BACKGROUND:After introducing IL-1/IL-6 inhibitors, some patients with Still and Still-like disease developed unusual, often fatal, pulmonary disease. This complication was associated with scoring as DReSS (drug reaction with eosinophilia and systemic symptoms) implicating these inhibitors, although DReSS can be difficult to recognize in the setting of systemic inflammatory disease. OBJECTIVE:To facilitate recognition of IL-1/IL-6 inhibitor-DReSS in systemic inflammatory illnesses (Still/Still-like) by looking at timing and reaction-associated features. We evaluated outcomes of stopping or not stopping IL-1/IL-6 inhibitors after DReSS reaction began. METHODS:In an international study collaborating primarily with pediatric specialists, we characterized features of 89 drug-reaction cases versus 773 drug-exposed controls and compared outcomes of 52 cases stopping IL-1/IL-6 inhibitors with 37 cases not stopping these drugs. RESULTS:Before the reaction began, drug-reaction cases and controls were clinically comparable, except for younger disease-onset age for reaction cases with preexisting cardiothoracic comorbidities. After the reaction began, increased rates of pulmonary complications and macrophage activation syndrome differentiated drug-reaction cases from drug-tolerant controls (P = 4.7 × 10-35 and P = 1.1 × 10-24, respectively). The initial DReSS feature was typically reported 2 to 8 weeks after initiating IL-1/IL-6 inhibition. In drug-reaction cases stopping versus not stopping IL-1/IL-6-inhibitor treatment, reaction-related features were indistinguishable, including pulmonary complication rates (75% [39 of 52] vs 76% [28 of 37]). Those stopping subsequently required fewer medications for treatment of systemic inflammation, had decreased rates of macrophage activation syndrome, and improved survival (P = .005, multivariate regression). Resolution of pulmonary complications occurred in 67% (26 of 39) of drug-reaction cases who stopped and in none who continued inhibitors. CONCLUSIONS:In systemic inflammatory illnesses, recognition of IL-1/IL-6-inhibitor-associated reactions followed by avoidance of IL-1/IL-6 inhibitors significantly improved outcomes.

IntroductionThe Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) is a North American learning health network focused on improving outcomes of children with juvenile idiopathic arthritis (JIA). JIA is a chronic autoimmune disease that can lead to morbidity related to persistent joint and ocular inflammation. PR-COIN has a shared patient registry that tracks twenty quality measures including ten outcome measures of which six are related to disease activity. The network's global aim, set in 2021, was to increase the percent of patients with oligoarticular or polyarticular JIA that had an inactive or low disease activity state from 76% to 80% by the end of 2023.MethodsTwenty-three hospitals participate in PR-COIN, with over 7,200 active patients with JIA. The disease activity outcome measures include active joint count, physician global assessment of disease activity, and measures related to validated composite disease activity scoring systems including inactive or low disease activity by the 10-joint clinical Juvenile Arthritis Disease Activity Score (cJADAS10), inactive or low disease activity by cJADAS10 at 6 months post-diagnosis, mean cJADAS10 score, and the American College of Rheumatology (ACR) provisional criteria for clinical inactive disease. Data is collated to measure network performance, which is displayed on run and control charts. Network-wide interventions have included pre-visit planning, shared decision making, self-management support, population health management, and utilizing a Treat to Target approach to care.ResultsFive outcome measures related to disease activity have demonstrated significant improvement over time. The percent of patients with inactive or low disease activity by cJADAS10 surpassed our goal with current network performance at 81%. Clinical inactive disease by ACR provisional criteria improved from 46% to 60%. The mean cJADAS10 score decreased from 4.3 to 2.6, and the mean active joint count declined from 1.5 to 0.7. Mean physician global assessment of disease activity significantly improved from 1 to 0.6.ConclusionsPR-COIN has shown significant improvement in disease activity metrics for patients with JIA. The network will continue to work on both site-specific and collaborative efforts to improve outcomes for children with JIA with attention to health equity, severity adjustment, and data quality.

ObjectiveTreat to target (T2T) is a strategy of adjusting treatment until a target is reached. An international task force recommended T2T for juvenile idiopathic arthritis (JIA) treatment. Implementing T2T in a standard and reliable way in clinical practice requires agreement on critical elements of (1) target setting, (2) T2T strategy, (3) identifying barriers to implementation, and (4) patient eligibility. A consensus conference was held among Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) stakeholders to inform a statement of understanding regarding the PR-COIN approach to T2T.MethodsPR-COIN stakeholders including 16 healthcare providers and 4 parents were invited to form a voting panel. Using the nominal group technique, 2 rounds of voting were held to address the above 4 areas to select the top 10 responses by rank order.ResultsIncorporation of patient goals ranked most important when setting a treatment target. Shared decision making (SDM), tracking measurable outcomes, and adjusting treatment to achieve goals were voted as the top elements of a T2T strategy. Workflow considerations, and provider buy-in were identified as key barriers to T2T implementation. Patients with JIA who had poor prognostic factors and were at risk for high disease burden were leading candidates for a T2T approach.ConclusionThis consensus conference identified the importance of incorporating patient goals as part of target setting and of the influence of patient stakeholder involvement in drafting treatment recommendations. The network approach to T2T will be modified to address the above findings, including solicitation of patient goals, optimizing SDM, and better workflow integration.

PURPOSE OF REVIEW:This article highlights efforts in pediatric rheumatology related to optimizing the care provided to patients with pediatric rheumatic diseases and describes various approaches to improve health outcomes.RECENT FINDINGS:Recent studies report low rates of remission, frequent occurrence of comorbidities, disease damage, and decreased health-related quality of life in pediatric rheumatic diseases. The Pediatric Rheumatology Care and Outcomes Improvement Network is a quality improvement learning network that has demonstrated improvement in the process of care measures through use of a centralized patient registry, and interventions, including previsit planning, population management, shared decision making, and patient/parent engagement. A pediatric rheumatology patient-powered research network was established to enable patient and caregiver participation in setting research priorities and to facilitate data sharing to answer research questions. Quality measure development and benchmarking are proceeding in multiple pediatric rheumatic diseases.SUMMARY:The review summarizes the current efforts to improve care delivery and outcomes in pediatric rheumatic diseases through a learning health system approach that harnesses knowledge from the clinical encounter to serve quality improvement, research, and discovery. Incorporating standard approaches to medication treatment plans may reduce variation in care, including using the patient voice to design research studies to bring focus on more patient relevant outcomes.VIDEO ABSTRACT:http://links.lww.com/COR/A28.

Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) is a multi-site learning network designed to improve outcomes of juvenile idiopathic arthritis (JIA) care. Teams collect point of care data on measures of process of care and outcomes of care for the purposes of analysis to guide improvement activities. Eleven North American pediatric rheumatology centers participate. This report illustrates our improvement in several JIA process quality measures (QMs). Process of care QMs targeted for improvement include measurement of: arthritis-related pain, physician global assessment, joint count, health-related quality of life, physical function, as well as screening for uveitis, medication toxicity, and tuberculosis per guidelines. Outcome measures for JIA include clinical inactive disease, clinical remission on and off medications, no or mild pain level, and optimal physical functioning. Network goals were determined for each process and outcome measure. Data are collected with IRB approval and informed consent, and the shared registry for data entry is the ACR's Rheumatology Clinical Registry. Site-specific and aggregate data are analyzed and displayed monthly via statistical process control charts allowing PR-COIN to track performance over time. Individual centers use established quality improvement methodology to reach and exceed pre-determined goals. Data from 5112 encounters for 1134 JIA patients have been collected since April 2011. QMs with performance meeting or exceeding initial goals include documentation of complete joint count and measurement of arthritis-related pain. For PR-COIN network as a collective unit, QMs improved in six processes—measurement of functional ability, completion of ongoing medication toxicity labs, documentation of complete joint count, medication counseling for newly prescribed DMARDs, documentation of annual medication counseling, and measurement of health-related quality of life. All of these measures had a shift above the baseline mean, demonstrating special cause. In addition, five sites have demonstrated individual improvement in at least one process QM. PR-COIN sites are collectively and individually demonstrating significant improvements in JIA process of care QMs. Quality improvement efforts in PR-COIN are ongoing with the goal of improving the outcome for patients with JIA.

Background/Purpose:The American College of Rheumatology (ACR) provisional criteria for Clinical Inactive Disease (CID) are an accepted outcome measure for juvenile idiopathic arthritis (JIA) patients (). The purpose of this study is to determine what components of CID are not met by Pediatric Rheumatology Care and Outcomes Improvement Network (PR–COIN) patients. The goal is to better understand the ways in which patients are not doing well. We hypothesize that many patients who do not meet CID have mild disease activity such as joint count <3, physician global assessment of disease activity score of <3, or short duration of morning stiffness, but otherwise would fulfill CID.Methods:PR‐COIN is a multi‐center quality improvement learning network comprised of 11 sites in North America whose mission is to improve outcomes for children with JIA. With IRB consent, patient data are entered into the ACR's Rheumatology Clinical Registry. The criteria for CID include the following: no joints with active arthritis, no systemic features, no active uveitis, normal ESR or CRP (if measured), physician global assessment of disease activity of 0, and duration of morning stiffness < 15 min (). Data were analyzed to determine which components of CID patients do not achieve. Only patients with complete data such that CID status could be calculated were included.Results:Data from 658 individual patients were analyzed. Sixty‐one percent of patients did not achieve clinical inactive disease at their most recent visit (404/ 658 patients). The top 3 components of clinical inactive disease that patients did not meet, from most frequent to least frequent, were physician global assessment (91%), joint count (66%), and duration of morning stiffness (28%). Of the patients with active disease, sixteen percent had elevated ESR or CRP (inflammatory markers were not measured in all patients), 11% had active uveitis, and <1% had active systemic JIA features. Sixty‐two percent of patients with active disease had a joint count <3, physician global assessment <3, and duration of morning stiffness < 30 minutes, perhaps indicating this was a subset of patients with mild disease activity. This represents 38% of the total population. Thus, 77% of PR‐COIN patients had clinical inactive disease or mild disease. Seven percent had active uveitis.Conclusion:Although only 39% of patients achieve CID at their most recent visit in the PR‐COIN cohort, and this falls short of our desired outcome, many of these patients with active disease appear to have only mild disease activity. Therefore, the ACR criteria for CID may be somewhat stringent and difficult to achieve in the clinical setting. CID being a binary “all or none” outcome measure makes it difficult to detect whether quality improvement activities are having any beneficial incremental impact on patient disease status. PR‐COIN will continue to seek ways to improve rates of CID.

Background Children with juvenile idiopathic arthritis (JIA) do not receive optimal care. There is wide variation in selection of treatment, adherence with published guidelines, and the use of evidence-based care delivery processes. Improving the quality of care for JIA by decreasing these existing wide variations is essential to improve long term outcomes and to increase the speed with which new discoveries and knowledge reach patients. PR-COIN is a “learning network” that uses data for quality improvement (QI) and research. Learning networks involve collaborations among multidisciplinary teams of clinicians and staff, patients and families, and researchers to improve delivery of care and patient outcomes. Objectives The mission of PR-COIN is to build a sustainable learning network to improve the outcomes of care for children with JIA and to accelerate adoption of evidence into medical practice. Methods PR-COIN is organized based on the Breakthrough Series collaborative improvement model developed by the Institute for Healthcare Improvement. Sites participating in PR-COIN establish a clinical improvement team consisting of 3 or more people including a physician champion and nurse. Teams attend face-to-face learning sessions, and receive intensive training and coaching on how to apply proven strategies for chronic disease management to JIA, including self-management support, delivery system design, decision support, and clinical information systems from nationally recognized quality improvement and JIA experts. During action periods between learning sessions, teams use QI tools to help with implementation of and tests of system changes. Teams collect and submit data to a shared registry regarding their performance on several published quality measures (QM). Teams receive feedback and learn from each other through monthly conference calls and reports of site-specific and aggregate data on QM presented as run charts enabling teams to track performance on QM over time. Results 12 North American sites are enrolled in the network and show considerable variation along several elements, including number of physicians, staffing, medical records systems, information technology capability and geography. A public website pr-coin.org has a members only section for transparent sharing of data reports. A population management tool allows segmentation of patient populations for targeted interventions. Baseline performance on selected QM include 69% of patients receiving uveitis screening per guidelines, 64% of patients on biologics screened for TB annually, and 100% of patient visits document complete joint counts and pain levels. Current work is focused on areas for improvement. Conclusions By sharing data and aligning practices, PR-COIN focuses on reducing unwanted variations in care, enhancing learning about how to effectively scale up improvement, and providing infrastructure for patient-centered outcomes research, especially with respect to understanding the comparative effectiveness of healthcare interventions. Disclosure of Interest T. Beukelman Grant/Research support from: Pfizer, Consultant for: Novartis, C. Bingham: None Declared, B. Gottlieb: None Declared, N. Griffin: None Declared, R. Laxer: None Declared, K. Marsolo: None Declared, M. Passo Grant/Research support from: Pfizer, Consultant for: Pfizer, C. Lannon: None Declared, P. Margolis: None Declared, E. DeWitt: None Declared