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microscopic polyangiitis

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https://www.readbyqxmd.com/read/28623488/vasculitis-for-the-internist-focus-on-anca-associated-vasculitis
#1
REVIEW
Benjamin Chaigne, Loïc Guillevin
Vasculitides comprise several diseases affecting vessels of different sizes, mainly arteries, but also capillaries and veins. Antineutrophil cytoplasm antibody-associated vasculitides (AAVs) belong to the systemic necrotizing small-vessel vasculitis group that comprises granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Internists often see AAV patients, and although their outcomes are usually good, the disease can be responsible for morbidity and mortality...
June 16, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28616269/elevated-serum-levels-of-immunoglobulin-a-correlate-with-the-possibility-of-readmission-in-patients-with-microscopic-polyangiitis
#2
Huijuan Wang, Chao Zhang, Zhaohui Tong, Xiaoning Bu
BACKGROUND: The evidence for the short-term prognosis of patients with microscopic polyangiitis (MPA) is weak, and the objective of this study was to analyze the clinical features of the disease and evaluate the risk factors for readmission in patients with MPA. METHODS: Fifty-seven patients with MPA were recruited into this study. The clinical data of these MPA patients were collected. Clinical manifestations, laboratory results, and imaging results were analyzed...
May 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28611282/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-year-observation-study
#3
Dario Roccatello, Savino Sciascia, Daniela Rossi, Mirella Alpa, Carla Naretto, Massimo Radin, Roberta Fenoglio, Simone Baldovino, Elisa Menegatti
BACKGROUND: ANCA associated vasculitides (AAV) often present with a chronic relapsing course. Relapse leads to increased immunosuppressive exposure and consequent toxicity. While two randomized controlled trials have shown rituximab (RTX) to be the most effective induction treatment in patients with relapsing disease, the optimal treatment duration and RTX dose remain debated. Whether to administer a maintenance dose to every patient, at a fixed time interval or on the basis of B cell count and ANCA titre or only when disease manifestations do occur is still debated as well...
May 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28591497/-anca-associated-vasculitides-at-mexico-city-s-metropolitan-eastern-area
#4
Hernando Cervera-Castillo, Francisco Blanco-Favela, Yolanda Floriberta Silva-López, Guadalupe Eugenia Paredez-Rivera, Verónica Torres-Caballero
BACKGROUND: The anti-neutrophil cytoplasmic antibody-associated (ANCA) vasculitides includes granulomatosis with polyangiitis (Wegener's) (GPW), Chrug-Strauss syndrome (CSS) and microscopic polyangiitis (MPA). Since it has low incidence in our field, there are a few published papers. The aim of this study was to report the clinical characteristics, activity and damage of these vasculitides in Mexico City's metropolitan eastern area. METHODS: We studied 28 cases with ANCA-associated vasculitis in the aforementioned area...
July 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28578472/cutaneous-manifestations-of-anca-associated-small-vessels-vasculitis
#5
REVIEW
Marzano Angelo Valerio, Raimondo Maria Gabriella, Berti Emilio, Meroni Pier Luigi, Ingegnoli Francesca
Skin lesions are frequent manifestations of underlying systemic conditions, including systemic autoimmune vasculitis. In particular, anti-neutrophil cytoplasmic antibodies (ANCA) are associated with distinct forms of vasculitis characterized by inflammatory cell infiltration of the walls of small and medium-sized vessels leading to vascular destruction and tissue necrosis. ANCA-associated vasculitis is rare and systemic diseases, which can be classified based on different distribution of vascular inflammation and presence or absence of granulomatosis and asthma...
June 3, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28577200/anca-associated-vasculitis-presenting-as-severe-pulmonary-hypertension-and-right-heart-failure
#6
Rakesh Kumar Pilania, Sumeet R Dhawan, Joseph L Mathew, Surjit Singh, K S Sodhi, Meenu Singh
Microscopic polyangiitis (MPA) is a small vessel multisystemic disorder characterised by necrotising small vessel vasculitis without any immune deposits. Kidney and lung are the predominant organs affected in MPA. Skin, gastrointestinal and neurological findings are also described. Isolated pulmonary manifestations are rare. The authors describe a two-year girl who presented with right heart failure and was subsequently diagnosed as ANCA-associated vasculitis. This case report is intended to sensitise pediatricians to consider systemic vasculitis with pulmonary hemorrhage in children with pulmonary hypertension even in the absence of severe pallor...
June 2, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28575531/myeloperoxidase-hla-class-ii-complexes-recognized-by-autoantibodies-in-microscopic-polyangiitis
#7
Ryosuke Hiwa, Koichiro Ohmura, Noriko Arase, Hui Jin, Kouyuki Hirayasu, Masako Kohyama, Tadahiro Suenaga, Fumiji Saito, Chikashi Terao, Tatsuya Atsumi, Hirotsugu Iwatani, Tsuneyo Mimori, Hisashi Arase
OBJECTIVE: Autoantibodies against myeloperoxidase (MPO) that are expressed in neutrophils play an important role in the pathogenesis of microscopic polyangiitis (MPA). We recently found that misfolded cellular proteins are transported to the cell surface by HLA class II molecules and are targeted by autoantibodies in rheumatoid arthritis and antiphospholipid syndrome, suggesting that HLA class II molecules play an important role in autoantibody recognition. Here, we demonstrate that MPO is expressed on the cell surface by associating with HLA class II molecules...
June 2, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28569178/prediction-of-response-to-remission-induction-therapy-by-gene-expression-profiling-of-peripheral-blood-in-japanese-patients-with-microscopic-polyangiitis
#8
Akihiro Ishizu, Utano Tomaru, Sakiko Masuda, Ken-Ei Sada, Koichi Amano, Masayoshi Harigai, Yasushi Kawaguchi, Yoshihiro Arimura, Kunihiro Yamagata, Shoichi Ozaki, Hiroaki Dobashi, Sakae Homma, Yasunori Okada, Hitoshi Sugiyama, Joichi Usui, Naotake Tsuboi, Seiichi Matsuo, Hirofumi Makino
BACKGROUND: Microscopic polyangiitis (MPA), which is classified as an anti-neutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis, is one of the most frequent primary vasculitides in Japan. We earlier nominated 16 genes (IRF7, IFIT1, IFIT5, OASL, CLC, GBP-1, PSMB9, HERC5, CCR1, CD36, MS4A4A, BIRC4BP, PLSCR1, DEFA1/DEFA3, DEFA4, and COL9A2) as predictors of response to remission induction therapy against MPA. The aim of this study is to determine the accuracy of prediction using these 16 predictors...
May 31, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28560688/anca-associated-vasculitis-with-renal-involvement
#9
REVIEW
Valentina Binda, Gabriella Moroni, Piergiorgio Messa
Systemic vasculitis is a rare but severe group of diseases characterized by inflammation and necrosis of blood vessels. The size of the vessel affected varies among the different forms of vasculitis and there are three main subgroups: large, medium and small vessel vasculitis. Among small vessel vasculitis, the antineutrophil cytoplasmic antibody (ANCA)-associated forms are of particular importance. This subgroup includes: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener's), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and the form limited to the kidney...
May 30, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/28559644/severe-microscopic-polyangiitis-with-unilateral-vocal-cord-paralysis-as-initial-manifestation
#10
Luis Felipe Flores-Suárez, Marco Antonio Alba, Gabriel Tona
CASE DESCRIPTION: A 16 year-old female who presented with initial ear, nose and throat manifestations who later progressed to severe renal disease, requiring hemodialysis after 11 months of unique laryngeal involvement. CLINICAL FINDINGS: Unilateral vocal cord paralysis without other symptoms or signs, but with positive perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) and anti-myeloperoxidase autoantibodies, followed an unfavorable course months later with rapidly progressive glomerulonephritis...
March 30, 2017: Colombia Médica: CM
https://www.readbyqxmd.com/read/28539536/remission-induction-therapy-with-rituximab-for-microscopic-polyangiitis-a-feasibility-study
#11
Ayako Saito, Yoichi Takeuchi, Saeko Kagaya, Yoshie Ojima, Hirotaka Fukami, Hiroyuki Sato, Ken Matsuda, Tasuku Nagasawa
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is systemic vascular inflammation. Microscopic polyangiitis (MPA) is a major type of AAV in Japan. MPA often affects the kidneys and lungs, leading to death if untreated. Induction therapy (i.e., initial treatment) for MPA has not been optimized, although methylprednisolone and cyclophosphamide are commonly used. Recently, rituximab (RTX) (a monoclonal antibody against the protein CD20) has also been used to treat refractory AAV. RTX at 375 mg/m(2)/week for 4 weeks (i...
2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28537941/pathophysiology-of-anca-associated-vasculitis
#12
Turki Al-Hussain, Maged H Hussein, Walter Conca, Hadeel Al Mana, Mohammed Akhtar
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is characterized as inflammation of small-sized to medium-sized blood vessels and encompasses several clinicopathologic entities including granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited ANCA-associated vasculitis. Over the past several decades, significant progress has been made in understanding the pathophysiology of ANCA-associated vasculitis. Although neutrophils contain a multitude of granular proteins, clinically significant autoantibodies are only recognized against myeloperoxidase and proteinase 3, both of which are present in the azurophilic granules...
July 2017: Advances in Anatomic Pathology
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
#13
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/28509135/renal-limited-vasculitis-with-elevated-levels-of-multiple-antibodies
#14
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/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
#15
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/28498830/the-influence-of-the-great-east-japan-earthquake-on-microscopic-polyangiitis-a-retrospective-observational-study
#16
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
#17
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/28479484/interstital-lung-disease-in-anca-vasculitis
#18
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
https://www.readbyqxmd.com/read/28476172/anca-associated-vasculitis-in-childhood-recent-advances
#19
REVIEW
Marta Calatroni, Elena Oliva, Davide Gianfreda, Gina Gregorini, Marco Allinovi, Giuseppe A Ramirez, Enrica P Bozzolo, Sara Monti, Claudia Bracaglia, Giulia Marucci, Monica Bodria, Renato A Sinico, Federico Pieruzzi, Gabriella Moroni, Serena Pastore, Giacomo Emmi, Pasquale Esposito, Mariagrazia Catanoso, Giancarlo Barbano, Alice Bonanni, Augusto Vaglio
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in childhood but North-American and European cohort studies performed during the last decade have clarified their phenotype, patterns of renal involvement and their prognostic implications, and outcome...
May 5, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28432167/a-tale-of-two-vasculitides-biopsy-proven-giant-cell-arteritis-followed-by-the-independent-development-of-renal-limited-microscopic-polyangiitis
#20
Roman Zuckerman, Mayur Patel, Deborah R Alpert
We describe an 80-year-old woman who presented with headaches, bilateral jaw claudication and visual symptoms. She was diagnosed with giant cell arteritis, which was confirmed by temporal artery biopsy. She was treated with high-dose prednisone followed by a slow taper, with complete resolution of symptoms. Approximately 4 years later, she developed progressively worsening renal function associated with haematuria. Serological workup revealed a high-titre perinuclear antinuclear cytoplasmic antibody and antibodies to myeloperoxidase...
April 21, 2017: BMJ Case Reports
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