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Objective.Aspects of antineutrophil cytoplasmic antibodies–associated vasculitis (AAV) prioritized by patients with AAV were described using the International Classification of Function, Disability, and Health (ICF).Methods.Items identified during 14 individual interviews were incorporated into an ICF-based questionnaire administered to participants of 2 vasculitis patient symposia: 36 in the United Kingdom and 63 in the United States.Results.Categories identified as at least “moderately relevant” by ≥ 5% of subjects included 44 body functions, 14 body structures, 35 activities and participation, 31 environmental factors, and 38 personal factors.Conclusion.Identified categories differ from those identified by the current Outcome Measures in Rheumatology (OMERACT) core set and those prioritized by vasculitis experts.

Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are multisystem diseases of small blood vessels, collectively known as the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). This study explores the patient’s perspective on the use of glucocorticoids, which are still a mainstay of treatment in AAV. Patients with AAV from the UK, USA, and Canada were interviewed, using purposive sampling to include a range of disease manifestations and demographics. The project steering committee, including patient partners, designed the interview prompts and cues about AAV, its treatment, and impact on health-related quality of life. Interviews were transcribed and analysed to establish themes grounded in the data. A treatment-related code was used to focus analysis of salient themes related to glucocorticoid therapy. Fifty interviews were conducted. Individual themes related to therapy with glucocorticoids emerged from the data and were analysed. Three overarching themes emerged: (1) Glucocorticoids are effective at the time of diagnosis and during relapse, and withdrawal can potentiate a flare, (2) glucocorticoids are associated with salient emotional, physical, and social effects (depression, anxiety, irritation, weight gain and change in appearance, diabetes mellitus, effect on family and work); and (3) patient perceptions of balancing the risks and benefits of glucocorticoids. Patients identified the positive aspects of treatment with glucocorticoids; they are fast-acting and effective, but, they voiced concerns about adverse effects and the uncertainty of the dose-reduction process. These results may be informative in the development of novel glucocorticoid-sparing regimens., Sponsored by University of Oxford and the Vasculitis Clinical Research Consortium, with support from the US National Institute of Arthritis and Musculoskeletal and Skin Diseases (U54 AR057319 and U01 AR51874), the National Center for Research Resources (U54 RR019497); and the Office of Rare Diseases Research. Additional support for the work of the OMERACT vasculitis working group was received by a Patient-Centered Outcomes Research Institute Pilot Project Grant, the Medical Research Fund, Oxford, and the Oxfordshire Health Services Research Committee. Dr. Robson and Professor Luqmani are supported in part by the National Institute for Health Research Musculoskeletal Biomedical Research Unit, Oxford, UK. Dr. Milman was supported by a UCB/Canadian Rheumatology Association/Arthritis Society postgraduate rheumatology fellowship award and a research fellowship from the Department of Medicine at the Ottawa Hospital.

The original publication listed author Alan Kaye as "A. Kaye," but his name should read "A.D. Kaye." The correct author name is presented in this article.

Background: ANCA-associated vasculitis (AAV) is a significant cause of morbidity and mortality. Meeting the challenges of aAV effectively requires patients to engage in self-management, including medical management, role management, and emotional management.1 Self-management is recognised as a key aspect of chronic disease management and forms an important part of the National Health Service 5-year forward plan and the UK strategy for rare diseases.2,3 Nonetheless, there has been limited specific research into the role of self-management in the aAV literature. Objectives: To explore experiences and views of self-management amongst aAV patients. Methods: A secondary analysis of all interview transcripts from a previous project exploring health-related quality of life in relation to aAV was performed.4 Deductive analysis was used to map appropriate areas discussed by patients to the self-management framework proposed by Lorig and Holman.1 inductive analysis was used to identify emergent themes that did not fit into this framework. Results: Interview data were collected from 50 patients with aAV (25 men), from the UK (n=18), USA (n=17) and Canada (n=15). Diagnoses included GPA (n=26), EGPA (n=17) and MPA (n=7). Thirty-four patients were diagnosed within the last 2 years and 16 were diagnosed more than 2 years ago.4 From the core tasks proposed in Lorig and Holman’s framework, patients with aAV appear to attribute particular importance to maintaining life roles and to a lesser extent medication management and dealing with the emotions of chronic disease. Core skills necessary to self-manage in aAV include learning to take action as part of self-efficacy, forming productive health-care partnerships, learning about decision making (requiring disease and self-awareness), utilising resources effectively, and problem-solving. Three themes emerged that were not included in Lorig and Holman’s framework that respondents related to self-management abilities including: 1) support received from family, support groups, and religion; 2) the influence of pre-morbid personality factors; 3) the role patients attributed to developing a foundation of specific aAV knowledge in their subsequent ability to self-manage. Conclusion: Patients with aAV self-manage in a variety of ways and their needs may change over time. Patients may benefit from support to help manage changing life roles and to deal with the challenges of having a chronic disease. Over time, support may need to shift focus onto core skills including recognising when to take action; forming effective partnerships with healthcare providers, family, and friends; adaptations to day-to-day changes in their condition; utilising resources appropriately; and strategies for problem-solving. References [1] Lorig, K.R. And Holman, H.R., 2003. Self-management education: history, definition, outcomes, and mechanisms. Annals of behavioral medicine, 26(1), pp.1-7. [2] NHS England. Five year forward view. Oct2014. Available from:https://www.england.nhs.uk/ourwork/futurenhs/ [3] UK Strategy for Rare Diseases. Department of Health,2013. [4] Robson, J.C., Dawson, J., Cronholm, P.F., Milman, N., Kellom, K.S., ashdown, S., Easley, E., Farrar, J.T., Gebhart, D., Lanier, G. AndMcAlear, C.A., 2018. Health-related quality of life in aNCA-associated vasculitis and item generation for a disease-specific patient-reported outcome measure. Patient related outcome measures, 9, p.17. Disclosure of interests: Tim Reynolds: None declared, Emma Dures Grant/research support from: Has previously received an independent learning grant from Pfizer, however the work has been completed and the grant has been closed., Sue ashdown: None declared, Peter Cronholm: None declared, Raashid Luqmani Grant/research support from: Roche, Vifor and GSK, Peter Merkel: None declared, Nataliya Milman: None declared, Jacqueline Peck: None declared, Joanna Robson: None declared

Neuropathic pain is often debilitating, severely limiting the daily lives of patients who are affected. Typically, neuropathic pain is difficult to manage and, as a result, leads to progression into a chronic condition that is, in many instances, refractory to medical management. Gabapentinoids, belonging to the calcium channel blocking class of drugs, have shown good efficacy in the management of chronic pain and are thus commonly utilized as first-line therapy. Various sodium channel blocking drugs, belonging to the categories of anticonvulsants and local anesthetics, have demonstrated varying degrees of efficacy in the in the treatment of neurogenic pain. Though there is limited medical literature as to efficacy of any one drug, individualized multimodal therapy can provide significant analgesia to patients with chronic neuropathic pain.

Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are multisystem diseases of the small blood vessels. Patients experience irreversible damage and psychological effects from AAV and its treatment. An international collaboration was created to investigate the impact of AAV on health-related quality of life (HRQoL), and develop a disease-specific patient-reported outcome measure to assess outcomes of importance to patients. Methods: Patients with AAV from the UK, USA, and Canada were interviewed to identify salient aspects of HRQoL affected by AAV. The study was overseen by a steering committee including four patient research partners. Purposive sampling of interviewees ensured representation of a range of disease manifestations and demographics. Inductive analysis was used to identify themes of importance to patients; these were further confirmed by a free-listing exercise in the US. Individual themes were recast into candidate items, which were scrutinized by patients, piloted through cognitive interviews and received a linguistic and translatability evaluation. Results: Fifty interviews, conducted to saturation, with patients from the UK, USA, and Canada, identified 55 individual themes of interest within seven broad domains: general health perceptions, impact on function, psychological perceptions, social perceptions, social contact, social role, and symptoms. Individual themes were constructed into >100 candidate questionnaire items, which were then reduced and refined to 35 candidate items. Conclusion: This is the largest international qualitative analysis of HRQoL in AAV to date, and the results have underpinned the development of 35 candidate items for a disease-specific, patient-reported outcome questionnaire., Sponsored by the University of Oxford, the Vasculitis Clinical Research Consortium and the University of Ottawa. With support from the Medical Research Fund, Oxford, the Oxfordshire Health Services Research Committee (Ref 1098), the US National Institute of Arthritis and Musculoskeletal and Skin Diseases (U54 AR057319 and U01 AR51874), the National Center for Research Resources (U54 RR019497), and the Office of Rare Diseases Research. Oxford University Innovation funded the translatability exercise. Additional support received by a Patient-Centred Outcomes Research Institute Pilot Project Grant.

Objectives o finalise and validate a disease-specific patient-reported outcome (PRO) measure: the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire. Using a 35-item candidate questionnaire developed following 50 qualitative interviews in the UK, USA and Canada, a longitudinal survey was conducted to determine the final scale structure and validate the AAV-PRO. Methods Participants were recruited via Vasculitis UK and the Vasculitis Patient-Powered Research Network. The 35-item candidate questionnaire was completed at baseline and 3 months; UK participants completed the EuroQol-5D-5L (EQ-5D-5L), while US participants completed a test–retest exercise, 3–5 days after baseline. Scale structure was defined using exploratory factor analysis (EFA) and Rasch analysis. Convergent and known groups validity, test–retest reliability and longitudinal construct validity were assessed. Results here were 626 participants with AAV; >25% reporting ’active disease’. EFA and Rasch analysis supported a 29-item profile measure comprising six domains: ’organ-specific symptoms’, ’systemic symptoms’, ’treatment side effects’, ’social and emotional impact’, ’concerns about the future’ and ’physical function’. Mean domain scores were higher for participants with ’active disease’ versus ’remission’ (p Conclusions he AAV-PRO, a new disease-specific PRO measure for AAV, has good face and construct validity, is reliable, feasible and discriminates among disease states.

Objective.The antineutrophil cytoplasmic antibody–associated vasculitides (AAV) are multiorgan diseases. Patients with AAV report impairment in their health-related quality of life (HRQOL) and have different priorities regarding disease assessment compared with physicians. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group previously received endorsement for a core set of domains in AAV. Two approaches to measure patient-reported outcomes (PRO) were presented at OMERACT 2016.Methods.A novel 5-step tool was used to facilitate assessment of the instruments by delegates: the OMERACT Filter 2.0 Instrument Selection Algorithm, with a red-amber-green checklist of questions, including (1) good match with domain (face and content validity), (2) feasibility, (3) do numeric scores make sense (construct validity)?, (4) overall ratings of discrimination, and (5) can individual thresholds of meaning be defined? Delegates gave an overall endorsement. Three generic Patient-Reported Outcomes Measurement Information System (PROMIS) instruments (fatigue, physical functioning, and pain interference) and a disease-specific PRO, the AAV-PRO (6 domains related to symptoms and HRQOL), were presented.Results.OMERACT delegates endorsed the use of the PROMIS instruments for fatigue, physical functioning, and pain interference (87.6% overall endorsement) and the disease-specific AAV-PRO instrument (89.4% overall endorsement).Conclusion.The OMERACT Vasculitis Working Group gained endorsement by OMERACT for use of the PROMIS and the AAV-PRO in clinical trials of vasculitis. These instruments are complementary to each other. The PROMIS and the AAV-PRO need further work to assess their utility in longitudinal settings, including their ability to discriminate between treatments of varying efficacy in the setting of a randomized controlled trial.

Objective.Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of linked multisystem life- and organ-threatening diseases. The Outcome Measures in Rheumatology (OMERACT) vasculitis working group has been at the forefront of outcome development in the field and has achieved OMERACT endorsement of a core set of outcomes for AAV. Patients with AAV report as important some manifestations of disease not routinely collected through physician-completed outcome tools; and they rate common manifestations differently from investigators. The core set includes the domain of patient-reported outcomes (PRO). However, PRO currently used in clinical trials of AAV do not fully characterize patients’ perspectives on their burden of disease. The OMERACT vasculitis working group is addressing the unmet needs for PRO in AAV.Methods.Current activities of the working group include (1) evaluating the feasibility and construct validity of instruments within the PROMIS (Patient-Reported Outcome Measurement Information System) to record components of the disease experience among patients with AAV; (2) creating a disease-specific PRO measure for AAV; and (3) applying The International Classification of Functioning, Disability and Health to examine the scope of outcome measures used in AAV.Results.The working group has developed a comprehensive research strategy, organized an investigative team, included patient research partners, obtained peer-reviewed funding, and is using a considerable research infrastructure to complete these interrelated projects to develop evidence-based validated outcome instruments that meet the OMERACT filter of truth, discrimination, and feasibility.Conclusion.The OMERACT vasculitis working group is on schedule to achieve its goals of developing validated PRO for use in clinical trials of AAV.

This workshop was designed to model and engage classroom teachers in reviewing their family experiences to find stories worth telling in hopes that they may support the students in their classrooms to do the same. Sharing our stories helps us to honestly connect with others and to celebrate diversity by recognizing both the similarities and differences in our stories.