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Management of ANCA associated vasculitis

Published on Mar 18, 2020in BMJ27.604
· DOI :10.1136/BMJ.M421
Zachary S. Wallace21
Estimated H-index: 21
(Harvard University),
Eli M. Miloslavsky14
Estimated H-index: 14
(Harvard University)
Abstract
ABSTRACT Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a small to medium vessel vasculitis associated with excess morbidity and mortality. This review explores how management of AAV has evolved over the past two decades with pivotal randomized controlled trials shaping the management of induction and maintenance of remission. Contemporary AAV care is characterized by approaches that minimize the cumulative exposure to cyclophosphamide and glucocorticoids, increasingly use rituximab for remission induction and maintenance, and consider therapies with less toxicity (for example, methotrexate, mycophenolate mofetil) for manifestations of AAV that do not threaten organ function or survival. Simultaneously, improvements in outcomes, such as renal and overall survival, have been observed. Additional trials and observational studies evaluating the comparative effectiveness of agents for AAV in various patient subgroups are needed. Prospective studies are necessary to assess the effect of psychosocial interventions on patient reported outcomes in AAV. Despite the expanding array of treatments for AAV, little guidance on how to personalize AAV care is available to physicians.
  • References (166)
  • Citations (2)
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References166
Newest
#1Michael Walsh (Population Health Research Institute)H-Index: 66
#2Peter A. Merkel (Population Health Research Institute)H-Index: 1
Last. Ron Wald (Population Health Research Institute)H-Index: 1
view all 36 authors...
Abstract Background More effective and safer treatments are needed for antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis. Methods We conducted a randomized trial with a 2-by-2 factor...
11 CitationsSource
#1Itthiphat Arunsurat (KKU: Khon Kaen University)H-Index: 1
#2Wipa Reechaipichitkul (KKU: Khon Kaen University)H-Index: 10
Last. Pongsakorn Kampan (KKU: Khon Kaen University)H-Index: 1
view all 8 authors...
Abstract Granulomatous polyangiitis (GPA) is a multiple systemic necrotizing vasculitis. Diagnosis of pulmonary nodules in GPA is still challenging in clinical practice, however, other extrapulmonary manifestations, serology, and histopathology may help the diagnosis of GPA. This case series was of limed GPA with one of the largest pulmonary nodules which had a poor treatment response in contrast with previous literature.
1 CitationsSource
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small- to medium-vessel necrotizing vasculitis responsible for excess morbidity and mortality (1). The AAVs, which include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), are among the most difficult types of vasculitis to treat. Although clinicopathologic disease definitions have been used traditionally to categorize patients into one of ...
2 CitationsSource
#1Eveline Y. Wu (UNC: University of North Carolina at Chapel Hill)H-Index: 6
#2Elizabeth A. McInnis (UNC: University of North Carolina at Chapel Hill)H-Index: 3
Last. Donna O. Bunch (UNC: University of North Carolina at Chapel Hill)H-Index: 15
view all 16 authors...
OBJECTIVE: There is accumulating evidence that complement activation is important in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) pathogenesis. This study was undertaken to investigate complement activation in AAV with myeloperoxidase (MPO) positivity and AAV with proteinase 3 (PR3) positivity after determining optimal methods for measuring activated complement factors in circulation. METHODS: Participants included 98 patients with AAV (45 MPO-ANCA positive, 53 PR3-ANCA...
Source
#1Gabrielle Y. Liu (UCSF: University of California, San Francisco)H-Index: 2
#2Iazsmin Bauer Ventura (U of C: University of Chicago)H-Index: 1
Last. Brett Ley (UCSF: University of California, San Francisco)H-Index: 21
view all 10 authors...
Background Antineutrophil cytoplasmic antibodies (ANCAs) have been reported to occur in 7% to 10% of patients with idiopathic pulmonary fibrosis (IPF), but their clinical relevance remains unclear. The aim of this study was to estimate the prevalence of ANCAs in a North American population with IPF and evaluate their clinical significance. Methods This was a retrospective study of two independent cohorts of patients diagnosed with IPF at the University of California San Francisco (discovery coho...
Source
#2Arnaud HotH-Index: 11
Last. Benjamin Terrier (Paris V: Paris Descartes University)H-Index: 38
view all 31 authors...
OBJECTIVE: Orbital mass is a rare and sight-threatening manifestation of ANCA-associated vasculitides, which remains a therapeutic challenge. We aimed to describe the presentation, therapeutic management and outcome of ANCA-associated vasculitides-related orbital mass. METHODS: We conducted a French nationwide retrospective study of patients with orbital mass in the setting of ANCA-associated vasculitides according to ACR criteria and/or Chapel Hill Consensus Conference definitions. RESULTS: Fif...
1 CitationsSource
#1Alvise Berti (Mayo Clinic)H-Index: 13
#2Roscoe L. Warner (UM: University of Michigan)H-Index: 36
Last. Peter A. Merkel (UPenn: University of Pennsylvania)H-Index: 71
view all 16 authors...
Abstract Objective To investigate serum IL-6 (sIL-6) levels during active disease, complete remission (CR), and relapse in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), and to explore the association of changes in sIL-6 with clinical outcomes. Methods sIL-6 levels were measured at baseline and longitudinally over 18 months, in 78 patients with AAV enrolled in a randomized controlled trial comparing treatment with either rituximab (RTX) or cyclophosphamide (CYC)/azathiop...
1 CitationsSource
Abstract Background Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis and a leading cause of systemic sclerosis–related death. Nintedanib, a tyrosine kinase inhibitor,...
20 CitationsSource
#1Kaitlin A. Quinn (Georgetown University)H-Index: 3
#2Cailin H. Sibley (OHSU: Oregon Health & Science University)H-Index: 9
Last. Peter C. GraysonH-Index: 18
view all 21 authors...
OBJECTIVES: To describe tracheobronchial disease in patients with granulomatosis with polyangiitis (GPA) and evaluate the utility of dynamic expiratory CT to detect large-airway disease. METHODS: Demographic and clinical features associated with the presence of subglottic stenosis (SGS) or endobronchial involvement were assessed in a multicentre, observational cohort of patients with GPA. A subset of patients with GPA from a single-centre cohort underwent dynamic chest CT to evaluate the airways...
Source
OBJECTIVES: Rituximab was proven superior to azathioprine for maintenance treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The high cost of rituximab might, however, limit its routine use. This study determined the cost-effectiveness of intravenous rituximab (5 x 500 mg until month 18), versus oral azathioprine (2 mg/kg per day, gradually decreased between month 12 and 22), for maintenance treatment of patients with granulomatosis with polyangiitis, microscopi...
3 Citations
Cited By2
Newest
#1Chebotareva NvH-Index: 5
#2E S KamyshovaH-Index: 2
Last. Sergey MoiseevH-Index: 9
view all 5 authors...
Abstract Background There are reports of circulating antineutrophil cytoplasmic autoantibodies (ANCA) in patients with immunoglobulin A (IgA) nephropathy with an uncertain pathogenic role. Aim of the work To present the findings of myeloperoxidase (MPO) ANCA-positive patients amid a different course of IgA nephropathy with crescents and to discuss the efficacy of immunosuppressive therapy. Case presentation Two cases of IgA nephropathy associated with positive ANCA are reported and a review of t...
Source
#1Itthiphat Arunsurat (KKU: Khon Kaen University)H-Index: 1
#2Wipa Reechaipichitkul (KKU: Khon Kaen University)H-Index: 10
Last. Pongsakorn Kampan (KKU: Khon Kaen University)H-Index: 1
view all 8 authors...
Abstract Granulomatous polyangiitis (GPA) is a multiple systemic necrotizing vasculitis. Diagnosis of pulmonary nodules in GPA is still challenging in clinical practice, however, other extrapulmonary manifestations, serology, and histopathology may help the diagnosis of GPA. This case series was of limed GPA with one of the largest pulmonary nodules which had a poor treatment response in contrast with previous literature.
1 CitationsSource