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"Microscopic polyangiitis"

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https://www.readbyqxmd.com/read/29142188/a-case-of-microscopic-polyangiitis-with-subarachnoid-hemorrhage-and-cardiovascular-complications
#1
Sae Aratani, Yukinao Sakai, Shuichi Tsuruoka
Microscopic polyangiitis (MPA) is a primary systemic vasculitis that predominantly affects small and medium vessels. MPA is rarely complicated with central nervous system or cardiovascular disease. We report a very rare case of MPA complicated with cerebral infarction, cardiovascular disease, and fatal subarachnoid hemorrhage in a 54-year-old man. During the first six days of hospitalization the patient was diagnosed with rapid progressive glomerulonephritis (RPGN), cerebral infarction, and unstable angina...
2017: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
https://www.readbyqxmd.com/read/29124398/patient-perceptions-of-glucocorticoids-in-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#2
Joanna C Robson, Jill Dawson, Peter F Cronholm, Susan Ashdown, Ebony Easley, Katherine S Kellom, Don Gebhart, Georgia Lanier, Nataliya Milman, Jacqueline Peck, Raashid A Luqmani, Judy A Shea, Gunnar Tomasson, Peter A Merkel
Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are multisystem diseases of small blood vessels, collectively known as the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). This study explores the patient's perspective on the use of glucocorticoids, which are still a mainstay of treatment in AAV. Patients with AAV from the UK, USA, and Canada were interviewed, using purposive sampling to include a range of disease manifestations and demographics...
November 9, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29119480/rheumatoid-factor-false-positivity-in-patients-with-anca-associated-vasculitis-not-having-medical-conditions-producing-rheumatoid-factor
#3
Jae-Seung Moon, Diane Da-Hyun Lee, Yong-Beom Park, Sang-Won Lee
We investigated the rate of rheumatoid factor (RF) false positivity at diagnosis and its influence on clinical and prognostic features and rheumatoid arthritis (RA) development during the follow-up in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients without RA or other medical conditions triggering RF false positivity. We reviewed the medical records of 128 AAV patients. We divided patients with AAV and each variant into two groups according to RF positivity and compared variables between the two groups...
November 8, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29082297/distinct-pathogenesis-in-nonsystemic-vasculitic-neuropathy-and-microscopic-polyangiitis
#4
Mie Takahashi, Haruki Koike, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Atsushi Hashizume, Masahisa Katsuno, Gen Sobue
OBJECTIVE: To investigate the mechanisms of vasculitis in nonsystemic vasculitic neuropathy (NSVN) and microscopic polyangiitis (MPA), focusing on complement- and antineutrophil cytoplasmic antibody (ANCA)-associated pathogenesis. METHODS: Sural nerve biopsy specimens taken from twenty-four patients with NSVN and 37 with MPA-associated neuropathy (MPAN) were examined. Twenty-two patients in the MPAN group tested positive for ANCA. RESULTS: Immunostaining for complement component C3d deposition showed more frequent positive staining of epineurial small vessels in NSVN than in MPAN (p = 0...
November 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29061243/central-nervous-system-disease-in-antineutrophil-cytoplasmic-antibodies-associated-vasculitis
#5
REVIEW
Jonathan Graf
Central nervous system (CNS) disease is an uncommon but significant complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and affects 3 primary areas of the CNS: the pituitary, the pachymeninges, and the CNS vasculature. Pituitary disease in uncommon, but hormonal deficiencies can be long lasting even in the face of excellent disease response. Chronic hypertrophic pachymeninigitis occurs in anti-proteinase 3-positive patients with systemic GPA and in anti-myeloperoxidase-positive patients with a milder and more limited form of the disease...
November 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/29016646/methotrexate-versus-cyclophosphamide-for-remission-maintenance-in-anca-associated-vasculitis-a-randomised-trial
#6
Federica Maritati, Federico Alberici, Elena Oliva, Maria L Urban, Alessandra Palmisano, Francesca Santarsia, Simeone Andrulli, Laura Pavone, Alberto Pesci, Chiara Grasselli, Rosaria Santi, Bruno Tumiati, Lucio Manenti, Carlo Buzio, Augusto Vaglio
OBJECTIVES: The treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is based on remission-induction and remission-maintenance. Methotrexate is a widely used immunosuppressant but only a few studies explored its role for maintenance in AAV. This trial investigated the efficacy and safety of methotrexate as maintenance therapy for AAV. METHODS: In this single-centre, open-label, randomised trial we compared methotrexate and cyclophosphamide for maintenance in AAV...
2017: PloS One
https://www.readbyqxmd.com/read/28988280/echocardiographic-features-in-patients-with-anca-associated-vasculitis-within-3%C3%A2-months-before-and-after-diagnosis
#7
Sung Soo Ahn, Eun Seong Park, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
We investigated the all-inclusive echocardiographic features in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) within 3 months before and after diagnosis. We reviewed the medical records of 89 AAV patients taking echocardiography and 35 age- and gender-matched controls. We collected clinical and laboratory data and echocardiographic results. We compared the variables between patients with AAV of each variant and controls and among those with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA)...
December 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28983940/outline-of-guidelines-for-the-management-of-vasculitis-and-vascular-disorders-in-japan-2016-revised-edition
#8
Takaharu Ikeda, Fukumi Furukawa, Tamihiro Kawakami, Naoko Ishiguro, Miwa Uzuki, Shoichi Ozaki, Kensei Katsuoka, Takeshi Kono, Seiji Kawana, Masanari Kodera, Takashi Sawai, Yasuyuki Sawada, Mariko Seishima, Akiko Tanikawa, Ko-Ron Chen, Minoru Hasegawa
The proposal by the 1994 International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC1994) and by the CHCC2012 markedly influenced the classification and way of considering cutaneous vasculitis. In the proposal by the CHCC1994, hypersensitivity angiitis was defined as an equivalent pathological condition to microscopic polyangiitis or cutaneous leukocytoclastic angiitis (CLA), and it was not adopted as a disease name. However, CLA which was positioned as a type of small-vessel vasculitis is only a pathological name...
October 6, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28977502/long-term-azathioprine-maintenance-therapy-in-anca-associated-vasculitis-combined-results-of-long-term-follow-up-data
#9
Anoek A E de Joode, Jan Stephan F Sanders, Xavier Puéchal, Loic P Guillevin, Thomas F Hiemstra, Oliver Flossmann, Nils Rasmussen, Kerstin Westman, David R Jayne, Coen A Stegeman
Objective: We studied whether in ANCA-associated vasculitis patients, duration of AZA maintenance influenced relapse rate during long-term follow-up. Methods: Three hundred and eighty newly diagnosed ANCA-associated vasculitis patients from six European multicentre studies treated with AZA maintenance were included; 58% were male, median age at diagnosis 59.4 years (interquartile range: 48.3-68.2 years); granulomatosis with polyangiitis, n = 236; microscopic polyangiitis, n = 132; or renal limited vasculitis, n = 12...
November 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28955487/validation-of-the-eular-era-edta-recommendations-for-the-management-of-anca-associated-vasculitis-by-disease-content-experts
#10
Max Yates, Richard Watts, Ingeborg Bajema, Maria Cid, Bruno Crestani, Thomas Hauser, Bernhard Hellmich, Julia Holle, Martin Laudien, Mark A Little, Raashid Ahmed Luqmani, Alfred Mahr, Peter Merkel, John Mills, Janice Mooney, Mårten Segelmark, Vladimir Tesar, Kerstin W A Westman, Augusto Vaglio, Nilüfer Yalçındağ, David R Jayne, Chetan Mukhtyar
The European League Against Rheumatism recommendations for the management of antineutrophil cytoplasmic antibody-associated vasculitis have been recently published. Unique to recommendation development, they were also voted on by members of a learned society. This paper explores the wider validity of the recommendations among people who self-identify as clinicians caring for patients with vasculitis. In addition to the task force, a learned society (European Vasculitis Society-EUVAS) was invited, through online survey, to rate independently the strength of evidence of each recommendation to obtain an indication of the agreement among the final target audience and ultimate end-users of the recommendations...
2017: RMD Open
https://www.readbyqxmd.com/read/28943556/two-cases-of-central-diabetes-insipidus-in-refractory-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#11
Keiji Ohashi, Michiko Morishita, Haruki Watanabe, Ken-Ei Sada, Takayuki Katsuyama, Yoshia Miyawaki, Eri Katsuyama, Mariko Narazaki, Noriko Tatebe, Katsue Watanabe, Tomoko Kawabata, Jun Wada
We herein describe two cases of refractory antineutrophil cytoplasmic antibody-associated vasculitis (AAV) complicated with diabetes insipidus (DI) possibly related to hypertrophic pachymeningitis (HP). One patient had microscopic polyangiitis and HP, which were refractory to cyclophosphamide, azathioprine, rituximab, mycophenolate mofetil (MMF), and mizoribine. Remission was finally achieved with the use of etanercept, but DI occurred 5 years later. The other patient had granulomatosis with polyangiitis, which that was refractory to cyclophosphamide, methotrexate, MMF, and rituximab...
September 25, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28921160/gp2013-a-rituximab-biosimilar
#12
Hannah A Blair
GP2013 is the second biosimilar of the reference monoclonal anti-CD20 antibody rituximab to be approved in the EU. 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. GP2013 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...
October 2017: BioDrugs: Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy
https://www.readbyqxmd.com/read/28916432/rituximab-as-induction-therapy-in-eosinophilic-granulomatosis-with-polyangiitis-refractory-to-conventional-immunosuppressive-treatment-a-36-month-follow-up-analysis
#13
Jens Thiel, Arianna Troilo, Ulrich Salzer, Theresa Schleyer, Kirsten Halmschlag, Marta Rizzi, Natalie Frede, Ana Venhoff, Reinhard E Voll, Nils Venhoff
BACKGROUND: Rituximab (RTX) is approved for induction therapy of granulomatosis with polyangiitis and microscopic polyangiitis. In eosinophilic granulomatosis with polyangiitis (EGPA), organ-threatening manifestations are mainly treated with cyclophosphamide (CYC). RTX as treatment in EGPA has been described in small case series; however long-term data and the efficacy of RTX in EGPA refractory to CYC have not been reported yet. OBJECTIVES: To investigate the efficacy and safety of RTX and conventional immunosuppressive therapy with CYC in EGPA as induction therapy and during long-term follow-up...
September 12, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28905856/position-paper-revised-2017-international-consensus-on-testing-of-ancas-in-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#14
REVIEW
Xavier Bossuyt, Jan-Willem Cohen Tervaert, Yoshihiro Arimura, Daniel Blockmans, Luis Felipe Flores-Suárez, Loïc Guillevin, Bernhard Hellmich, David Jayne, J Charles Jennette, Cees G M Kallenberg, Sergey Moiseev, Pavel Novikov, Antonella Radice, Judith Anne Savige, Renato Alberto Sinico, Ulrich Specks, Pieter van Paassen, Ming-Hui Zhao, Niels Rasmussen, Jan Damoiseaux, Elena Csernok
Anti-neutrophil cytoplasmic antibodies (ANCAs) are valuable laboratory markers used for the diagnosis of well-defined types of small-vessel vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). According to the 1999 international consensus on ANCA testing, indirect immunofluorescence (IIF) should be used to screen for ANCAs, and samples containing ANCAs should then be tested by immunoassays for proteinase 3 (PR3)-ANCAs and myeloperoxidase (MPO)-ANCAs. The distinction between PR3-ANCAs and MPO-ANCAs has important clinical and pathogenic implications...
November 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28895041/unmet-needs-in-the-pathogenesis-and-treatment-of-vasculitides
#15
REVIEW
Francesco Muratore, Giulia Pazzola, Alessandra Soriano, Nicolò Pipitone, Stefania Croci, Martina Bonacini, Luigi Boiardi, Carlo Salvarani
Despite the progress in the last years on the field of vasculitides, there are several unmet needs regarding classification, disease activity assessment, predictors of flares and complications, and type of treatment for the different forms. The 1990 American College of Rheumatology (ACR) classification criteria currently used to define giant cell arteritis and Takayasu arteritis were designed to discriminate between different types of vasculitides but not to differentiate vasculitis from other disorders. Recently, efforts have been made to overcome the shortcomings of the ACR criteria...
September 11, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28881714/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-years-observation-study
#16
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...
August 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28881446/the-epidemiology-of-antineutrophil-cytoplasmic-autoantibody-associated-vasculitis-in-olmsted-county-minnesota-a-twenty-year-us-population-based-study
#17
Alvise Berti, Divi Cornec, Cynthia S Crowson, Ulrich Specks, Eric L Matteson
OBJECTIVE: To estimate the annual incidence, prevalence, and mortality of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (Wegener's) (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), in a US-based adult population. METHODS: All medical records of patients with a diagnosis of, or suspicion of having, AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed...
September 7, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28880680/the-effect-and-possible-clinical-efficacy-of-in-vivo-inhibition-of-neutrophil-extracellular-traps-by-blockade-of-pi3k-gamma-on-the-pathogenesis-of-microscopic-polyangiitis
#18
Hirotaka Kimura, Yasushi Matsuyama, Sachiko Araki, Atsushi Koizumi, Yumi Kariya, Shunsuke Takasuga, Satoshi Eguchi, Hiroki Nakanishi, Junko Sasaki, Takehiko Sasaki
OBJECTIVE: Neutrophil extracellular traps (NETs) are peculiar structures composed of the externalized chromatin with intracellular proteins and formed by activated neutrophils in a reactive oxygen species (ROS)-dependent manner. Aberrant NETs are considered to be autoantigens for anti-neutrophil cytoplasmic antibodies (ANCAs) underling the development of microscopic polyangiitis (MPA). However, little is known regarding the therapeutic efficacy of in vivo inhibition of NET formation (NETosis) on MPA pathogenesis...
September 7, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28863794/immunoadsorption-in-autoimmune-diseases-affecting-the-kidney
#19
REVIEW
Georg Stummvoll, Martin Aringer, Ammon Handisurya, Kurt Derfler
Autoantibodies play an important role in the pathophysiology of renal involvement in systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), systemic vasculitis, and anti-glomerular basement membrane disease (or Goodpasture syndrome). Direct removal of autoantibodies therefore has been tried in various ways, first by plasma exchange. Today, immunoadsorption is the extracorporeal method that most effectively removes (pathogenic) immune complexes and antibodies. Although past data have shown efficacy and biocompatibility of immunoadsorption in (renal) SLE, it is still an experimental and expensive procedure, and evidence from randomized controlled trials is needed...
September 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28852465/gallbladder-bleeding-associated-with-microscopic-polyangiitis-a-case-report
#20
Daichi Kitaguchi, Masanao Kurata, Osamu Shimomura, Tatsuya Oda, Nobuhiro Ohkohchi
A 71-year-old male who had a 6 years history of microscopic polyangiitis (MPA) was admitted to our hospital with a chief complaint of upper abdominal pain and nausea. Abdominal contrast-enhanced CT revealed extravasation of contrast medium in the gallbladder, and the patient was diagnosed with gallbladder bleeding. Although we started conservative treatment, anemia, hypotension and tachycardia had progressed gradually. Therefore, we performed emergent laparoscopic cholecystectomy on the following day. Intraoperative findings showed a remarkably distended gallbladder due to interior clots; however, the cholecystitis itself was not significant...
August 2017: Journal of Surgical Case Reports
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