CanVasc Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitides

McGeoch, Lucy; Twilt, Marinka; Famorca, Leilani; Bakowsky, Volodko; Barra, Lillian; Benseler, Susanne M.; Cabral, David A.; Carette, Simon; Cox, Gerald; Dhindsa, Navjot; Dipchand, Christine; Fifi‐Mah, Aurore; Goulet, M; Khalidi, Nader; Khraishi, Majed; Liang, Patrick; Milman, Nataliya; Pineau, Christian; Reich, Heather N.; Samadi, Nooshin; Shojania, Kam; Taylor-Gjevre, Regina M.; Towheed, Tanveer; Trudeau, Judith; Walsh, Michael; Yacyshyn, Elaine; Pagnoux, Christian

doi:10.3899/jrheum.150376

Cited by 69 publications

(77 citation statements)

References 127 publications

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“…No specific pediatric management guidelines are available to guide the therapeutic approach in pediatric patients with AAV. Therefore, treatment recommendations are adapted from the adult clinical trials and expert consensus ( 42 , 43 ). Survival rates in AAV has improved secondary to better disease management, the expertise of care teams at academic and referral vasculitis centers and treatment based on intensive remission induction followed by maintenance therapy.…”

Section: Treatmentmentioning

confidence: 99%

“…Survival rates in AAV has improved secondary to better disease management, the expertise of care teams at academic and referral vasculitis centers and treatment based on intensive remission induction followed by maintenance therapy. The Canadian Vasculitis research network (CanVasc) recommends that children with newly diagnosed AAV should be treated as per adult recommendations for induction of remission and then maintenance ( 43 ). Glucocorticoids and cyclophosphamide has been associated with dramatic improvement in patients with AAV, however this combination has not prevented relapses in the majority of patients and is associated with short and long term toxicity risk ( 44 ).…”

Section: Treatmentmentioning

confidence: 99%

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Primary Vasculitis in Childhood: GPA and MPA in Childhood

Jariwala

Laxer

2018

Front. Pediatr.

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Childhood onset anti-neutrophilic cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a rare group of primary systemic vasculitides affecting medium and small blood vessels. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and renal limited ANCA vasculitis. These disorders are associated with severe clinical manifestations, frequent relapses and a high cumulative morbidity, and often present with multisystem involvement. Renal involvement is common in the pediatric age group, characterized by pauci-immune necrotizing and crescentic glomerulonephritis which frequently progresses to chronic kidney disease in adulthood. ANCAs against proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO) (MPO-ANCA) remain the hallmark of AAV and are integral to the disease pathogenesis. Newer understanding of neutrophil extracellular traps and complement activation have provided better insights into disease pathogenesis. A pediatric vasculitis working group has developed and validated childhood vasculitis classification criteria and disease activity and damage scores. No specific pediatric treatment recommendations exist due to rare nature of the illness in pediatric population. Smaller case series have been published on the efficacy of adult treatment regimens in pediatric patients. The prognosis often remains guarded with frequent relapses and a high cumulative morbidity. The aim of this article is to provide a comprehensive review on pediatric AAV with a focus on recent observations regarding epidemiology, disease pathogenesis, treatment, and prognosis.

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Section: Treatmentmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Primary Vasculitis in Childhood: GPA and MPA in Childhood

Jariwala

Laxer

2018

Front. Pediatr.

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“…The distinction between the 2 categories varies somewhat. For example, CanVasc defines severe disease as the “presence of life- or major organ-threatening manifestations, including severe and progressive kidney involvement; severe alveolar hemorrhage; severe GI, cardiac, CNS, and/or eye involvements; or any other manifestations considered severe enough to require induction treatment with CYC or RTX.” 3 , 4 In contrast, BSR/BHPR seems to group more patients into nonsevere disease as “those with no evidence of organ damage who may be considered for alternative induction therapy with MTX or MMF,” and all other patients should be considered to have severe disease and treated with CYC or rituximab (RTX). In addition to these categorizations, CanVasc refers to the use of the 5-factor score for prognostication of EGPA and MPA.…”

Section: Diagnosis Of Aavmentioning

confidence: 99%

“…Guidelines for management of AAV have been published by various medical societies. This review compares 4 guidelines published in the English language, from the: (i) British Society for Rheumatology (BSR) and British Health Professionals for Rheumatology (BHPR) (2014), 1 updated from their 2007 guidelines 2 ; (ii) the Canadian Vasculitis Research Network (CanVasc) (2015) 3 developed by members of the core committee of the CanVasc research network; (iii) the European League Against Rheumatism (EULAR)/European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) (2016), 4 developed by an international task force representing EULAR, ERA, and the European Vasculitis Society (EUVAS), updated from their 2008 guidelines 5 ; and (iv) the Brazilian Society of Rheumatology (SBR) (2017), which focused only on induction therapy of AAV ( Table 1 ). 6 There are no guidelines published from the United States, and although the American College of Rheumatology did not endorse the recently published EULAR/ERA-EDTA guidelines, an American College of Rheumatology representative contributed to these guidelines.…”

mentioning

confidence: 99%

Comparisons of Guidelines and Recommendations on Managing Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Geetha

Jin

Scott

et al. 2018

Kidney International Reports

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Antineutrophil cytoplasmic antibodies−associated vasculitis (AAV) is associated with high morbidity or mortality, especially if not promptly diagnosed and treated. Many inroads have been made in the understanding of the pathophysiology that leads to exploration of novel therapies. Randomized controlled trials over the last 2 decades have better delineated and expanded therapeutic options and set the stage for an evidence-based approach. Since 2014, 4 scientific societies have systematically reviewed the existing data and have formulated evidence-based recommendations for the management of AAV. These recommendations cover diagnosis, remission induction and maintenance treatment, and prevention of long-term complications. This review is a comparative analysis of the recently published recommendations of the European League Against Rheumatism/European Renal Association-European Dialysis and Transplant Association, the British Society of Rheumatology, the Canadian Vasculitis Research Network, and the Brazilian Society of Rheumatology, and aims to determine common ground among them and highlights the differences among the recommendations.

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“…The large number of clinical trials in AAV has fostered enough evidence for three major international societies, the European League against Rheumatism (EULAR), the British Society for Rheumatology (BSR) and the Canadian Vasculitis researchers network (CanVasc) to propose guidelines for the management of AAV. Importantly, all these guidelines recognize the equivalence of cyclophosphamide‐ and rituximab‐based regimens for remission induction in AAV, as well as recommend the need to screen for and appropriately manage cardiovascular risk factors and the risk of future malignancy in such patients . There is also an increasing thrust to use patient‐driven and patient‐related outcome assessment tools such as the Patient Related Outcomes Measurement Information System (PROMIS) and the AAV‐patient related outcomes (AAV‐PRO) tool in this group of patients to help assess better the actual impact of the disease …”

Section: Anca Vasculitismentioning

confidence: 99%

Vasculitis research: Current trends and future perspectives

et al. 2018

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We discuss recent and prospective research in small and large vessel vasculitis. Large cohorts of Takayasu arteritis (TA) have been recently published from across the world, clarifying our understanding of this uncommon disease. Novel open-ended approaches like large-scale genotyping, proteomics and metabolomics have helped gain novel insights into TA, giant cell arteritis (GCA) and anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Recent advances in the imaging of TA and GCA offer promise for earlier diagnosis and better monitoring of response to therapy. Although two randomized controlled trials of abatacept and tocilizumab failed to meet their primary end-points, successful large-scale studies of abatacept and tocilizumab in GCA hold promise for better disease control. While cyclophosphamide has revolutionized the management of AAV, increasing use of rituximab as an alternative induction regimen, as well as use of novel approaches involving reduced or no corticosteroid use for AAV and alternative agents such as avacopan (a complement 5a receptor antagonist) hold promise for lesser toxic induction regimens in the future. Increasingly, the risk of cardiovascular events and comorbidities such as osteoporosis are being recognized as factors affecting long-term prospects of patients with vasculitis. There is a shift in emphasis to utilize patient-reported outcomes to more accurately gauge the impact of vasculitides and their treatment.

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CanVasc Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitides

Cited by 69 publications

References 127 publications

Primary Vasculitis in Childhood: GPA and MPA in Childhood

Primary Vasculitis in Childhood: GPA and MPA in Childhood

Comparisons of Guidelines and Recommendations on Managing Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Vasculitis research: Current trends and future perspectives

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