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Immune checkpoint inhibitors (ICIs) are associated with a unique set of immune-related adverse events (irAEs). Few studies have evaluated the risk factors and outcomes of patients who develop ICI-induced hepatitis (ICIH).We utilized an institutional database of patients with advanced cancers treated with ICI to identify patients with ICIH. irAEs were graded using the Common Terminology Criteria for Adverse Events v4. Overall survival (OS) was calculated from the date of ICI to death from any cause or the date of the last follow-up. OS with 95% confidence intervals were estimated using the Kaplan-Meier method and stratified by the occurrence of ICIH.We identified 1096 patients treated with ICI. The most common ICIs were PD1/L1 (n = 774) and CTLA-4 inhibitors (n = 195). ICIH occurred among 64 (6%) patients: severity was grade 3 in 30 and ≥ grade 3 in 24 patients (3.1% overall). Median time to ICIH was 63 days. ICIH was more frequent in women (p = 0.038), in patients treated with combination ICIs (p 0.001), and when given as first-line therapy (p = 0.018). Occurrence of ICIH was associated with significantly longer OS, median 37.0 months (95% CI 21.4, NR) compared to 11.3 months (95% CI 10, 13, p 0.001); there was no difference in OS between patients with ≥ grade 3 ICIH vs grade 1-2.Female sex, combination immunotherapy, and the first line of immunotherapy were associated with ICIH. Patients with ICIH had improved clinical survival compared to those that did not develop ICIH. There is a need for prospective further studies to confirm our findings.

INTRODUCTION: OMERACT uses an evidence-based framework known as the 'OMERACT Filter Instrument Selection Algorithm' (OFISA) to guide decisions in the assessment of outcome measurement instruments for inclusion in a core outcome set for interventional and observational clinical trials.METHODS: A group of OMERACT imaging and patient-centered outcome methodologists worked with imaging outcome groups to facilitate the selection of imaging outcome measurement instruments using the OFISA approach. The lessons learned from this work influenced the evolution to Filter 2.2 and necessitated changes to OMERACT's documentation and processes.RESULTS: OMERACT has revised documentation and processes to incorporate the evolution of instrument selection to Filter 2.2. These revisions include creation of a template for detailed definitions of the target domain which is a necessary first step for instrument selection, modifications to the Summary of Measurement Properties (SOMP) table to account for sources of variability, and development of standardized reporting tables for each measurement property.CONCLUSIONS: OMERACT Filter 2.2 represents additional modifications of the OMERACT guide for working groups in their rigorous assessment of measurement properties of instruments of various types, including imaging outcome measurement instruments. Enhanced reporting aims to increase the transparency of the evidence base leading to judgements for the endorsement of instruments in core outcome sets.

Paraneoplastic syndromes are rare diseases caused by malignancies through means other than mass effect or metastasis. Paraneoplastic phenomena can be the first sign of cancer and can be fatal. Paraneoplastic rheumatic syndromes can occur with hematologic cancers, lymphoproliferative disease, and solid tumors. Diseases that feature an advanced age at onset, significant constitutional upset, inadequate response to treatment, and otherwise atypical characteristics should increase the index of suspicion for a paraneoplastic syndrome.

Objective Increased treatment options and longer survival for lung cancer have generated increased interest in patient preferences. Previous studies of patient preferences in lung cancer have not fully explored preference heterogeneity. We demonstrate a method to explore preference heterogeneity in the willingness of patients with lung cancer and caregivers to trade progression-free survival (PFS) with side effects. Patients and methods Patients and caregivers attending a national lung cancer meeting completed a discrete-choice experiment (DCE) designed through a collaboration with patients. Participants answered 13 choice tasks described across PFS, short-term side effects, and four long-term side effects. Side effects were coded as a one-level change in severity (none-mild, mild-moderate, or moderate-severe). A mixed logit model in willingness-to-pay space estimated preference heterogeneity in acceptable tradeoffs (time equivalents) between PFS and side effects. The study was reported following quality indicators from the United States Food and Drug Administration's patient preference guidance. Results A total of 87 patients and 24 caregivers participated in the DCE. Participants would trade 3.7 month PFS (95% CI (CI): 3.3-4.1) for less severe functional long-term treatment side effects, 2.3 months for less severe physical long-term effects (CI: 1.9-2.8) and cognitive long-term effects (CI: 1.8-2.8), 0.9 months (CI: 0.4-1.4) for less severe emotional long-term effects, and 1.8 months (CI: 1.4-2.3) for less severe short-term side effects. Most participants (90%) would accept treatment with more severe functional long-term effects for 8.4 additional month PFS. Conclusion Participants would trade PFS for changes in short-term side effects and long-term side effects, although preference heterogeneity existed. Lung cancer treatments that offer less PFS but also less severe side effects might be acceptable to some patients.

Objective.The Outcome Measures in Rheumatology (OMERACT) Shared Decision Making (SDM) Working Group aims to determine the core outcome domain set for measuring the effectiveness of SDM interventions in rheumatology trials.Methods.A white paper was developed to clarify the draft core domain set. It was then used to prepare for interviews to investigate reasons for lack of consensus on it and to suggest further improvements.Results.OMERACT scientists/clinicians (n = 13) and patients (n = 10) suggested limiting the core domain set to outcome domains, removing process domains, and clarifying remaining domains.Conclusion.A revised core domain set will undergo further consensus-building.

Context/Objectives We sought to characterize patterns of social support types (i.e., emotional, informational, appraisal, and instrumental) within the caregiver/spouse, family, and spiritual/religious contexts for patients diagnosed with cancer. Methods Focus groups were conducted with mixed groups of patients with cancer and caregiver/family members at a Midwestern comprehensive cancer center. Participants completed brief demographic questionnaires. Focus groups were moderated using semistructured interviews. The 90-minute discussions were audio-recorded, transcribed, and uploaded into NVivo for analysis using a deductive approach based on four social support types and the constant comparative method. Results Four focus groups were conducted (n = 25). The average age was 58.4 years (SD = 15.1, range 26.0–76.0). Patient participants reported different malignancy types, including breast, gynecologic, skin, oral, and Non-Hodgkin's lymphoma. Participants acknowledged changes within their social network across the cancer journey. Overall, the caregiver/spouse fulfilled all types of social support. Spirituality/religion was often discussed as a form of appraisal social support. Fellow survivors were sources of informational support. Across groups, nondirective/emotional support was most frequently mentioned. Conclusion Cancer is a unique experience, and understanding the importance of social support, including types of social support needed from different contexts to best meet the needs of the patient, may promote optimal, patient-centered care across the cancer trajectory.

The current review highlights the growing number of available methods used to measure patient preferences and discusses how this impacts preference research in rheumatology. Spurred by the growing role of preferences in regulatory decisions and drug development, researchers have begun applying preference methods to study questions beyond the clinical context. We explore these trends, provide case studies highlighting changes in measuring patient preferences, compare strengths and weaknesses of common stated-preference methods, and discuss considerations for the future use of these methods.Early literature on patient preferences often mimicked clinical practice, asking whether treatment A is better or worse than treatment B for a patient. Early applications of patient preference methods in rheumatology aimed to value different attributes of treatments, but remained focused on informing clinical questions. Spurred by interest in preferences by regulatory agencies and patient-centeredness throughout the product lifecycle, there are now a wide array of methods available to measure preference.Although these different preference methods have strengths and weaknesses, they serve to highlight the broad number of questions that could help rheumatology beyond the clinical context. Researchers in rheumatology now have the opportunity to better serve diverse stakeholders by considering how these methods could aid in clinical trial design, regulatory policy, and other elements of the medical product life cycle.

Introduction Immune checkpoint inhibitors (ICI) are associated with unique immune-related adverse events (irAEs). Immune-related thrombocytopenia (irTCP) is an understudied and poorly understood toxicity; little data are available regarding either risk of irTCP or the effect of irTCP on clinical outcomes of patients treated with ICI. Methods We conducted a retrospective review of sequential cancer patients treated with ICI between 2011 and 2017 at our institution. All patients who received ICI alone or in combination with other systemic therapy in any line of treatment were included; those with thrombocytopenia ≥ grade 3 at baseline were excluded. We calculated the incidence of ≥ grade 3 irTCP and overall survival (OS). Patient factors associated with irTCP were assessed. Results We identified 1,038 patients that met eligibility criteria. Overall, 89 (8.6%) patients developed grade ≥ 3 thrombocytopenia; eighteen were attributed to ICI (1.73% overall). Patients who developed grade ≥ 3 irTCP had worse overall survival compared to those whose thrombocytopenia was unrelated to ICI (4.17 vs. 10.8 month; HR. 1.94, 95% CI 1.13, 3.33; log-rank p = 0.0164). Patients with grade ≥ 3 irTCP also had worse survival compared to those without thrombocytopenia (4.17 vs. 13.31 months; HR 2.22, 95% CI 1.36, 3.62; log-rank p = 0.001). The incidence of irTCP appeared lowest among those treated with PD-1/L1 monotherapy (p = 0.059) and was not associated with cancer type, smoking status, age, gender, race, or line of therapy. Conclusions Unlike other irAEs, we found that irTCP was associated with worse overall survival. The incidence of irTCP appeared lowest among those treated with PD-1/L1 monotherapy.

Objective: To gain consensus on the Outcome Measures in Rheumatology (OMERACT) core domain set for rheumatology trials of shared decision making (SDM) interventions.Methods: The process followed the OMERACT Filter 2.1 methodology, and used consensus-building methods, with patients involved since the inception. After developing the draft core domain set in previous research, we conducted five steps: (i) improving the draft core domain set; (ii) developing and disseminating whiteboard videos to promote its understanding; (iii) conducting an electronic survey to gather feedback on the draft core domain set; (iv) finalizing the core domain set and developing summaries, a plenary session video and discussion boards to promote its understanding; and (v) conducting virtual workshops with voting to endorse the core domain set.Results: A total of 167 participants from 28 countries answered the survey (62% were patients/caregivers). Most participants rated domains as relevant (81%-95%) and clear (82%-93%). A total of 149 participants (n = 48 patients/caregivers, 101 clinicians/researchers) participated in virtual workshops and voted on the proposed core domain set which received endorsement by 95%. Endorsed domains are: 1-Knowledge of options, their potential benefits and harms; 2-Chosen option aligned with each patient's values and preferences; 3-Confidence in the chosen option; 4-Satisfaction with the decision-making process; 5-Adherence to the chosen option and 6-Potential negative consequences of the SDM intervention.Conclusion: We achieved consensus among an international group of stakeholders on the OMERACT core domain set for rheumatology trials of SDM interventions. Future research will develop the Core Outcome Measurement Set.Clinical significance: Prior to this study, there had been no consensus on the OMERACT core domain set for SDM interventions. The current study shows that the OMERACT core domain set achieved a high level of endorsement by key stakeholders, including patients/caregivers, clinicians and researchers.(c) 2021 Elsevier Inc. All rights reserved.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a group of small vessel vasculitides characterized by granulomatous and neutrophilic tissue inflammation, often associated with the production of antibodies that target neutrophil antigens. The two major antigens targeted by ANCAs are leukocyte proteinase 3 (PR3) and myeloperoxidase (MPO). AAV can be classified into 3 categories based on patterns of clinical involvement: namely, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA). Clinically, AAV involves many organ systems including the lungs, kidneys, skin, and nervous system. The prognosis of AAV has improved dramatically due to advances in the understanding of its pathogenesis and treatment modalities. This review will highlight some of the recent updates in our understanding of the pathogenesis, clinical manifestations, and treatment options in patients with AAV focusing on kidney involvement.

No evidence-based guidelines exist for the management of rheumatic irAEs. The goal of management is to control irAEs while preserving the drug’s antitumor immune response. Glucocorticoids are the first-line agent, but they should be used with caution and limited in both dose and duration if possible. Early referral to rheumatology is the key to addressing the issues in these complex patients. More human trials using DMARDs and biologic treatments are necessary.

An unmet need exists for reliable, validated, and widely-accepted outcome measures for randomized clinical trials in Behçet's syndrome. The Outcome Measures in Rheumatology (OMERACT) Behçet's Syndrome Working Group, a large, multidisciplinary group of experts in Behçet's syndrome and patients with Behçet's syndrome, had an objective of developing a core set of data-driven outcome measures for use in all clinical trials of Behçet's syndrome.The core domain set was developed through a comprehensive, iterative, multistage project that included a systematic review, a focus group meeting and qualitative patient interviews, a survey among experts in Behçet's syndrome, a Delphi exercise involving both patients and physician experts in Behçet's syndrome, and use of the data, insight, and feedback generated by these processes to develop a final core domain set.All steps were completed and domains were delineated across the organ systems involved in this disease. Since trials in Behçet's syndrome often focus on specific manifestations and not on the disease in its entirety, the final proposed core set includes 5 domains mandatory for study in all trials in Behçet's syndrome (disease activity, new organ involvement, quality of life, adverse events, and death) with additional subdomains mandatory for study of specific organ-systems. The final core set was endorsed at the 2018 OMERACT meeting.The core set of domains in Behçet's syndrome provides the foundation through which the international research community, including clinical investigators, patients, the biopharmaceutical industry, and government regulatory bodies can harmonize the study of this complex disease, compare findings across studies, and advance development of effective therapies.

TopicThis paper reviews the ocular side effects of medications used in the treatment of rheumatological conditions.Clinical relevanceRheumatic diseases are inflammatory conditions that may affect the skin, blood vessels, joints, muscles and internal organs. Immunosuppressive agents are often used as treatment and while powerful, they carry side effects and toxicities that need careful monitoring. Ophthalmic complications have been reported with the use of antirheumatic medications; however, there is a lack of literature synthesising these reports. This paper addresses this gap and hopes to inform both rheumatologists and ophthalmologists as they work together on the management of patients with rheumatological conditions.MethodsPubMed literature search was conducted from November to September 2019 searching for ocular side effects with the use of 25 rheumatological drugs.ResultsA total of 111 papers were included in this review. Adverse side effects were divided into non-infectious and infectious causes. Traditional disease-modifying antirheumatic drugs (DMARDs) were associated with pruritus, irritation and dryness of the conjunctiva while biologic DMARDS showed reports of new-onset/recurrent uveitis and demyelinating conditions. Infectious side effects included episodes of cytomegalovirus retinitis, toxoplasmic chorioretinitis and endophthalmitis. Other serious side effects were encountered and included in this review.ConclusionThe goal of this paper is to inform healthcare providers about potential ocular side effects from rheumatological medications. Healthcare providers are encouraged to learn more about these ophthalmic complications and find relevance within their clinical practice.

Objective Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that can affect the central nervous system in multiple ways, including causing cognitive dysfunction. Cognitive dysfunction is a common complaint of SLE patients yet diagnosis is challenging, time consuming, and costly. This study evaluated the Self-Administered Gerocognitive Exam (SAGE) as a screening test for cognitive impairment in a cohort of SLE patients. Methods A total of 118 SLE patients completed the SAGE. Providers completed the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the Systemic Lupus International Collaborative Clinics Damage Index (SLICC-DI). SAGE scores were grouped into normal (>16) and abnormal (≤16) categories. Univariate and multivariate analyses were performed. Results Of the 118 participants, 21(18%) scored ≤16 on the SAGE instrument. In univariate analysis, race, ethnicity, household income, and SLICC-DI scores were associated with the SAGE ( p $50,000, 95% CI 2.45–57, p = 0.002). Conclusions In SLE patients, this study demonstrates an independent relationship between neurocognitive impairment (as measured by the SAGE) and higher lupus-related damage, as measured by the SLICC-DI, and lower household income. Abnormal SAGE scores were also associated with Hispanic ethnicity. A language barrier could explain this because the SAGE instrument was conducted in English only. The SAGE was feasible to measure in the clinic setting.

SARS-CoV-2 has been described to cause "COVID [coronavirus disease] toes," a chilblains-like syndrome.1,2,3 A 33-year-old White woman was evaluated in the hospital for painful new-onset blue and purple nodules on the tips of 3 fingers and 2 toes, 7 days after receiving the Moderna SARS-CoV-2 vaccine (Figure 1).

Objective.The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group has been working toward developing a data-driven core set of outcome measures for use in clinical trials of Behçet’s syndrome [Behçet disease (BD)]. This paper summarizes the group’s work through OMERACT 2016, discussions during the meeting, and the future research agenda.Methods.Qualitative patient interviews were conducted among 20 patients with BD who have different types of organ involvement. A 3-round Delphi among BD experts and patients was initiated to identify domains, subdomains, and outcomes to be assessed in clinical trials of BD. The results of these studies were discussed during OMERACT 2016 and next steps were planned.Results.Patients’ perspectives and priorities were identified through qualitative interviews that identified candidate domains and subdomains for inclusion in the Delphi and characterized some shortcomings of the currently used patient-reported outcomes in BD. The first round of the Delphi was completed and several domains or subdomains were endorsed by the experts and/or the patients. Because many more items were endorsed than would be feasible to assess during a clinical trial, rating and ranking of items by physicians and patients was planned as a next critical step. The challenges of assessing specific organ system involvement was also discussed.Conclusion.The OMERACT Behçet Syndrome Working Group research program will identify core domains for assessment in BD with the goal of developing a core set of outcome measures for use in all trials of BD with the option to incorporate additional outcomes for specific organ involvement.

ObjectiveThe heterogeneous multisystem manifestations of SLE include fatigue, pain, depression, sleep disturbance and cognitive dysfunction, and underscore the importance of a multidimensional approach when assessing health-related quality of life. The US Food and Drug Administration has emphasised the importance of patient-reported outcomes (PROs) for approval of new medications and Outcome Measures in Rheumatology has mandated demonstration of appropriate measurement properties of selected PRO instruments.MethodsPublished information regarding psychometric properties of the Medical Outcomes Survey Short Form 36 (SF-36), Lupus Quality of Life Questionnaire (LupusQoL) and Functional Assessment of Chronic Illness Therapy-Fatigue Scale (FACIT-F), and their suitability as end points in randomised controlled trials (RCTs) and longitudinal observational studies (LOS) were assessed. A search of English-language literature using MEDLINE and EMBASE identified studies related to development and validation of these instruments. Evidence addressed content validity, reliability (internal consistency and test-retest reliability), construct validity (convergent and divergent) and longitudinal responsiveness, including thresholds of meaning and discrimination.ResultsAll instruments demonstrated strong internal consistency, reliability and appropriate face/content validity, indicating items within each instrument that measure the intended concept. SF-36 and LupusQoL demonstrated test-retest reliability; although not published with FACIT-F in SLE supported by evidence from other rheumatic diseases. All instruments demonstrated convergent validity with other comparable PROs and responsivity to treatment.ConclusionThe measurement properties of PRO instruments with published data from RCTs including: SF-36, LupusQoL and FACIT-F indicate their value as secondary end points to support labelling claims in RCTs and LOS evaluating the efficacy of SLE treatments.

Outcome Measures in Rheumatology Conference (OMERACT) -- MAY 15-18, 2018 -- Terrigal, AUSTRALIA Gul, Ahmet/0000-0001-8219-3720; ALIBAZ-ONER, FATMA/0000-0002-6653-1758; Hill, Catherine/0000-0001-8289-4922; Mahr, Alfred/0000-0002-1564-9115 WOS:000484027700024 PubMed ID: 30877212 Objective. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group seeks to develop validated outcome measures for use in trials for large-vessel vasculitis (LVV). Methods. An international Delphi exercise conducted among investigators identified items considered important to measure active disease. In parallel, qualitative research with patients was conducted, including interviews and focus groups. Results. Next steps prioritized by the group for LVV include (1) defining disease states (remission, flare, and patient-acceptable symptom state) and (2) selection of patient-reported outcome tools. Conclusion. The ultimate goal is to develop an OMERACT-endorsed core set of outcome measures for use in clinical trials of LVV. Outcome Measures Rheumatol US Vasculitis Clinical Research Consortium (VCRC); NCATSUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Center for Advancing Translational Sciences (NCATS) [U54 AR057319]; National Institute of Arthritis and Musculoskeletal and Skin DiseasesUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute of Arthritis & Musculoskeletal & Skin Diseases (NIAMS) [U54 AR057319]; National Center for Research ResourcesUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Center for Research Resources (NCRR) [U54 RR019497]; NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASESUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute of Arthritis & Musculoskeletal & Skin Diseases (NIAMS) [U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319, U54AR057319] Funding Source: NIH RePORTER This work was funded in part by the US Vasculitis Clinical Research Consortium (VCRC). The VCRC is part of the Rare Diseases Clinical Research Network, an initiative of the Office of Rare Diseases Research, National Center for Advancing Translational Science (NCATS). The VCRC is funded through collaboration between NCATS, and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (U54 AR057319), and has received funding from the National Center for Research Resources (U54 RR019497).

Background Medication decision-making is challenging in lupus. No validated, effective decision-aids are available to assist patients with medication decision-making. Objectives Our objective was to assess the effectiveness of an individualized, culturally-tailored, computerized decision-aid for immunosuppressive medications for lupus nephritis. Methods In a multicenter, randomized controlled trial, diverse adult women with lupus nephritis, largely racial/ethnic minorities with low socio-economic status, were randomized to decision-aid vs. American College of Rheumatology lupus pamphlet (1:1 ratio). Co-primary outcomes were change in decisional conflict and informed choice regarding immunosuppressive medications. Results Of 301 randomized women, 47% were African-American, 26% were Hispanic, and 15% White. Mean age (standard deviation [SD]) was 37 (12) years, 57% had annual income of Conclusion An individualized decision-aid was effective in reducing decisional conflict for immunosuppressive medications in diverse women with lupus nephritis. Disclosure of Interests jasvinder singh Shareholder of: Amarin pharmaceuticals and Viking therapeutics, Consultant for: Crealta/Horizon, Fidia, UBM LLC, Medscape, WebMD, the National Institutes of Health and the American College of Rheumatology, Liana Fraenkel: None declared, Candace Green: None declared, Graciela S Alarcon: None declared, Jennifer Barton: None declared, Kenneth Saag Grant/research support from: Amgen, Ironwood/AstraZeneca, Horizon, SOBI, Takeda, Consultant for: Abbvie, Amgen, Ironwood/AstraZeneca, Bayer, Gilead, Horizon, Kowa, Radius, Roche/Genentech, SOBI, Takeda, Teijin, Leslie Hanrahan: None declared, Sandra Raymond: None declared, Robert Kimberly: None declared, Amye Leong: None declared, Elyse Reyes: None declared, Richard Street: None declared, Maria Suarez-Almazor : None declared, Guy Eakin: None declared, Laura Marrow: None declared, Charity Morgan: None declared, Brennda Caro: None declared, Jeffrey Sloan: None declared, Bochra Jandali: None declared, Salvador Garcia: None declared, Jennifer Grossman: None declared, Kevin Winthrop Consultant for: Gilead, Galapagos, Eli Lilly and Company, Abbvie, Pfizer, GSK, Laura Trupin: None declared, Maria Dall’Era Grant/research support from: University has received funds to serve as a site on this clinical study, Consultant for: On Data Monitoring Committee for Janssen, Biogen, and Genentech; on Steering Committee for EMD Serono., Alexa Meara: None declared, Tara Rizvi: None declared, Winn Chatham: None declared, Jinoos Yazdany Grant/research support from: Pfizer, Consultant for: AstraZeneca

Background: There is an unmet need for reliable, validated, and widely-accepted outcome measures for clinical trials in Behcet’s syndrome (BS). Objectives: The Outcome Measures in Rheumatology Clinical Trials (OMERACT) Behcet’s Syndrome Working Group has worked to advance the creation of a data-driven Core Domain Set for use in all clinical trials. Methods: The Core Domain Set was developed through a comprehensive, iterative, multi-stage, multi-year project that followed the methodologically rigorous processes and standards set forth by OMERACT: i) a systematic review; ii) a survey among experts in BS; iii) an outcome measures interest group meeting during the International Conference on Behcet’s Disease; iv) qualitative patient interviews; v) a three-round modified Delphi exercise involving both patients with BS and a multidisciplinary set of physicians expert in BS, focused on obtaining consensus on the domains of illness necessary in the study of BS; and vi) utilization of the data, insight, and feedback generated by the outlined processes to develop a final Core Domain Set. The final Core Set was presented and put up for a vote of endorsement at the 2018 OMERACT meeting. Results: All steps in the processes outlined were completed. The systematic review clearly demonstrated the substantial variability in the domains studied in clinical trials of BS and a lack availability of validated outcome measures in BS. The survey of physicians, the in-person meeting of experts, and the qualitative research with patients all helped generate an extensive list of candidate domains and sub-domains to consider for use in clinical trials. It also become clear that there was a need and strong interest in delineating domains across the several major organ systems involved in this disease and in recognizing that clinical trials in BS often focus on specific manifestations and not the disease in its entirety. The Delphi involved 74 physicians expert in BS from 21 countries and from a wide range of specialties, and 64 patients from 10 countries. The Delphi utilized both ratings and rankings to prioritize the 56 domains and sub-domains originally under consideration. The final proposed Core Set included 5 sub-domains mandatory for study in all trials in BS, with additional sub-domains mandatory for study of specific organ-systems when that system is the focus of a trial: mucocutaneous (2 additional sub-domains), ocular (4), central nervous system (3), musculoskeletal (2), vascular (4), and gastrointestinal (2). The final Core Set was strongly endorsed at the 2018 OMERACT meeting. Conclusion: Multiple disease-related domains in BS have been identified by physicians and patients as important to address in clinical trials, leading to the development and endorsement of a final Core Set of Domains for use in clinical trials in BS. The Core Set provides the foundation through which the international research community, including clinical investigators, patients, the biopharmaceutical industry, and government regulatory bodies can harmonize the study of this complex disease, compare findings across studies, and advance development of effective agents. Disclosure of Interests: : Gulen Hatemi Consultant for: Abbvie, Amgen, BMS, Janssen, MSD, Pfizer, UCB, Speakers bureau: Abbvie, Amgen, BMS, Jansen, MSD, Pfizer, UCB, Alexa Meara: None declared, Yesim Ozguler: None declared, Haner Direskeneli: None declared, Alfred Mahr: None declared, Beverley Shea: None declared, Esen Cam: None declared, Ahmet Gul: None declared, Yusuf Yazici Shareholder of: Samumed, LLC, Consultant for: Celgene Corporation, BMS, Genentech, Sanofi, Employee of: Samumed, LLC, Peter Tugwell: None declared, Hasan Yazici: None declared, Peter Merkel: None declared

Background Treatment decision-making regarding immunosuppressive therapy is challenging for individuals with lupus. We assessed the effectiveness of a decision aid for immunosuppressive therapy in lupus nephritis. Methods and findings In a United States multicenter, open-label, randomized controlled trial (RCT), adult women with lupus nephritis, mostly from racial/ethnic minority backgrounds with low socioeconomic status (SES), seen in in- or outpatient settings, were randomized to an individualized, culturally tailored, computerized decision aid versus American College of Rheumatology (ACR) lupus pamphlet (1:1 ratio), using computer-generated randomization. We hypothesized that the co-primary outcomes of decisional conflict and informed choice regarding immunosuppressive medications would improve more in the decision aid group. Of 301 randomized women, 298 were analyzed; 47% were African-American, 26% Hispanic, and 15% white. Mean age (standard deviation [SD]) was 37 (12) years, 57% had annual income of, Jasvinder Singh and colleagues reveal how a decision aid helps women with lupus to make decisions about immunosuppressive therapy., Author summary Why was this study done? Lupus kidney disease is a serious immune system condition that can lead to kidney failure and dialysis in young women, if not treated appropriately. Effective treatment with immune-blocking medicines is available, but patients report difficulty making informed decisions that weigh benefits and risks, such as side effects. No patient decision aids are available for lupus medication decision-making. The research team made an online decision aid for women with lupus kidney disease, with extensive input from individuals with lupus. The aid can be individualized to help patients make informed decisions about immune-blocking medicines for lupus. What did the researchers do and find? In 301 women with lupus kidney disease across four centers, researchers assessed whether an online decision aid improved lupus treatment decisions, compared with the use of a paper pamphlet on lupus kidney disease from the American College of Rheumatology. Compared with women who received the lupus pamphlet, women who used the decision aid reported feeling less doubt about their medication choice. More women who used the online decision aid said that the information source was easy to use, compared with women who used the lupus pamphlet. What do these findings mean? This study provides evidence that a decision aid for women with kidney disease may help patients feel more confident in their treatment choices.

The purpose of the current study was to characterize the experiences of cancer patients and their caregiver/family members around their relationship with their oncologist, health care team, and the hospital environment. Participants were recruited from The Ohio State University Comprehensive Cancer Center. Participant sociodemographic factors were assessed. Focus groups were moderated and recorded by two members of the research team using a semi-structured interview format. The audio recordings were transcribed and uploaded to NViVO 11 for analyses. Four focus groups were conducted with 25 participants. The mean age of participants was 58.4 years (SD = 15.1, range 26.0–76.0). Participants who were identified as patients (84%) reported different malignancy types including breast (56%), gynecologic (16%), skin (6%) oral (6%), and non-Hodgkin’s lymphoma (6%). Three major themes that emerged around the patient-oncologist relationship, include (1) choosing a physician and health care location, (2) relationship with the physician, health care team, and hospital environment; and (3) patient engagement and decision-making. Subthemes highlighted the importance of the flexible communication behaviors and trustworthiness of the oncologist, and the impact of other health care team members. Patients also reported the desire to be engaged in making treatment-related decisions and to include the caregiver/spouse in all stages of cancer care. Understanding the experience of cancer patients in a relationship with their oncologist in the context of the health care team and health care environment will be an important area of future research to provide optimal, tailored patient-centered cancer care.

INTRODUCTION The patient-physician relationship is a critical component of patient-centred health care. The patient-oncologist relationship is particularly important due to the uncertainties that surround treatment of cancer. The goal of the current review was to summarise current methodological approaches to studying the relationship between cancer patients and oncologists. METHODS A systematic review using PsychInfo, Ebsco, PubMed and Google Scholar was performed using combinations and variations of the MESH terms: "relationship," "doctor-patient," and "oncology." The included studies explicitly measured the "relationship" as an independent or dependent variable. Data were extracted and analysed. RESULTS The 13 studies included in the review were published from 2004 to 2018. There was little agreement between studies on the definition of the patient-oncologist relationship. Trust was most frequently measured, but methods varied. Most studies evaluated the patient perspective (n = 10). The few studies that considered the oncologist perspective did not measure their perception of the relationship. CONCLUSIONS The current review demonstrates that current approaches used to assess the patient-oncologist relationship are inconsistent. These differences may limit our understanding of patient needs in current research and practice. Future research should focus on the use of a relational lens as a theoretical framework to assess the patient-oncologist relationship.

Background Systemic lupus erythematous (SLE) is a heterogeneous disease with high morbidity and mortality with complex long-term treatments. These dynamic treatments can be daunting especially to the 25%–60% of SLE patients who have cognitive and neuropsychiatric deficits. Patients lacking understanding of their own baseline health status and treatment options cannot effectively collaborate in making informed choices with their physicians. Objectives This project aimed to identify SLE patients’ comprehension of their medication regimens and disease outcomes in relation to standard markers of disease activity (SLEDAI) and damage (SLICC DI). Methods Patients>18 years were recruited from The Ohio State University (OSU) Lupus Vasculitis Glomerulonephritis (LVG) clinic. An IRB-approved 25-item true/false disease questionnaire was administered to 75 SLE patients who provided informed consent. Individual question and composite scores for each patient were correlated with their SLEDAI and SLICC DI scores. To our knowledge, a disease comprehension questionnaire has never been used in lupus patients. Results 75 SLE patients completed the comprehension questionnaire. Lower comprehension was associated with greater disease activity (r=−0.14), while no correlation was found between composite score and disease damage (SLICC DI) (r=−0.03). Figures 1 plot the comprehension composite scores against the SLEDAI. P values were not significant, but trending to correlate with the r. Evaluation of individual questionnaire items demonstrated: approximately 80% of patients did not associate heart disease with lupus, over 25% of patients did not recognise the side effects of prednisone, and over 15% of patients did not know lupus affects bone health. Conclusions These preliminary results suggest that patients with more understanding of their SLE diagnosis, comorbidities, and treatments had less measured disease activity. The comprehension questionnaire and SLEDAI characterise patients in their present disease state, so this correlation likely reflects the evolution of patient understanding and the fluctuating nature of their disease. Alternatively, higher disease activity scores represent more severe disease, which could be associated with greater neurocognitive deficits leading to poorer scores on the comprehension questionnaire. The lack of correlation between composite score and SLICC DI is likely attributed to the discrepancy between a metric evaluating current knowledge and a long-term indicator that may derive from decisions made when patients’ comprehension was different than current. These initial results are promising and may represent a cost-effective opportunity for physicians to evaluate and address their patients’ comprehension gaps in an effort to improve shared decision making. However, more data are needed to test the robustness of these trends. Disclosure of Interest None declared

Background Systemic lupus erythematous (SLE) is a heterogeneous disease with high morbidity and mortality; requiring complex treatment plans which can be daunting especially to SLE patients with neuropsychiatric deficits. Numeracy is the “ability to use and understand numbers in daily life” and a key component for effective shared-decision making between physician and patient to ensure the best patient outcomes. Numeracy skills are needed for decisions about health care risk stratification when understanding treatments, understanding potential harm and benefits of therapy and for counting pills and taking medications as prescribed. Objectives As there is a lack of information within the lupus patient population, this proposal explores the relationship between patients’ numeric ability (both actual and perceived ability) and clinical outcomes fulfilling a gap in our current knowledge of how to support physician communication with patients to ensure patient understanding. Methods Patients>18 recruited from IRB approved The Ohio State University Lupus, Vasculitis, Glomerulonephritis Registry. 112 patients had finished subjective numeracy (SNS) which measures patients’ perception of their math ability, objective numeracy (ONS) which measures patients’ actual math ability. We conducted regression analyses with ONS, SNS, and demographics as predictors of disease activity (measured by SLEDAI). We used backwards-stepwise regression, removing nonsignificant variables one at a time from the model. Results Logistic regression analysis revealed a significant interaction between ONS and SNS in predicting whether patients had the active disease or not (interaction b(SE)=-0.70 (0.33), p=0.03; ONS b(SE)=-0.13 (0.30), p=0.68; SNS b(SE)=-0.40 (0.32), p=0.21). Patients higher in both numeric competencies were least likely to have the active disease. Specifically, among those higher in ONS (+1 SD), the predicted probability of having the active disease was 11% vs. 52% for those higher vs. lower in SNS (±1 SD), respectively. In comparison, among those lower in ONS (−1SD), the likelihood of active disease was 38% vs 25% for those higher vs. lower in SNS, respectively. Thus, among patients with greater math abilities, having greater beliefs in one’s abilities appeared beneficial; however, among patients with lower math abilities, greater ability beliefs appeared harmful. Conclusions Patients with greater math ability, coupled with greater beliefs in one’s numeric ability, were least likely to have the active disease. This is consistent with psychological theory about actual math ability and perceived math ability as an outcome predictor. Patients that are overconfident and more active in their health and numeric tasks may make decisions that lead them to worse outcomes. The SNS and ONS may be an efficient screening tool to identify high risk SLE patients that may require extra health care needs. Disclosure of Interest None declared

OBJECTIVE To evaluate how demographics, health literacy, numeracy, and patient activation are related to transition readiness in adolescent and young adult (AYA) patients and to describe how parent/guardian (PG) performance on these metrics predicts AYA patients' transition readiness. METHODS In this single center, cross-sectional study, consecutive English-speaking AYA patients ages 17-21 years and PGs were recruited from outpatient rheumatology clinics. Participants completed the following self-reported instruments: demographic questionnaire, Short Test of Fundamental Health Literacy, Objective Numeracy Scale, Subjective Numeracy Scale, Symbolic-number mapping, Patient Activation Measure, and Transition Readiness Assessment Questionnaire (TRAQ; AYA patients only). RESULTS Ninety-one AYA patients participated in the study, of whom 64 of 91 (70%) had juvenile idiopathic arthritis, and 54 PGs. Mean ± SD TRAQ score was 4.0 ± 0.65, correlating with "I am starting to do this" stage of change. Most participants (98%) had adequate health literacy. Multivariable regression analysis showed that AYA patients of female sex, older age, and higher patient activation significantly predicted higher TRAQ scores (P < 0.05). No PG characteristics were linked to higher AYA patient TRAQ scores. CONCLUSIONS Transition readiness in AYA patients as measured by TRAQ is associated with female sex, older age, and higher patient activation. Though sex and age are nonmodifiable, interventions to boost patient activation represent a promising opportunity to improve transition readiness and outcomes.

Background Over the last 20 years, there have been marked improvements in the availability of effective medications for rheumatic conditions such as gout, osteoporosis and rheumatoid arthritis (RA), which have led to a reduction in disease flares and the risk of re-fracture in osteoporosis, and the slowing of disease progression in RA. However, medication adherence remains suboptimal, as treatment regimens can be complex and difficult to continue long term. Many trials have been conducted to improve adherence to medication. Core domains, which are the outcomes of most relevance to patients and clinicians, are a pivotal component of any trial. These core domains should be measured consistently, so that all relevant trials can be combined in systematic reviews and meta-analyses to reach conclusions that are more valid. Failure to do this severely limits the potential for trial-based evidence to inform decisions on how to support medication adherence. The Outcome Measures in Rheumatology (OMERACT) – Interventions for Medication Adherence study by the OMERACT-Adherence Group aims to develop a core domain set for interventions that aim to support medication adherence in rheumatology. Methods/design This OMERACT-Adherence study has five phases: (1) a systematic review to identify outcome domains that have been reported in interventions focused on supporting medication adherence in rheumatology; (2) semi-structured stakeholder interviews with patients and caregivers to determine their views on the core domains; (3) focus groups using the nominal group technique with patients and caregivers to identify and rank domains that are relevant to them, including the reasons for their choices; (4) an international three-round modified Delphi survey involving patients with diverse rheumatic conditions, caregivers, health professionals, researchers and other stakeholders to develop a preliminary core domain set; and (5) a stakeholder workshop with OMERACT members to review, vote on and reach a consensus on the core domain set for interventions to support medication adherence in rheumatology. Discussion Establishing a core domain set to be reported in all intervention studies undertaken to support patients with medication adherence will enhance the relevance and the impact of these results and improve the lives of people with rheumatic conditions. Electronic supplementary material The online version of this article (10.1186/s13063-018-2565-z) contains supplementary material, which is available to authorized users.

Objective.The aim of this Outcome Measures in Rheumatology (OMERACT) Working Group was to determine the core set of outcome domains and subdomains for measuring the effectiveness of shared decision-making (SDM) interventions in rheumatology clinical trials.Methods.Following the OMERACT Filter 2.0, and based on a previous literature review of SDM outcome domains and a nominal group process at OMERACT 2014, (1) an online Delphi survey was conducted to gather feedback on the draft core set and refine its domains and subdomains, and (2) a workshop was held at the OMERACT 2016 meeting to gain consensus on the draft core set.Results.A total of 170 participants completed Round 1 of the Delphi survey, and 116 completed Round 2. Respondents came from 29 countries, with 49% being patients/caregivers. Results showed that 14 out of the 17 subdomains within the 7 domains exceeded the 70% criterion (endorsement ranged from 83% to 100% of respondents). At OMERACT 2016, only 8% of the 96 attendees were patients/caregivers. Despite initial votes of support in breakout groups, there was insufficient comfort about the conceptualization of these 7 domains and 17 subdomains for these to be endorsed at OMERACT 2016 (endorsement ranged from 17% to 68% of participants).Conclusion.Differences between the Delphi survey and consensus meeting may be explained by the manner in which the outcomes were presented, variations in participant characteristics, and the context of voting. Further efforts are needed to address the limited understanding of SDM and its outcomes among OMERACT participants.

Background Despite numerous studies indicating the positive effects of exercise and psychological stress reduction in patients with autoimmune disease, these therapeutic modalities are currently underemphasized due to the absence of comprehensive immunological characterization and regimen standardization. Objectives In order to examine the influence on the immune system at the cellular and tissue level, disease pathology was analyzed in the NZM2410 mouse model of lupus nephritis. To translate these results and begin to characterize a consensus treatment regimen, a pilot cohort of systemic lupus erythematosus (SLE) patients with active disease was enrolled into a daily Tai Chi program, which emphasized moderate exercise levels with meditative breathing to provide daily physical activity and stress reduction. Methods Mice were exercised daily by treadmill walking at moderate intensity. Social disruption stress was induced in mice by disturbing the social order within an established hierarchy. All mice were removed from the study when experimental removal criteria was reached [blood urea nitrogen (BUN)>50 mg/dL; weight loss>20%]. Kidney tissue and serum were collected from mice at experimental endpoint. SLE patients completed daily Tai Chi exercises and data was collected at baseline and throughout the study via questionnaires to access physical activity and stress levels, activity trackers (Fitbit), and serum sample analysis. Results Histopathological analysis of NZM2410 mice demonstrated that psychosocial stress induction significantly exacerbated and daily moderate exercise significantly reduced lupus nephritis disease pathology, as measured by BUN levels, complement component 3 and IgG complex deposition in glomeruli, pathological grading of H&E-stained kidney sections, and renal macrophage infiltration. Furthermore, stressors induced levels of IL-6, TNF-α, and IL-1β, while exercise suppressed IL-6, TNF-α, IL-10, and CXCL1 in mice. Compared to baseline data, questionnaires confirmed a significant reduction in perceived social stress and an increase in combined metabolic equivalent of task (MET) and overall physical activity in SLE patients. Moreover, fitness activity tracker data showed a significant increase in steps, distance, and activity calories with no changes in body mass index or vigorous activity levels. Interestingly, this correlated with an increased average time in bed each night. Analysis of pro-inflammatory serum cytokine expression revealed suppression in the relative fold change of IL-6 by 23%, IL-8 by 30%, TNF-α by 11%, and IFN-γ by 21% with Tai Chi. Conclusions These data suggest that moderate exercise and stress management can have potent immunoregulatory effects on the chronic, systemic inflammation associated with SLE and identify daily Tai Chi exercise as a viable adjunct therapy to compliment current pharmacological interventions. References Wang C, et al. A randomized trial of tai chi for fibromyalgia. The New England journal of medicine 2010, 363(8): 743–754. Acknowledgements Funding: Center for Integrative Health and Wellness and Wexner Medical Center. Disclosure of Interest None declared

SLE is a chronic inflammatory disease that affects the kidneys in about 50% of patients. Lupus nephritis is a major risk factor for overall morbidity and mortality in SLE, and despite potent anti-inflammatory and immunosuppressive therapies still ends in CKD or ESRD for too many patients. This review highlights recent updates in our understanding of disease epidemiology, genetics, pathogenesis, and treatment in an effort to establish a framework for lupus nephritis management that is patient-specific and oriented toward maintaining long-term kidney function in patients with lupus.

©2016 Turkish League Against Rheumatism. All rights reserved. Tumor necrosis factor alpha (TNF-a) antagonistinduced lupus-like syndrome (TAILs)1 is characterized by rashes, arthralgias, fever, and/or serositis along with positive lupus serologies temporally associated with initiation of TNF-a antagonist medications.2 The incidence of TAILs may be increasing as TNF-a antagonists are more widely utilized. The management of rheumatoid arthritis (RA) patients who develop TAILs and the safety of re-challenging these patients with other biologic therapies remain largely unknown and understudied.

Objective.The rarity of large vessel vasculitis (LVV) is a major factor limiting randomized controlled trials in LVV, resulting in treatment choices in these diseases that are guided mainly by observational studies and expert opinion. Further complicating trials in LVV is the absence of validated and meaningful outcome measures. The Outcome Measures in Rheumatology (OMERACT) vasculitis working group initiated the Large Vessel Vasculitis task force in 2009 to develop data-driven, validated outcome tools for clinical investigation in LVV. This report summarizes the progress that has been made on a disease activity assessment tool and patient-reported outcomes in LVV as well as the group’s research agenda.Methods.The OMERACT LVV task force brought an international group of investigators and patient research partners together to work collaboratively on developing outcome tools. The group initially focused on disease activity assessment tools in LVV. Following a systematic literature review, an international Delphi exercise was conducted to obtain expert opinion on principles and domains for disease assessment. The OMERACT vasculitis working group’s LVV task force is also conducting qualitative research with patients, including interviews, focus groups, and engaging patients as research partners, all to ensure that the approach to disease assessment includes measures of patients’ perspectives and that patients have input into the research agenda and process.Results.The preliminary results of both the Delphi exercise and the qualitative interviews were discussed at the OMERACT 12 (2014) meeting and the completion of the analyses will produce an initial set of domains and instruments to form the basis of next steps in the research agenda.Conclusion.The research agenda continues to evolve, with the ultimate goal of developing an OMERACT-endorsed core set of outcome measures for use in clinical trials of LVV.