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Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

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Group of systemic vasculitis with a strong association with ANCA. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.

Also Known As:

ANCA Associated Vasculitides; ANCA Associated Vasculitis; ANCA-Associated Vasculitide; Anti Neutrophil Cytoplasmic Antibody Associated Vasculitis; Pauci Immune Vasculitis; Pauci-Immune Vasculitides; Vasculitide, ANCA-Associated; Vasculitides, ANCA-Associated; Vasculitides, Pauci-Immune; Vasculitis, ANCA-Associated; Vasculitis, Pauci-Immune; ANCA-Associated Vasculitides; ANCA-Associated Vasculitis; Pauci-Immune Vasculitis

Networked: 890 relevant articles (81 outcomes, 113 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Vasculitis (Vasculitides)
2.	Systemic Lupus Erythematosus (Libman-Sacks Disease)
3.	Inflammation
4.	Infection
5.	Microscopic Polyangiitis

Experts

1.	Kallenberg, Cees G M: 37 articles (01/2016 - 11/2004)
2.	Chen, Min: 28 articles (11/2015 - 02/2007)
3.	Specks, Ulrich: 27 articles (07/2015 - 01/2005)
4.	Zhao, Ming-Hui: 24 articles (11/2015 - 10/2005)
5.	Jayne, David R W: 20 articles (07/2015 - 01/2002)
6.	Salama, Alan D: 18 articles (02/2015 - 10/2007)
7.	Pusey, Charles D: 18 articles (09/2014 - 11/2004)
8.	Heeringa, Peter: 17 articles (11/2015 - 01/2004)
9.	Guillevin, Loïc: 17 articles (08/2015 - 10/2006)
10.	Merkel, Peter A: 16 articles (11/2015 - 05/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis:

1.	Antineutrophil Cytoplasmic Antibodies (ANCA)IBA 01/01/2012 - "Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis--a meta-analysis." 12/01/2010 - "Current remission maintenance therapies for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are limited by partial efficacy and toxicity. " 11/01/2006 - "Increased T cell activation in patients with ANCA-associated vasculitis in remission during and after immunosuppressive treatment is associated with persistent or renewed ANCA positivity." 03/01/2003 - "Serial measurements of PR3- and MPO-ANCA titers in patients with ANCA-associated vasculitis during remission can help predict relapses, and preemptive increases in immunosuppression following fourfold titer rises reduces the risk of relapses. " 06/01/2015 - "Recent data from randomized controlled clinical trials have allowed the development of recommendations for treatment of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV). "
2.	rituximab (Mabthera)FDA Link 11/01/2006 - "Rituximab is an effective and well-tolerated treatment for patients with ANCA-associated vasculitis and should be strongly considered in severely affected patients who do not respond to standard therapy or in those in whom cytotoxic therapy bears a high risk of morbidity." 10/01/2011 - "Rituximab (RTX) has been shown to be effective as an induction agent in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV), but studies have been limited by short-term follow-up. " 01/01/2015 - "Rituximab (RTX) was reported effective in ANCA-associated vasculitis (AAV). " 07/01/2015 - "Long-term safety and efficacy of rituximab in 7 Japanese patients with ANCA-associated vasculitis." 05/01/2014 - "To discuss in detail the efficacy and safety of rituximab (RTX) for induction and maintenance of remission in patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) who are either treatment naive, relapsing or refractory to standard of care. "
3.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 04/01/2005 - "In the treatment of ANCA-associated vasculitis therapy with GC, later in combination with cyclophosphamide, lead to a significant reduction of mortality. " 12/01/2012 - "The introduction of (oral) cyclophosphamide (CYC) in the treatment of ANCA-associated vasculitides (AAV) has strongly improved prognosis but the side effects of long-term CYC treatment are serious. " 01/01/2004 - "In this study we used a prolonged course of low-dose intravenous cyclophosphamide for 18-24 months for ANCA-associated vasculitis, evaluated the effectiveness of pCyc and analysed the outcome of a prolonged treatment on the rate of relapse. " 10/01/2012 - "Do adult disease severity subclassifications predict use of cyclophosphamide in children with ANCA-associated vasculitis? " 12/01/2011 - "Since cyclophosphamide was introduced for the treatment of ANCA-associated vasculitis, the mortality of these diseases has decreased considerably. "
4.	Azathioprine (Imuran)FDA LinkGeneric 05/01/2009 - "Thiopurinemethyltransferase (TPMT) genotype and TPMT activity in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis: relation to azathioprine maintenance treatment and adverse effects." 03/15/2006 - "Data from the European Vasculitis Study Group (EUVAS) demonstrate the efficacy of azathioprine in maintaining remission in ANCA-associated vasculitides on the basis of large patient numbers. " 12/01/2013 - "Azathioprine-induced Sweet syndrome in ANCA-associated vasculitis." 11/06/2014 - "More patients with ANCA-associated vasculitides had sustained remission at month 28 with rituximab than with azathioprine. " 01/22/2015 - "Rituximab or azathioprine maintenance in ANCA-associated vasculitis."
5.	Peroxidase (Myeloperoxidase)IBA 01/01/2016 - "This study investigated the clinical course of myeloperoxidase-antineutrophil cytoplasm autoantibody (MPO-ANCA)-associated vasculitis after starting dialysis. " 10/01/2013 - "Clinical trial for Japanese patients with myeloperoxidase anti-neutrophil cytoplasmic antibody-associated vasculitis: the JMAAV study." 12/01/2012 - "Taken together, these studies establish a role for mast cells in mediating peripheral tolerance to myeloperoxidase, protecting them from the development of focal necrotizing GN in ANCA-associated vasculitis." 08/01/2007 - "A meta-analysis of our study and two previous studies showed that there was no association between the myeloperoxidase G-463/A polymorphism and the risk of developing ANCA-associated vasculitis; GG versus GA plus AA (odds ratio 1.14; 95% confidence interval 0.86-1.50). " 03/01/2013 - "Loss of tolerance to neutrophil myeloperoxidase (MPO) underlies the development of ANCA-associated vasculitis and GN, but the mechanisms underlying this loss of tolerance are poorly understood. "
6.	Prednisolone (Predate)FDA LinkGeneric 01/01/2012 - "Four years ago, she suffered recurrence of ANCA-associated vasculitis and with 30 mg/day prednisolone was induced to remission. " 01/01/2012 - "A 42-year-old female who was an asymptomatic carrier of hepatitis B virus (HBV) was diagnosed with antineutrophil cytoplasm antibody- (ANCA-) associated vasculitis and was induced to remission with 30 mg/day prednisolone nine years ago. " 02/01/2000 - "The patients were diagnosed initially as having MPO-ANCA-associated vasculitis and were treated with prednisolone. " 06/01/2009 - "The introduction of cyclophosphamide- and prednisolone-based treatment regimens has significantly improved outcome in patients with anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis. " 11/01/2012 - "Improvement in health-related quality of life in MPO-ANCA-associated vasculitis patients treated with cyclophosphamide plus prednisolone: an analysis of 18 months of follow-up data from the JMAAV study."
7.	Immunosuppressive Agents (Immunosuppressants)IBA 08/01/2015 - "The introduction of biologics for the management of severe ANCA-associated vasculitis and severe, relapsing disease refractory to conventional immunosuppressants, has significantly improved the clinical prognosis of these autoimmune disorders. " 05/01/2014 - "Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) treatment strategy is based on immunosuppressive agents. " 07/01/2009 - "Several immunosuppressive agents can be used for maintenance therapy after induction of remission in patients with ANCA-associated vasculitis, with no firm evidence that one agent is superior to others. " 01/01/2014 - "We report two elderly patients with systemic ANCA-associated vasculitis (AAV) in whom the administration of EPA in concert with steroids safely induced and maintained remission, without the use of additioal immunosuppressants. " 02/01/2000 - "We recommend the aggressive therapy, including prednisolone, immunosuppressive agents and plasma exchange for MPO-ANCA associated vasculitis. "
8.	GlucocorticoidsIBA 06/01/2011 - "The ANCA-associated vasculitides (AAVs) are conventionally treated with a strategy of remission induction followed by maintenance therapy using glucocorticoids combined with CYC during induction and AZA for maintenance. " 11/06/2014 - "The combination of cyclophosphamide and glucocorticoids leads to remission in most patients with antineutrophil cytoplasm antibody (ANCA)-associated vasculitides. " 06/01/2015 - "The introduction of glucocorticoids and cyclophosphamide has transformed ANCA associated vasculitis (AAV) from a fatal to a largely treatable condition. " 01/01/2014 - "The introduction of cyclophosphamide and high-dose glucocorticoids for anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) has allowed a reduction in 1-year mortality from 80% to 10-20%. " 06/01/2013 - "Glucocorticoids plus cyclophosphamide, and, on occasion, plasmapheresis are effective in 70%-90% of patients with ANCA-associated vasculitis (AAV). "
9.	Myeloblastin (Proteinase 3)IBA 10/01/2007 - "Increased numbers of neutrophils expressing proteinase 3 on their membrane (mPR3) have been reported in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and are suggested to be involved in AAV immunopathogenesis. " 01/01/2000 - "T lymphocytes from the peripheral blood of patients with ANCA-associated vasculitis have been shown to proliferate in response to proteinase 3 (PR3). " 08/01/2007 - "Furthermore, patients with MPO-ANCA-associated vasculitis (n = 47) were compared with patients with proteinase 3 (PR3)-ANCA associated vasculitis (n = 46). " 07/19/2012 - "These data provide preliminary support for the concept that proteinase 3 ANCA-associated vasculitis and myeloperoxidase ANCA-associated vasculitis are distinct autoimmune syndromes. " 09/01/2011 - "As the proteinase 3 subtype of antineutrophil cytoplasmic antibodies (ANCA) was positive ANCA-associated vasculitis with gastrointestinal, cutaneous and kidney involvement was diagnosed. "
10.	AutoantibodiesIBA 01/01/2015 - "The discovery of anti-neutrophil cytoplasmic autoantibodies (ANCAs) in the 1980s opened the door for studies that eventually led to the description of a new previously undescribed disease, ANCA-associated vasculitis (AAV). " 04/01/2013 - "Animal models of ANCA associated vasculitis: the contribution of autoantibodies and autoreactive T cells." 03/01/2012 - "We conclude that when robust assays are used, circulating autoantibodies to hLAMP-2 can be detected in most European patients with ANCA-associated vasculitis. " 03/01/2012 - "In untreated patients at presentation, the frequencies of autoantibodies to LAMP-2 were 89%, 91%, and 80%, respectively, among three groups of patients with ANCA-associated vasculitis from Vienna, Austria (n=19); Groningen, the Netherlands (n=50) and Cambridge, United Kingdom (n=53). " 03/01/2012 - "High prevalence of autoantibodies to hLAMP-2 in anti-neutrophil cytoplasmic antibody-associated vasculitis."

Therapies and Procedures

1.	Remission Induction 08/01/2013 - "Efficacy of remission-induction regimens for ANCA-associated vasculitis." 03/01/2014 - "[Remission induction in ANCA-associated vasculitis: Follow-up data of the RAVE study]." 07/01/2009 - "Several immunosuppressive agents can be used for maintenance therapy after induction of remission in patients with ANCA-associated vasculitis, with no firm evidence that one agent is superior to others. " 01/01/2015 - "Is rituximab effective for induction of remission in ANCA-associated vasculitis?" 08/01/2013 - "In anti-neutrophil cytoplasmic antibody-associated vasculitis, rituximab is effective for remission induction and in relapsing disease. "
2.	Plasmapheresis 02/01/2016 - "Role of therapeutic plasmapheresis in ANCA-associated vasculitis." 10/01/2014 - "Plasmapheresis rescue therapy in progressive systemic ANCA-associated vasculitis: single-center results of stepwise escalation of immunosuppression." 01/01/2007 - "This case report suggests that plasmapheresis may be an effective treatment for ANCA-associated vasculitis with diffuse alveolar hemorrhage that is resistant to aggressive immunosuppressive therapy." 01/01/2007 - "[A case of ANCA-associated vasculitis with diffuse alveolar hemorrhage successfully treated by plasmapheresis]." 02/01/2013 - "Intravenous cyclophosphamide and plasmapheresis in dialysis-dependent ANCA-associated vasculitis."
3.	Kidney Transplantation 04/01/2015 - "Renal transplantation in anti-neutrophil cytoplasmic antibody-associated vasculitis." 04/01/2013 - "Renal transplantation in ANCA-associated vasculitis." 04/01/2012 - "We report a case of recurrent anti-cytoplasmic neutrophil antibody (ANCA)-associated vasculitis post kidney transplantation. " 12/15/2010 - "Recurrent ANCA-associated vasculitis after renal transplantation." 12/01/2007 - "Renal transplantation in the ANCA-associated vasculitides."
4.	Plasma Exchange 10/01/2009 - "However, no studies of plasma exchange in ANCA-associated vasculitis focused on pulmonary involvement have been reported. " 02/01/2016 - "This article summarizes the developmental history of this treatment and then looks in more detail at data on the use of plasma exchange in treating antineutrophil antibody (ANCA)-associated vasculitis. " 01/01/2015 - "Benefits of an expanded use of plasma exchange for anti-neutrophil cytoplasmic antibody-associated vasculitis within a dedicated clinical service." 04/01/2011 - "Plasma exchange for severe ANCA-associated vasculitis?" 01/01/2012 - "Plasma exchange (PE) has been shown to improve renal outcome in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and severe renal failure; however the effect of PE in AAV with moderate renal impairment is controversial. "
5.	Aftercare (After-Treatment) 12/01/2011 - "Markers of vascular injury and angiogenesis were measured before and after treatment in a large clinical trial in AAV: 163 subjects enrolled in the Rituximab in ANCA-Associated Vasculitis trial were screened for the present study. " 08/01/2005 - "To evaluate the efficacy of cytapheresis for the treatment of rapidly progressive glomerulonephritis (RPGN) caused by myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis, the renal prognosis and the mortality rate at 1 year after treatment were compared between a Cytapheresis Group and a Steroid Pulse Group. "