoimmune vasculitis that affects small arteries, arterioles, and capillaries, most notably in the kidneys and lungs. In this disease, proteinase 3 (PR3), produced by neutrophils, is targeted by antineutrophil cytoplasmic antibodies (ANCA). Recent work by our group has shown how PR3 impairs the resolution of inflammation and deregulates the immune system. Normally, the clearance of activated neutrophils triggers an anti-inflammatory, pro-resolution process...
May 8, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28493095/can-a-saddle-nose-deformity-of-granulomatosis-with-polyangiitis-be-repaired
#17
Osamu Ito, Tomoyuki Yano, Minako Ito
No abstract text is available yet for this article.
May 10, 2017: Aesthetic Plastic Surgery
https://www.readbyqxmd.com/read/28488228/pneumocystis-pneumonia-and-the-rheumatologist-which-patients-are-at-risk-and-how-can-pcp-be-prevented
#18
REVIEW
Rachel M Wolfe, James E Peacock
PURPOSE OF REVIEW: Immunosuppressive therapy for connective tissue diseases (CTDs) is steadily becoming more intense. The resultant impairment in cell-mediated immunity has been accompanied by an increasing risk for opportunistic infection (OI). Pneumocystis pneumonia (PCP) has been recognized as an OI in patients with CTDs, but specific risk factors and precise indications for PCP prophylaxis remain poorly defined. This review was undertaken to update information on the risk of PCP in patients with CTDs and to examine current guidelines for PCP prophylaxis in this population...
June 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28487300/granulomatosis-with-polyangiitis-seeing-the-diagnosis
#19
Max J Blumberg, Cynthia I Tung, Lindsay A May, Sangita P Patel
A 41-year-old woman presented to her primary doctor with nausea, back pain and lower extremity oedema. Initial labs showed elevated serum creatinine and white blood cell count (WBC), which her doctor attributed to ibuprofen use and a recent upper respiratory infection. Five days later, she presented to the eye clinic with eye pain, redness and blurred vision. She was diagnosed with iritis, conjunctivitis and keratitis. The inflammatory eye disease with decreased renal function prompted the ophthalmologist to initiate systemic autoimmune and infectious disease work-up...
May 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28479484/interstital-lung-disease-in-anca-vasculitis
#20
REVIEW
Marco A Alba, Luis Felipe Flores-Suárez, Ashley G Henderson, Hong Xiao, Peiqi Hu, Patrick H Nachman, Ronald J Falk, J Charles Jennette
Anti-neutrophil cytoplasmic antibodies (ANCA) vasculitides are immune-mediated disorders that primarily affect small blood vessels of the airway and kidneys. Lung involvement, one of the hallmarks of microscopic polyangiitis and granulomatosis with polyangiitis, is associated with increased mortality and morbidity. In recent years, several retrospective series and case reports have described the association of interstitial lung disease (ILD) and ANCA vasculitis, particularly those positive for ANCA specific for myeloperoxidase...
May 4, 2017: Autoimmunity Reviews
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