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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
M Yates, R A Watts, I M Bajema, M C Cid, B Crestani, T Hauser, B Hellmich, J U Holle, M Laudien, M A Little, R A Luqmani, A Mahr, P A Merkel, J Mills, J Mooney, M Segelmark, V Tesar, K Westman, A Vaglio, N Yalçındağ, D R Jayne, C Mukhtyar
In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate...
September 2016: Annals of the Rheumatic Diseases
Ágnes Haris, Szilveszter Dolgos, Kálmán Polner
This paper reviews the recently published scientific information regarding ANCA-associated vasculitis (AAV), aiming to highlight the most important data from the clinical nephrologists' perspective. The classification, pathomechanism, recent achievements of the treatment, short-term and long-term outcomes of the disease, and the difficulties nephrologists face when taking care for patients with AAV are summarized. There has been significant progress in the understanding of the genetic and pathologic background of the disease in the last years, and results of histological studies guide us to predict long-term renal function...
September 26, 2016: International Urology and Nephrology
Vincent Cottin, Elisabeth Bel, Paolo Bottero, Klaus Dalhoff, Marc Humbert, Romain Lazor, Renato A Sinico, Pasupathy Sivasothy, Michael E Wechsler, Matthieu Groh, Sylvain Marchand-Adam, Chahéra Khouatra, Benoit Wallaert, Camille Taillé, Philippe Delaval, Jacques Cadranel, Philippe Bonniaud, Grégoire Prévot, Sandrine Hirschi, Anne Gondouin, Bertrand Dunogué, Gérard Chatté, Christophe Briault, Christian Pagnoux, David Jayne, Loïc Guillevin, Jean-François Cordier
OBJECTIVE: To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. METHODS: Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. RESULTS: The study population included 157 patients (mean age 49...
September 23, 2016: Autoimmunity Reviews
Hong Xiao, Peiqi Hu, Ronald J Falk, J Charles Jennette
BACKGROUND: Antineutrophil cytoplasmic autoantibodies (ANCA) are associated with a spectrum of necrotizing vasculitis including granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited necrotizing and crescentic glomerulonephritis. Clinical observations and in vitro and in vivo experimental evidence strongly indicate that ANCA are pathogenic. SUMMARY: The etiology and pathogenesis of ANCA-associated vasculitis (AAV) are multifactorial, with contributions from genetic factors, environmental exposures, infections, characteristics of the innate and adaptive immune system, and the intensity and duration of the injury...
March 2016: Kidney Diseases
Shivani Shah, Duvuru Geetha
Granulomatosis with polyangiitis and microscopic polyangiitis are small vessel vasculitides characterized by circulating antineutrophil circulating antibodies. Standard treatment for active severe disease has consisted of cyclophosphamide with glucocorticoids with or without plasmapheresis, which achieves approximately 75% sustained remission, but carries significant adverse effects such as malignancy, infertility, leukopenia, and infections. The role of B cells in the pathogenesis of anti-neutrophil circulating antibodies-associated vasculitis has been established, and as such, rituximab, a monoclonal anti-CD20 antibody, has been studied in treatment of active granulomatosis with polyangiitis and microscopic polyangiitis (induction) and in maintaining remission...
2015: ImmunoTargets and Therapy
Jan Damoiseaux, Elena Csernok, Niels Rasmussen, Frank Moosig, Pieter van Paassen, Bo Baslund, Pieter Vermeersch, Daniel Blockmans, Jan-Willem Cohen Tervaert, Xavier Bossuyt
OBJECTIVE: This multicentre study was performed to evaluate the diagnostic accuracy of a wide spectrum of novel technologies nowadays available for detection of myeloperoxidase (MPO) and proteinase 3 (PR3)-antineutrophil cytoplasmic antibodies (ANCAs). METHODS: Sera (obtained at the time of diagnosis) from 251 patients with ANCA-associated vasculitis (AAV), including granulomatosis with polyangiitis and microscopic polyangiitis, and from 924 disease controls were tested for the presence of cytoplasmic pattern/perinuclear pattern and atypical ANCA (A-ANCA) by indirect immunofluorescence (IIF) (at two sites) and for the presence of PR3-ANCA and MPO-ANCA by eight different immunoassays...
August 1, 2016: Annals of the Rheumatic Diseases
Maria Weiner, Mårten Segelmark
Anti-neutrophil cytoplasmic antibodies (ANCA) are a family of autoantibodies that react with proteins predominantly expressed in cytoplasmic granules of polymorphonuclear neutrophil granulocytes (PMNs). ANCA was initially detected using indirect immunofluorescence, allowing for different patterns such as p-ANCA (perinuclear) and c-ANCA (cytoplasmic) to be distinguished. Today it is common to detect the antibodies by immunochemical assays such as ELISA using purified proteins as antigens. The strongest association with ANCA is found in the pauci-immune small vessel vasculitides granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA)...
October 2016: Autoimmunity Reviews
Divi Cornec, Emilie Cornec-Le Gall, Fernando C Fervenza, Ulrich Specks
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a heterogeneous group of rare syndromes characterized by necrotizing inflammation of small and medium-sized blood vessels and the presence of ANCAs. Several clinicopathological classification systems exist that aim to define homogeneous groups among patients with AAV, the main syndromes being microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Two main types of ANCA can be detected in patients with AAV...
October 2016: Nature Reviews. Rheumatology
Doubravka Frausová, Zdenka Hrušková, Věra Lánská, Jana Lachmanová, Vladimír Tesař
BACKGROUND: Plasma exchange (PLEX) has been used routinely for treatment of severe renal vasculitis and/or alveolar haemorrhage (AH) in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but the long-term benefit of PLEX in AAV remains unclear. We aimed to describe the characteristics and outcomes of patients treated with PLEX in a single centre. METHODS: Patients with AAV were identified by performing a case review of medical records of 705 patients who received PLEX in a single tertiary referral centre between 2000 and 2010...
2016: Arthritis Research & Therapy
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
B A McNicholas, T P Griffin, S Donnellan, L Ryan, A Garrahy, R Coughlan, L Giblin, D Lappin, D Reddan, J J Carey, M D Griffin
BACKGROUND: Anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitis (AAV) is a disease characterized by inflammation of small vessels and detectable ANCA in the circulation. Patients may develop a broad spectrum of clinical features ranging from indolent sino-nasal disease and rashes to fulminant renal failure or acute life-threatening pulmonary haemorrhage. Consequently, patients with AAV present to a variety of specialties including nephrology and rheumatology, whose training and approaches to management of such patients may differ...
June 17, 2016: QJM: Monthly Journal of the Association of Physicians
Giles Walters, Narelle S Willis, Jonathan C Craig
BACKGROUND: Renal vasculitis presents as rapidly progressive glomerulonephritis which comprises of a group of conditions characterised by acute kidney injury (AKI), haematuria and proteinuria. Treatment of these conditions comprises steroid and non-steroid agents in combination with plasma exchange. Although immunosuppression overall has been very successful in treatment of these conditions, many questions remain unanswered in terms of dose and duration of therapy, the use of plasma exchange and the role of new therapies...
2015: Cochrane Database of Systematic Reviews
Matthew J Koster, Kenneth J Warrington
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are near universally fatal conditions if untreated. Although effective therapeutic options are available for these diseases, treatment regimens are associated with both short- and long-term adverse effects. The recent identification of effective B-cell-targeted therapy with an anti-CD20 monoclonal antibody has transformed the treatment landscape of AAV. Questions, nevertheless, remain regarding the appropriate timing, dose, frequency, duration, and long-term effects of treatment...
2016: F1000Research
Jan H Schirmer, Marvin N Wright, Kristine Herrmann, Martin Laudien, Bernhard Nölle, Eva Reinhold-Keller, Jan P Bremer, Frank Moosig, Julia U Holle
Objectives Comparing phenotype, clinical course and outcome of Myeloperoxidase(MPO)-ANCA associated Granulomatosis with polyangiitis (GPA) with GPA associated with Proteinase 3(PR3)-ANCA and with Microscopic Polyangiitis (MPA) associated with MPO-ANCA. Methods We characterized all MPO-ANCA-positive patients classified as GPA by the European Medicines Agency algorithm who attended our center in a retrospective chart review. A second cohort with GPA(PR3-ANCA) patients matched for age and gender was characterized...
June 22, 2016: Arthritis & Rheumatology
Jan-Stephan F Sanders, Anoek A E de Joode, Ruud G DeSevaux, Jan Broekroelofs, Alexandre E Voskuyl, Pieter van Paassen, Cees G M Kallenberg, Jan Willem Cohen Tervaert, Coen A Stegeman
BACKGROUND: Cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) positivity at remission has been associated with an increased relapse rate in patients with proteinase 3 anti-neutrophil cytoplasmic antibody-associated vasculitis (PR3-AAV) after a switch to azathioprine maintenance therapy. We therefore hypothesized that extended azathioprine maintenance therapy could reduce the incidence of relapse in this setting. METHODS: Patients newly diagnosed with PR3-AAV at 12 centres in The Netherlands during 2003-11 who received a standardized induction regimen consisting of oral cyclophosphamide and corticosteroids were enrolled (n = 131)...
September 2016: Nephrology, Dialysis, Transplantation
Yasuaki Harabuchi, Kan Kishibe, Kaori Tateyama, Yuka Morita, Naohiro Yoshida, Yasuomi Kunimoto, Takamichi Matsui, Hiroshi Sakaguchi, Masahiro Okada, Takeshi Watanabe, Akira Inagaki, Shigeto Kobayashi, Yukiko Iino, Shingo Murakami, Haruo Takahashi, Tetsuya Tono
OBJECTIVE: We aimed to analyze clinical features and treatment outcomes of otitis media caused by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), i.e. otitis media with AAV (OMAAV). METHODS: This survey was performed between December 2013 and February 2014. The study began with a preliminary survey to 123 otolaryngology institutions in Japan to inquire about their experiences with OMAAV patients during the past 10 years, and was followed by a questionnaire survey to investigate clinical and laboratory findings...
May 11, 2016: Modern Rheumatology
J Antonio Aviña-Zubieta, Alice Mai, Neda Amiri, Natasha Dehghan, Ju Ann Tan, Eric C Sayre, Hyon K Choi
OBJECTIVE: To assess the relative risk of myocardial infarction (MI) and ischemic stroke in cases with newly diagnosed granulomatosis with polyangiitis (GPA) compared to controls from the general population. METHODS: Using a population-based database from the province of British Columbia, Canada, we conducted a matched cohort study of all patients with incident GPA with up to ten age-, sex-, and entry time-matched individuals from the general population. GPA cases required the use of oral glucocorticoids, methotrexate, cyclophosphamide, leflunomide, azathioprine, cyclosporine, mycophenolate mofetil or rituximab within one-month before or six-months after the index date...
May 23, 2016: Arthritis & Rheumatology
Rodrigo Cartin-Ceba, Luis Diaz-Caballero, Mazen O Al-Qadi, Stavros Tryfon, Fernando C Fervenza, Steven R Ytterberg, Ulrich Specks
OBJECTIVE: To identify predictors of respiratory failure and to evaluate the therapeutic efficacy of plasma exchange (PE) and of rituximab versus cyclophosphamide in a cohort of patients with diffuse alveolar hemorrhage (DAH) secondary to antineutrophil cytoplasmic antibody-associated vasculitis (AAV) with or without respiratory failure. METHODS: We performed a single-center historical cohort study of all consecutive patients with AAV-associated DAH who were evaluated over a 16-year period...
June 2016: Arthritis & Rheumatology
Giacomo Maria Guidelli, Sara Tenti, Nicola Antonio Pascarelli, Mauro Galeazzi, Antonella Fioravanti
Granulomatosis with polyangiitis, formerly known as Wegener's granulomatosis or disease, is a systemic, necrotizing small-vessel vasculitis, belonging to the group of anti-neutrophil cytoplasm antibody vasculitis. The therapeutic strategy includes, in most cases, corticosteroids associated, at least in severe forms of the disease, with immunosuppressive agents: cyclophosphamide and rituximab to induce remission, methotrexate, azathioprine and mycophenolate mofetil to prevent relapses. Intravenous immunoglobulins represent an alternative adjuvant therapy...
August 2015: Autoimmunity Reviews
2016-04-08 20:33:54
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