Dataset Information

Variability in experts' opinions regarding risks and treatment choices in patients with antineutrophil cytoplasmic antibody-associated vasculitis.

ABSTRACT: There are insufficient toxicity data available to guide treatment decisions in patients with ANCA (antineutrophil cytoplasmic antibody)-associated vasculitis. The objective of this study was to obtain expert input related to available treatment options.We performed a Web-based survey of experts (defined as physicians whose practices focus on vasculitis and physicians engaged in research in vasculitis) regarding adverse events (AEs) associated with cyclophosphamide and/or rituximab, the 2 treatment options for remission induction of severe ANCA-associated vasculitis (AAV). Using scaled measures, experts rated (i) the probability of 30 AEs associated with the treatments and (ii) the importance of disclosing each AE and reported (iii) their treatment preferences using standardized scenarios.Ratings of the probabilities of specific AEs associated with cyclophosphamide and rituximab varied significantly among the experts. The majority agreed that AEs related to fertility, infections, and serious infusion reactions were "extremely" or "very important" to disclose. Less than half of the experts surveyed endorsed disclosing the risks of progressive multifocal leukoencephalopathy, hepatitis reactivation, or zoster. For patients with newly diagnosed AAV, the majority of experts preferred intravenous cyclophosphamide for older adults and rituximab for younger women with newly diagnosed AAV. For patients with recurrent disease who had been previously treated with cyclophosphamide, the majority of experts preferred rituximab, regardless of age or sex.The variability noted in this study suggests that the information and treatment patients receive may differ depending on where they receive their care. This type of unwarranted variability could be reduced if data from long-term extension and observational studies generate more precise outcome estimates for treatment-related AEs in AAV.

SUBMITTER: Cozmuta R

PROVIDER: S-EPMC3838999 | biostudies-literature | 2013 Dec

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Variability in experts' opinions regarding risks and treatment choices in patients with antineutrophil cytoplasmic antibody-associated vasculitis.

Cozmuta Raluca R Merkel Peter A PA Fraenkel Liana L

Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases 20131201 8

<h4>Objective</h4>There are insufficient toxicity data available to guide treatment decisions in patients with ANCA (antineutrophil cytoplasmic antibody)-associated vasculitis. The objective of this study was to obtain expert input related to available treatment options.<h4>Methods</h4>We performed a Web-based survey of experts (defined as physicians whose practices focus on vasculitis and physicians engaged in research in vasculitis) regarding adverse events (AEs) associated with cyclophosphami ...[more]

PMID: 24263144

Similar Datasets

Project description:OBJECTIVE:To evaluate circulating cytokine profiles in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV), classified by antineutrophil cytoplasmic antibody (ANCA) specificity (proteinase 3 ANCA [PR3-ANCA] versus myeloperoxidase ANCA [MPO-ANCA]) or by clinical diagnosis (granulomatosis with polyangiitis [GPA] versus microscopic polyangiitis [MPA]). METHODS:A panel of 29 cytokines was tested in 186 patients with active AAV at inclusion into the Rituximab in AAV trial. Cytokine concentrations were compared between groups within each classification system. Multivariable analyses adjusted for age, sex, and renal insufficiency were performed, with each biomarker as a dependent variable and ANCA specificity and clinical diagnosis as explanatory variables of interest. RESULTS:Levels of 9 circulating cytokines (interleukin-6 [IL-6], granulocyte-macrophage colony-stimulating factor [GM-CSF], IL-15, IL-18, CXCL8/IL-8, CCL-17/thymus and activation-regulated chemokine [TARC], IL-18 binding protein [IL-18 BP], soluble IL-2 receptor ? [sIL-2R?], and nerve growth factor ? [NGF?]) were significantly higher in PR3-AAV than MPO-AAV, 4 cytokines (sIL6R, soluble tumor necrosis factor receptor type II [sTNFRII], neutrophil gelatinase-associated lipocalin [NGAL], and soluble intercellular adhesion molecule 1 [sICAM-1]) were higher in MPO-AAV than in PR3-AAV, 6 cytokines (IL-6, GM-CSF, IL-15, IL-18, sIL-2R?, and NGF?) were higher in GPA than in MPA, and 3 cytokines (osteopontin, sTNFRII, and NGAL) were higher in MPA than in GPA (all P < 0.05). For nearly all cytokines, the difference between PR3-AAV and MPO-AAV was larger than that between GPA and MPA. The multivariate analysis showed that 8 cytokines (IL-15, IL-8, IL-18 BP, NGF-?, sICAM-1, TARC, osteopontin, and kidney injury molecule 1 (P < 0.05) distinguished patients with AAV better (lower P values and larger effect sizes) when grouped by ANCA specificity than by clinical diagnosis. CONCLUSION:Distinct cytokine profiles were identified for PR3-AAV versus MPO-AAV and for GPA versus MPA. Differences in these circulating immune mediators are more strongly associated with ANCA specificity than with clinical diagnosis, suggesting that heterogeneity in the AAV subtypes extends beyond clinical phenotypes.

Project description:ImportanceAntineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic small vessel vasculitis characterized by circulating ANCAs targeting proteinase 3 (PR3) or myeloperoxidase (MPO) and associated with excess morbidity and mortality. Myeloperoxidase-ANCA-positive AAV and PR3-ANCA-positive AAV are increasingly recognized to have differences in genetic risk, pathogenesis, and response to treatment. Risk factors for AAV, including cigarette smoking, are poorly understood.ObjectiveTo examine the association between cigarette smoking and AAV.Design, setting, and participantsThis case-control study included a consecutive inception cohort of 484 patients with AAV diagnosed from 2002 to 2017 compared with a cohort of sex-, race-, and age-matched controls. Eleven cases were excluded owing to discordant smoking information in the electronic health record. Controls were randomly selected from participants recruited to the Partners HealthCare Biobank between its inception in 2010 and 2018 and who completed a smoking questionnaire and were not diagnosed with AAV (n = 30 536).ExposuresSmoking status (current, former, never) and pack-years of cigarette smoking were determined from review of the electronic medical record and smoking questionnaires.Main outcomes and measuresPatients with AAV were individually matched with 3 randomly-selected controls based on sex, race, and age (within 2 years difference). Conditional logistic regression was performed to examine the association between cigarette smoking and AAV using odds ratios (OR) and 95% confidence intervals (CIs).ResultsOverall, 473 cases were matched with 1419 controls (mean [SD] age, 59 [16] years; 281 women [59%], 396 white [84%]). Patients with AAV were more likely to be former (OR, 1.6; 95% CI, 1.3-2.0) or current smokers (OR, 2.7; 95% CI, 1.8-4.1); there was a dose-response relationship according to pack-years of exposure (P < .001). These associations were especially strong among participants with MPO-ANCA-positive disease (former smokers: OR, 1.7; 95% CI, 1.3-2.3; current smokers: OR, 3.5; 95% CI, 2.1-6.1) but not in participants with PR3-ANCA-positive AAV (former smokers: OR, 1.3; 95% CI, 0.9-2.0; current smokers: OR, 1.7; 95% CI, 0.8-3.5). After stratifying by selected demographics and disease manifestations, these associations remained strong.Conclusions and relevanceCigarette smoking was associated with AAV, especially MPO-ANCA-positive AAV. Further studies are needed to investigate a potential pathogenic mechanism.

Dataset Information

Variability in experts' opinions regarding risks and treatment choices in patients with antineutrophil cytoplasmic antibody-associated vasculitis.

Publications

Variability in experts' opinions regarding risks and treatment choices in patients with antineutrophil cytoplasmic antibody-associated vasculitis.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure