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SAGE Open Medicine 2020The ear, nose and throat region has been reported to be one of the commonest sites involved in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis diseases... (Review)
Review
BACKGROUND
The ear, nose and throat region has been reported to be one of the commonest sites involved in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis diseases and often precedes the diagnosis of ANCA-associated vasculitis by many months. Although treatment for ANCA-associated vasculitis primarily requires systemic immunosuppressive therapy, there are specific indications for sinonasal surgery during the course of the disease process. The three major roles for surgery in sinonasal vasculitis are to aid diagnosis through biopsy, enable symptom relief and nasal reconstructive surgery consideration when in remission.
PURPOSE
The aim of this systematic review is to provide an overview of the surgical procedures which can be performed in patients with ANCA-associated vasculitis presenting with sinonasal involvement.
MATERIALS AND METHODS
A systematic literature search was performed for scientific articles on MEDLINE (PubMed Advanced MEDLINE Search) and EMBASE. The search included all articles up to April 2020.
CONCLUSION
Surgical intervention during the active phase of ANCA-associated vasculitis disease can improve the patient's symptoms and enable histological diagnosis. The surgical decision to manage the nose requires a multidisciplinary approach involving the vasculitis specialist and the ear, nose and throat surgeon. Nasal reconstruction can be performed to restore form and function but only when the disease is in remission so as to maximise success and minimise complications.
PubMed: 32676189
DOI: 10.1177/2050312120936731 -
Cureus Feb 2022Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare multisystem autoimmune condition that causes inflammation of small and medium-sized blood... (Review)
Review
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare multisystem autoimmune condition that causes inflammation of small and medium-sized blood vessels and is more commonly seen in the geriatric population. ANCA-associated vasculitis (AAV) is typically characterized as necrotizing vasculitis and includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). The mortality rate remains high, with especially cardiovascular disease, infections, and malignancies being the leading causes of death. Existing treatment options depend heavily on the use of glucocorticoids (GCs), often in combination with cyclophosphamide (CYC); however, as the multitude of adverse effects associated with these agents has increased, numerous studies are being conducted to reduce not only these harmful effects but also improve remission rates. Rituximab, avacopan, corticosteroids, including intravenous pulse methylprednisolone, plasma exchange, and immunological targeting are among the emerging treatments. The purpose of this review is to emphasize the pathogenesis and traditional treatment modalities and give insights into the recent advances in managing this disorder in an attempt to spare the adverse effects of conventional therapies while achieving better remission rates with combination therapies as well. The authors explored multiple databases, employing appropriate keywords, satisfying the quality appraisal, after which a total of 14 reports were included in this review. Upon overall analysis, it can be concluded that rituximab and CYC, when used in combination, provided a safer alternative to GCs while exhibiting equal, if not superior, effectiveness and results, thus, paving the way for additional in-depth research in a larger population of interest.
PubMed: 35155037
DOI: 10.7759/cureus.21814 -
Seminars in Arthritis and Rheumatism Feb 2016Published small case series suggest that inflammatory bowel disease [IBD; Crohn's disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than... (Review)
Review
BACKGROUND
Published small case series suggest that inflammatory bowel disease [IBD; Crohn's disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than would be expected by chance.
OBJECTIVES
To describe this association by an analysis of a large cohort of carefully studied patients and through a systematic literature review.
METHODS
Patients with both IBD and vasculitis enrolled in the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Studies, followed in Canadian Vasculitis research network (CanVasc) centers and/or in the University of Toronto's IBD clinic were included in this case series. A systematic literature review of patients with IBD and vasculitis involved a PubMed search through February 2014. The main characteristics of patients with Takayasu arteritis (TAK) and IBD were compared to those in patients with TAK without IBD followed in the VCRC.
RESULTS
The study identified 32 patients with IBD and vasculitis: 13 with large-vessel vasculitis [LVV; 12 with TAK, 1 with giant cell arteritis (GCA); 8 with CD, 5 with UC]; 8 with ANCA-associated vasculitis [AAV; 6 granulomatosis with polyangiitis (GPA), 2 with eosinophilic granulomatosis with polyangiitis (EGPA)]; 5 with isolated cutaneous vasculitis; and 6 with other vasculitides. Patients with LVV and AAV were mostly female (18/21). The diagnosis of IBD preceded that of vasculitis in 12/13 patients with LVV and 8/8 patients with AAV. The review of the literature identified 306 patients with IBD and vasculitis: 144 with LVV (133 TAK; 87 with IBD preceding LVV), 19 with AAV [14 GPA, 1 EGPA, 4 microscopic polyangiitis (MPA)], 66 with isolated cutaneous vasculitis, and 77 with other vasculitides. Patients with IBD and TAK were younger and had more frequent headaches, constitutional symptoms, or gastrointestinal symptoms compared to those patients in the VCRC who had TAK without IBD.
CONCLUSIONS
These findings highlight the risk of vasculitis, especially TAK, in patients with IBD (both CD and UC).
Topics: Female; Humans; Inflammatory Bowel Diseases; Male; Retrospective Studies; Vasculitis
PubMed: 26315859
DOI: 10.1016/j.semarthrit.2015.07.006 -
The Journal of Rheumatology Apr 2021In 2015, the Canadian Vasculitis Research Network (CanVasc) created recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated...
OBJECTIVE
In 2015, the Canadian Vasculitis Research Network (CanVasc) created recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) in Canada. The current update aims to revise existing recommendations and create additional recommendations, as needed, based on a review of new available evidence.
METHODS
A needs assessment survey of CanVasc members informed questions for an updated systematic literature review (publications spanning May 2014 to September 2019) using Medline, Embase, and Cochrane. New and revised recommendations were developed and categorized according to the level of evidence and strength of each recommendation. The CanVasc working group used a 2-step modified Delphi procedure to reach > 80% consensus on the inclusion, wording, and grading of each new and revised recommendation.
RESULTS
Eleven new and 16 revised recommendations were created and 12 original (2015) recommendations were retained. New and revised recommendations are discussed in detail within this document. Five original recommendations were removed, of which 4 were incorporated into the explanatory text. The supplementary material for practical use was revised to reflect the updated recommendations.
CONCLUSION
The 2020 updated recommendations provide rheumatologists, nephrologists, and other specialists caring for patients with AAV in Canada with new management guidance, based on current evidence and consensus from Canadian experts.
Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antibodies, Antineutrophil Cytoplasmic; Canada; Consensus; Cytoplasm; Humans
PubMed: 32934123
DOI: 10.3899/jrheum.200721 -
The Cochrane Database of Systematic... Sep 2020Anti-neutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) are a group of rare auto-inflammatory diseases that affects mainly small vessels. AAV... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Anti-neutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) are a group of rare auto-inflammatory diseases that affects mainly small vessels. AAV includes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Anti-cytokine targeted therapy uses biological agents capable of specifically targeting and neutralising cytokine mediators of the inflammatory response.
OBJECTIVES
To assess the benefits and harms of anti-cytokine targeted therapy for adults with AAV.
SEARCH METHODS
We searched the Cochrane Central Register of Controlled Trials (2019, Issue 7), MEDLINE and Embase up to 16 August 2019. We also examined reference lists of articles, clinical trial registries, websites of regulatory agencies and contacted manufacturers.
SELECTION CRITERIA
Randomised controlled trials (RCTs) or controlled clinical trials of targeted anti-cytokine therapy in adults (18 years or older) with AAV compared with placebo, standard therapy or another modality and anti-cytokine therapy of different type or dose.
DATA COLLECTION AND ANALYSIS
We used standard methodological procedures expected by Cochrane.
MAIN RESULTS
We included four RCTs with a total of 440 participants (mean age 48 to 56 years). We analysed the studies in three groups: 1) mepolizumab (300 mg; three separate injections every four weeks for 52 weeks) versus placebo in participants with relapsing or refractory EGPA; 2) belimumab (10 mg/kg on days 0, 14, 28 and every 28 days thereafter until 12 months after the last participant was randomised) or etanercept (25 mg twice a week) with standard therapy (median 25 months) versus placebo with standard therapy (median 19 months) in participants with GPA/MPA; and 3) infliximab (3 mg/kg on days 1 and 14, before the response assessment on day 42) versus rituximab (0.375g/m on days 1, 8, 15 and 22) in participants with refractory GPA for up to 12 months. None of the studies were assessed as low risk of bias in all domains: one study did not report randomisation or blinding methods clearly. Three studies were at high risk and one study was at unclear risk of bias for selective outcome reporting. One trial with 136 participants with relapsing or refractory EGPA compared mepolizumab with placebo during 52 weeks of follow-up and observed one death in the mepolizumab group (1/68, 1.5%) and none in the placebo group (0/68, 0%) (Peto odds ratio (OR) 7.39, 95% confidence interval (CI) 0.15 to 372.38; low-certainty evidence). Low-certainty evidence suggests that more participants in the mepolizumab group had ≥ 24 weeks of accrued remission over 52 weeks compared to placebo (27.9% versus 2.9%; risk ratio (RR) 9.5, 95% CI 2.30 to 39.21), and durable remission within the first 24 weeks sustained until week 52 (19.1% mepolizumab versus 1.5% placebo; RR 13.0, 95% CI 1.75 to 96.63; number needed to treat for an additional beneficial outcome (NNTB) 6, 95% Cl 4 to 13). Mepolizumab probably decreases risk of relapse (55.8% versus 82.4%; RR 0.68, 95% CI 0.53 to 0.86; NNTB 4, 95% CI 3 to 9; moderate-certainty evidence). There was low-certainty evidence regarding similar frequency of adverse events (AEs): total AEs (96.9% versus 94.1%; RR 1.03, 95% CI 0.96 to 1.11), serious AEs (17.7% versus 26.5%; RR 0.67, 95% CI 0.35 to 1.28) and withdrawals due to AEs (2.9% versus 1.5%; RR 2.00, 95% CI 0.19 to 21.54). Disease flares were not measured. Based on two trials with different follow-up periods (mean of 27 months for etanercept study; up to four years for belimumab study) including people with GPA (n = 263) and a small group of participants with MPA (n = 22) analysed together, we found low-certainty evidence suggesting that adding an active drug (etanercept or belimumab) to standard therapy does not increase or reduce mortality (3.4% versus 1.4%; Peto OR 2.45, 95% CI 0.55 to 10.97). Etanercept may have little or no effect on remission (92.3% versus 89.5%; RR 0.97, 95% CI 0.89 to 1.07), durable remission (70% versus 75.3%; RR 0.93, 95% CI 0.77 to 1.11; low-certainty evidence) and disease flares (56% versus 57.1%; RR 0.98, 95% CI 0.76 to 1.27; moderate-certainty evidence). Low-certainty evidence suggests that belimumab does not increase or reduce major relapse (1.9% versus 0%; RR 2.94, 95% CI 0.12 to 70.67) or any AE (92.5% versus 82.7%; RR 1.12, 95% CI 0.97 to 1.29). Low-certainty evidence suggests a similar frequency of serious or severe AEs (47.6% versus 47.6%; RR 1.00, 95% CI 0.80 to 1.27), but more frequent withdrawals due to AEs in the active drug group (11.2%) compared to the placebo group (4.2%), RR 2.66, 95% CI 1.07 to 6.59). One trial involving 17 participants with refractory GPA compared infliximab versus rituximab added to steroids and cytotoxic agents for 12 months. One participant died in each group (Peto OR 0.88, 95% CI, 0.05 to 15.51; 11% versus 12.5%). We have very low-certainty evidence for remission (22% versus 50%, RR 0.44, 95% Cl 0.11 to 1.81) and durable remission (11% versus 50%, RR 0.22, 95% CI 0.03 to 1.60), any severe AE (22.3% versus 12.5%; RR 1.78, 95% CI 0.2 to 16.1) and withdrawals due to AEs (0% versus 0%; RR 2.70, 95% CI 0.13 to 58.24). Disease flare/relapse and the frequency of any AE were not reported.
AUTHORS' CONCLUSIONS
We found four studies but concerns about risk of bias and small sample sizes preclude firm conclusions. We found moderate-certainty evidence that in patients with relapsing or refractory EGPA, mepolizumab compared to placebo probably decreases disease relapse and low-certainty evidence that mepolizumab may increase the probability of accruing at least 24 weeks of disease remission. There were similar frequencies of total and serious AEs in both groups, but the study was too small to reliably assess these outcomes. Mepolizumab may result in little to no difference in mortality. However, there were very few events. In participants with GPA (and a small subgroup of participants with MPA), etanercept or belimumab may increase the probability of withdrawal due to AEs and may have little to no impact on serious AEs. Etanercept may have little or no impact on durable remission and probably does not reduce disease flare.
Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antibodies, Monoclonal, Humanized; Churg-Strauss Syndrome; Etanercept; Granulomatosis with Polyangiitis; Humans; Immunosuppressive Agents; Infliximab; Microscopic Polyangiitis; Middle Aged; Numbers Needed To Treat; Placebos; Randomized Controlled Trials as Topic; Rituximab; Secondary Prevention; Steroids
PubMed: 32990324
DOI: 10.1002/14651858.CD008333.pub2 -
Revista Brasileira de Reumatologia 2017The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with...
The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed), EMBASE and Cochrane database to retrieve articles until October 2016. PRISMA guidelines were used for the systematic review and articles were assessed according to the Oxford levels of evidence. Sixteen recommendations were made regarding different aspects of induction therapy for AAV. The purpose of these recommendations is to serve as a guide for therapeutic decisions by health care professionals in the management of AAV patients presenting active disease.
Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Brazil; Consensus; Humans; Rheumatology; Societies, Medical
PubMed: 28754431
DOI: 10.1016/j.rbre.2017.06.003 -
Autoimmunity Reviews Aug 2015To describe the clinical presentation, management and prognosis of patients diagnosed with both primary Sjögren's syndrome (pSS) and anti-neutrophil cytoplasmic... (Review)
Review
OBJECTIVES
To describe the clinical presentation, management and prognosis of patients diagnosed with both primary Sjögren's syndrome (pSS) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
METHODS
French nation-wide survey completed by a systematic literature review.
RESULTS
This work identified 7 new cases of coexisting pSS and AAV: 2 microscopic polyangiitis (MPA), 2 granulomatosis with polyangiitis (GPA), 2 anti-myeloperoxidase (MPO)-ANCA renal-limited AAV, and 1 eosinophilic granulomatosis with polyangiitis (EGPA). The systematic literature search identified 15 previously published cases. Among the 22 patients, 19 were females. Mean age at diagnosis of AAV was 63.9±9.8years. All individuals with available information experienced at least one extra-glandular manifestation attributable to pSS. p-ANCA with anti-MPO specificity were found in 76.2% (16/21), c-ANCA with anti-PR3 specificity in 14.3% (3/21) and isolated c-ANCA in 13.6% (3/22). Vasculitis involved kidneys (n=13), lungs (n=8), skin (n=6), peripheral nerves (n=5), central nervous system (n=2), small bowel (n=1), muscle (n=1), ear chondritis (n=1) and sinuses (n=1). The mean AAV follow-up was 73.5 (±120.0) months. While on treatment, disease remission occurred in 77.3% of cases, and one death was reported in the first 6months after diagnosis.
CONCLUSION
This work shows that AAV may occur in patients with pSS. These are most commonly p-ANCA associated vasculitis with anti-MPO specificity. AAV may reveal an underlying pSS or arise during its evolution, but did not precede pSS in any of these cases. AAV occurrence appears to be correlated with extra-glandular manifestations of pSS.
Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Humans; Kidney Diseases; Prognosis; Sjogren's Syndrome
PubMed: 25916811
DOI: 10.1016/j.autrev.2015.04.009 -
Clinical Rheumatology Feb 2023Spontaneous renal hemorrhage (SRH) in ANCA-associated vasculitis (AAV) is rare but fatal. We aimed to characterize clinical manifestations and managements of AAV...
Clinical features and management of Chinese anti-neutrophil cytoplasmic antibody-associated vasculitis patients with spontaneous renal hemorrhage: a single-center report and systematic review.
INTRODUCTION
Spontaneous renal hemorrhage (SRH) in ANCA-associated vasculitis (AAV) is rare but fatal. We aimed to characterize clinical manifestations and managements of AAV patients with SRH.
METHOD
Hospitalized AAV patients were screened from January 2000 to April 2021, at Peking Union Medical College Hospital (PUMCH). Also, a systematic review was based on retrieving all the relevant literature from PubMed, MedlinePlus, and Web of Science until April 2021. Clinical features, management, and prognosis of the patients were collected and concluded.
RESULTS
In PUMCH, four out of 1640 AAV patients with SRH were included in our study; three had granulomatosis with polyangiitis (GPA) and one had microscopic polyangiitis (MPA). The ratio of men to women was 3 to 1, and the average age of onset was 55 years. The Birmingham Vasculitis Activity Score (BVAS) ranged from 21 to 23. Combining with documented reports, 13 patients were diagnosed as AAV complicated with SRH (including four from PUMCH), 7 with GPA, and 6 with MPA. Mean BVAS was 25.2 ± 6.6. The symptoms of SRH presented as severe back or abdominal pain. Patients with SRH to age- and gender-matched patients without SRH were compared, and we found that in the SRH group, the duration of disease was shorter, and BVAS, renal function, and inflammatory markers (WBC and ESR) were significantly greater, whereas Hb, Alb, and renal function greatly reduced.
CONCLUSION
This is the first summary of clinical features and treatments of SRH in AAV. Patients with AAV in early stage and with high disease activity appeared to be more likely to develop SRH. Key Points • This is the first summary of clinical features and treatments of SRH in AAV. • SRH more likely occurs in AAV patients in the early stage (≤ 3 months) and with high disease activity. • Clinicians should be aware of the possibility of SRH when AAV patients complain of back or abdominal pain.
Topics: Male; Humans; Female; Middle Aged; East Asian People; Antibodies, Antineutrophil Cytoplasmic; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Microscopic Polyangiitis; Kidney; Kidney Diseases; Hemorrhage; Granulomatosis with Polyangiitis
PubMed: 36190664
DOI: 10.1007/s10067-022-06397-4 -
Modern Rheumatology Jan 2019To provide evidence for the revision of clinical practice guideline (CPG) for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) by... (Meta-Analysis)
Meta-Analysis
Systematic review and meta-analysis for 2017 clinical practice guidelines of the Japan research committee of the ministry of health, labour, and welfare for intractable vasculitis for the management of ANCA-associated vasculitis.
OBJECTIVES
To provide evidence for the revision of clinical practice guideline (CPG) for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) by the Japan Research Committee for Intractable Vasculitis.
METHODS
PubMed, CENTRAL, and the Japan Medical Abstracts Society were searched for articles published between January 1994 and January 2015 to conduct systematic review (SR), and the quality of evidence was assessed with GRADE approach.
RESULTS
Nine randomized controlled trials (RCTs) and two non-RCTs were adopted for remission induction therapy, three RCTs and two non-RCTs for plasma exchange, and five RCTs and one non-RCT for remission maintenance therapy. A significant difference was found in efficacy and safety for the following comparisons. In the non-RCT adopted for remission induction therapy, glucocorticoid (GC) + cyclophosphamide (CY) was significantly superior to GC monotherapy regarding remission. GC + intravenous CY for remission induction therapy was superior to GC + oral CY regarding death at one year, serious adverse events, and serious infection. Concomitant use of plasma exchange for remission induction therapy of AAV with severe renal dysfunction reduced risk of end-stage renal disease versus non-users at month 3.
CONCLUSION
This SR provided necessary evidence for developing CPG for the management of ANCA-associated vasculitis.
Topics: Advisory Committees; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Government Agencies; Humans; Immunosuppressive Agents; Japan; Practice Guidelines as Topic; Randomized Controlled Trials as Topic
PubMed: 29996690
DOI: 10.1080/14397595.2018.1500111 -
Clinical and Experimental Immunology Dec 2020We compared the common pathway components C3a, C5a and membrane attack complex (MAC), also known as C5b-9, and the alternative pathway components factor B and properdin... (Meta-Analysis)
Meta-Analysis
We compared the common pathway components C3a, C5a and membrane attack complex (MAC), also known as C5b-9, and the alternative pathway components factor B and properdin in patients with ANCA-associated vasculitis (AAV) and healthy controls, and conducted a meta-analysis of the available clinical evidence for the role of complement activation in the pathogenesis of AAV. Complement components were evaluated in 59 patients with newly diagnosed or relapsing granulomatosis with polyangiitis or microscopic polyangiitis and 36 healthy volunteers. In 28 patients, testing was repeated in remission. Next, we performed a meta-analysis by searching databases to identify studies comparing complement levels in AAV patients and controls. A random-effects model was used for statistical analyses. The median concentrations of MAC, C5a, C3a and factor B were higher in active AAV patients (P < 0·001). Achievement of remission was associated with reductions in C3a (P = 0·005), C5a (P = 0·035) and factor B levels (P = 0·045), whereas MAC and properdin levels did not change. In active AAV, there were no effects of ANCA specificity, disease phenotype, previous immunosuppression or disease severity on complement levels. A total of 1122 articles were screened, and five studies, including this report, were entered into the meta-analysis. Plasma MAC, C5a and factor B in patients with active AAV were increased compared to patients in remission (excluding factor B) and controls. Changes in C3a were of borderline significance. Our findings and the results of the meta-analysis support activation of the complement system predominantly via the alternative pathway in AAV patients.
Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Complement Pathway, Alternative; Complement System Proteins; Humans
PubMed: 32691878
DOI: 10.1111/cei.13498