Implementation of Rheumatology Health Care Transition Processes and Adaptations to Systems Under Stress: A Mixed-Methods Study.

OBJECTIVES: Despite poor health care transition outcomes among young adults with pediatric rheumatic diseases, adoption of transition best practices is low. We sought to understand how structured transition processes were operationalized within pediatric rheumatology practices and what factors were perceived to enable adaptations during a global pandemic. METHODS: We conducted a mixed methods study of team leaders' experiences during an interim analysis of a pilot project to implement transition policy discussions at sites in the Childhood Arthritis and Rheumatology Research Alliance Transition Learning Collaborative. We combined quantitative assessments of organizational readiness for change (9 sites) and semistructured interviews of team leaders (8 sites) using determinants in the Exploration, Preparation, Implementation, Sustainment Framework. RESULTS: Engagement of nursing and institutional improvement efforts facilitated decisions to implement transition policies. Workflows incorporating educational processes by nonphysicians were perceived to be critical for success. When the pandemic disrupted contact with nonphysicians, capacity for automation using electronic medical record (EMR)-based tools was an important facilitator, but few sites could access these tools. Sites without EMR-based tools did not progress despite reporting high organizational readiness to implement change at the clinic level. Lastly, educational processes were often superseded by acute issues, such that youth with greater medical/psychosocial complexity may not receive the intervention. CONCLUSION: We generated several considerations to guide implementation of transition processes within pediatric rheumatology from the perspectives of team leaders. Careful assessment of institutional and nursing support is advisable before conducting complex transition interventions. Ideally, new strategies would ensure interventions reach youth with high complexity.

Patient-Reported Outcomes Among Transition-Age Young Adults With Juvenile Idiopathic Arthritis in the Childhood Arthritis and Rheumatology Research Alliance Registry.

OBJECTIVE: To evaluate patient-reported care utilization and outcomes among young adults with juvenile idiopathic arthritis (JIA), including factors associated with complete transfer to adult rheumatology. METHODS: We included young adults with JIA enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry from 2015 to 2019 with age ≥ 18 years at their last clinical site visit. We used data from the CARRA Registry Long-term Follow-up program, which follows inactive CARRA Registry patients and collects patient-reported information through phone surveys. We compared the characteristics of respondents with complete and incomplete transfer to adult rheumatology care at their first Long-term Follow-up phone survey. RESULTS: We identified 540 young adults with JIA; 187 (35%) responded to the Long-term Follow-up phone survey. The 54% of respondents with complete transfer to adult rheumatology were slightly older and reported more self-assessed disease activity, morning stiffness, and pain compared to those with incomplete transfer. Biologic use was high at both timepoints and did not differ by transfer status. Patients who completed the transfer were more likely to have private insurance and be actively pursuing postsecondary education compared to those with an incomplete transfer. Across the cohort, 65% reported problems with pain or discomfort and 45% with anxiety or depression. CONCLUSION: Young adult respondents with JIA in the CARRA Registry commonly report persistent medication use, but still report more problems with pain as compared to population norms. Additional work is needed to understand how best to address comorbid pain around the period of transition to adult care.

Translating research into practice-implementation recommendations for pediatric rheumatology; Proceedings of the childhood arthritis and rheumatology research alliance 2020 implementation science retreat.

The translation of research findings into clinical practice is challenging, especially fields like in pediatric rheumatology, where the evidence base is limited, there are few clinical trials, and the conditions are rare and heterogeneous. Implementation science methodologies have been shown to reduce the research- to- practice gap in other clinical settings may have similar utility in pediatric rheumatology. This paper describes the key discussion points from the inaugural Childhood Arthritis and Rheumatology Research Alliance Implementation Science retreat held in February 2020. The aim of this report is to synthesize those findings into an Implementation Science Roadmap for pediatric rheumatology research. This roadmap is based on three foundational principles: fostering curiosity and ensuring discovery, integration of research and quality improvement, and patient-centeredness. We include six key steps anchored in the principles of implementation science. Applying this roadmap will enable researchers to evaluate the full range of research activities, from the initial clinical design and evidence acquisition to the application of those findings in pediatric rheumatology clinics and direct patient care.

Transitioning to Adulthood with a Rheumatic Disease: A Case-Based Approach for Rheumatology Care Teams.

Both pediatric and adult rheumatology care teams play a central role in health care transition, the shift from child- and family-centered to adult-oriented health care. Components of transition preparation include readiness assessment, setting self-management goals, and spending time in clinical visits without a parent present. Pediatric providers and families should work together to create a transfer plan, identifying a new adult rheumatology care provider, providing a medical summary before transfer, and anticipating changes in health insurance. For high-risk transfers, direct communication between providers is recommended. Finally, adult rheumatologists need to build rapport with young adults to support future engagement in care.

Patterns of Healthcare Use and Medication Adherence among Youth with Systemic Lupus Erythematosus during Transfer from Pediatric to Adult Care.

OBJECTIVE: Youth with systemic lupus erythematosus (SLE) transferring from pediatric to adult care are at risk for poor outcomes. We describe patterns of rheumatology/nephrology care and changes in healthcare use and medication adherence during transfer. METHODS: We identified youth ages 15-25 with SLE using US private insurance claims from Optum's deidentified Clinformatics Data Mart. Rheumatology/nephrology visit patterns were categorized as (1) unilateral transfers to adult care within 12 months, (2) overlapping pediatric and adult visits, (3) lost to followup, or (4) continuing pediatric care. We used negative binomial regression and paired t tests to estimate changes in healthcare use and medication possession ratios (MPR) after the last pediatric (index) visit. We compared MPR between youth who transferred and age-matched peers continuing pediatric care. RESULTS: Of the 184 youth transferred out of pediatric care, 41.8% transferred unilaterally, 31.5% had overlapping visits over a median of 12 months before final transfer, and 26.6% were lost to followup. We matched 107 youth continuing pediatric care. Overall, ambulatory care use decreased among those lost to followup. Acute care use decreased across all groups. MPR after the index date were lower in youth lost to followup (mean 0.24) compared to peers in pediatric care (mean 0.57, p < 0.001). CONCLUSION: Youth with SLE with continuous private insurance coverage do not use more acute care after transfer to adult care. However, a substantial proportion fail to see adult subspecialists within 12 months and have worse medication adherence, placing them at higher risk for adverse outcomes.

Disease activity and health-care utilization among young adults with childhood-onset lupus transitioning to adult care: data from the Pediatric Lupus Outcomes Study.

BACKGROUND: Individuals with childhood-onset systemic lupus erythematosus (cSLE) must transfer from pediatric to adult care. The goal of this study was to examine disease activity and health-care utilization among young adults with cSLE who are undergoing or have recently completed the transfer to adult care. METHODS: The Pediatric Lupus Outcomes Study (PLOS) is a prospective longitudinal cohort study of young adults aged 18-30 diagnosed with cSLE. We conducted a cross-sectional analysis comparing 47 participants under the care of pediatric rheumatologists to 38 who had completed transfer to adult care. Demographics, disease manifestations, health- care utilization and transition readiness were compared between groups. RESULTS: Those in the post-transfer group had significantly lower medication usage and were less likely to have seen a rheumatologist in the past year. Disease manifestations, flare rates, and hospitalizations were similar between groups. Nearly a quarter of patients who had transferred to adult care reported difficulties with the process. CONCLUSION: Post-transfer patients had lower health-care utilization as evidenced by less medication usage and lack of rheumatology follow-up, in spite of the fact that disease activity was similar in both groups. Future studies will assess longitudinal changes in disease activity and damage in this population.

US Adult Rheumatologists' Perspectives on the Transition Process for Young Adults With Rheumatic Conditions.

OBJECTIVE: To assess the attitudes and common practices of adult rheumatologists in the US regarding health care transition (HCT) for young adults with rheumatic diseases. METHODS: An anonymous online survey was sent to US adult rheumatologist members of the American College of Rheumatology to collect demographic data and information on attitudes and common practices regarding the transition process. RESULTS: Of 4,064 contacted rheumatologists, 203 (5%) completed the survey. Almost half of respondents (45.1%) were never trained in transition practices, and 74.7% were not familiar with the American Academy of Pediatrics/American Academy of Family Physicians/American College of Physicians Consensus Statement About Transitions for Youth with Special Healthcare Needs. Only 56.2% felt comfortable caring for former pediatric patients. The vast majority of respondents (90.7%) did not have a multidisciplinary transition team, and 37% did not have a plan for transitioning pediatric patients into their practice. Most adult rheumatologists were unsatisfied with the current transition process (92.9%), due to insufficient resources, personnel (91.1%), and time in clinic (86.9%). They also were unsatisfied with referral data received concerning previous treatments (48.9%), hospitalization history (48%), disease activity index (45.1%), medical history summary (43.9%), comorbidities (36.4%), medication list (34.1%), and disease classification (32.6%). Three major barriers to HCT were lack of insurance reimbursement (33.7%), knowledge about community resources (30.8%), and lapses in care between primary provider and specialist (27.8%). CONCLUSION: This survey identified substantial gaps in knowledge and resources regarding HCT for young adults with rheumatic diseases. These may be best addressed by further training, research, dedicated resources, adequate payment, and practice guidelines.

Longitudinal disease- and steroid-related damage among adults with childhood-onset systemic lupus erythematosus.

OBJECTIVES: Determine whether adults with childhood-onset systemic lupus erythematosus (cSLE) are at increased risk for disease- and steroid-related damage as compared to individuals with adult-onset SLE (aSLE), and whether they continue to accumulate disease damage in adulthood. METHODS: Data derive from the 2007-2015 cycles of the Lupus Outcomes Study, a longitudinal cohort of adults with confirmed SLE. The Brief Index of Lupus Damage (BILD), a validated, patient-reported measure, was used to assess SLE-associated damage. Participants with baseline BILD were included (N = 1035). Diagnosis at age < 18 years was defined as cSLE (N = 113). Outcome variables included BILD score at baseline and follow-up, clinically significant change in BILD score over follow-up period, and presence of steroid-related damage (cataracts, osteoporosis-related fracture, avascular necrosis or diabetes mellitus). RESULTS: Mean time between baseline and follow up BILD assessment was 6.3 ±â€¯1.7 years. In adjusted analyses, participants with cSLE and aSLE had similar levels of disease-related damage, and accumulated damage at similar rates. Participants with cSLE were more likely to report steroid-related damage (OR 1.7, 95% CI 1.1-2.8) in the adjusted analysis as compared to those with aSLE. Likelihood of steroid-related damage increased with disease duration for both groups, but was consistently higher among cSLE participants. CONCLUSION: In this longitudinal cohort of adults with SLE, participants continued to accumulate damage at similar rates over time, regardless of age at onset or disease duration. Childhood-onset predicted increased risk of steroid-related damage. Aggressive use of steroid-sparing treatment strategies during childhood may be important to prevent steroid-related damage in adulthood.

Final adult height of patients with childhood-onset systemic lupus erythematosus: a cross sectional analysis.

BACKGROUND: To compare final height to mid-parental target height among adults with childhood-onset systemic lupus erythematosus (cSLE) versus adult-onset SLE (aSLE), and to evaluate the impact of age at SLE onset on final height. METHODS: Data derived from the Lupus Outcomes Study, a longitudinal cohort of adults with SLE, was used for this cross-sectional analysis (N = 728). Participants aged 18-63 years with complete height data were included (N = 566) and were classified as cSLE if age at diagnosis was < 18 years (N = 72). The Tanner formula was used to calculate mid-parental target height. Multivariate linear regression was used to determine mean difference between final height and target height. Multivariate logistic regression was used to compare odds of substantially reduced final height, defined as > 2 SD below target height. Separate analyses were conducted for females and males to account for differences in timing of the pubertal growth spurt for each sex. RESULTS: Participants with cSLE were, on average, 2.4 cm shorter than their target height (95% CI -4, - 0.7). The adjusted odds ratio (OR) for substantially reduced final height was 3.9 (95% CI + 2.0, + 7.2, p < 0.001) as compared to participants with aSLE. Females diagnosed between 11 and 13 years were at greatest risk for substantially reduced final height, with adjusted OR of 11.2 (95% CI + 3.4, + 36.3) as compared to participants with aSLE (p < 0.001). CONCLUSIONS: cSLE is associated with shorter-than-expected final height. Onset of SLE in the pubertal period, near the time of maximum linear growth, may have a particularly significant impact on final height.

Depression Risk in Young Adults With Juvenile- and Adult-Onset Lupus: Twelve Years of Followup.

OBJECTIVE: To compare major depression risk among young adults with juvenile-onset and adult-onset systemic lupus erythematosus (SLE), and to determine demographic and health-related predictors of depression. METHODS: Young adults with SLE ages 18-45 years (n = 546) in the Lupus Outcomes Study completed annual telephone surveys from 2002-2015, including assessment of depression using the Center for Epidemiologic Studies Depression Scale (CES-D), and self-report measures of sociodemographics and health characteristics. Juvenile-onset SLE was defined as age <18 years at diagnosis (n = 115). Repeated-measures analysis was performed to assess the risk for major depression (CES-D ≥24) at any point in study, and logistic regression was used to assess for recurrent (present on ≥2 assessments) major depression. RESULTS: Major depression was experienced by 47% of the cohort at least once during the 12-year study period. In adjusted analyses, juvenile-onset SLE patients had an increased risk of having a major depressive episode (odds ratio [OR] 1.7 [95% confidence interval (95% CI) 1.0-2.7]) and recurrent episodes (OR 2.2 [95% CI 1.2-4.3]), compared to participants with adult-onset SLE. Older age, lower educational attainment, and physical function, higher disease activity, and a history of smoking were associated with an increased depression risk. Juvenile-onset SLE patients had a higher risk of major depression across all educational groups. CONCLUSION: Young adults with SLE, particularly those with juvenile-onset disease, are at high risk for major depression, which is associated with increased disease activity, poorer physical functioning, and lower educational attainment. Early depression intervention in young adults with SLE has the potential to improve both medical and psychosocial outcomes.

Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

OBJECTIVE: To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and the 12-month outcomes in children with AAV. METHODS: Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of <0.2 mg/kg/day. Secondary outcome measures included the rates of inactive disease (PVAS of 0, with any corticosteroid dosage) and rates of improvement at postinduction (4-6 months after diagnosis) and at 12 months, presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months. RESULTS: In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9-15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4-6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0-6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months. CONCLUSION: This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one-half of this patient cohort experienced damage to various organ systems early in their disease course.

Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study.

OBJECTIVE: To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e-entry) cohort, with the data censored to November 2015. The EMA algorithm was used to uniquely distinguish children with MPA from children with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons. RESULTS: In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n = 48) or GPA (n = 183). The median time to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA compared to those with GPA, pulmonary manifestations were less frequent (44% versus 74%) and less severe (primarily, hemorrhage, requirement for supplemental oxygen, and pulmonary failure). Renal pathologic features were frequently found in both groups (75% of patients with MPA versus 83% of patients with GPA) but tended toward greater severity in those with MPA (primarily, nephrotic-range proteinuria, requirement for dialysis, and end-stage renal disease). Airway/eye involvement was absent among patients with MPA, because these GPA-defining features preclude a diagnosis of MPA within the EMA algorithm. Similar proportions of patients with MPA and those with GPA received combination therapy with corticosteroids plus cyclophosphamide (69% and 78%, respectively) or both drugs in combination with plasmapheresis (19% and 22%, respectively). Other treatments administered, ranging in decreasing frequency from 13% to 3%, were rituximab, methotrexate, azathioprine, and mycophenolate mofetil. CONCLUSION: Younger age at disease onset and, perhaps, both gastrointestinal manifestations and more severe kidney disease seem to characterize the clinical profile in children with MPA compared to those with GPA. Delay in diagnosis suggests that recognition of these systemic vasculitides is suboptimal. Compared with adults, initial treatment regimens in children were comparable, but the complete reversal of female-to-male disease prevalence ratios is a provocative finding.

Reasons for failure to receive pneumococcal and influenza vaccinations among immunosuppressed patients with systemic lupus erythematosus.

OBJECTIVE: To better understand why immunosuppressed individuals with systemic lupus erythematosus (SLE) fail to receive influenza and pneumococcal vaccines. METHODS: These cross-sectional data were derived from the 2009 cycle of the Lupus Outcomes Study (LOS), an annual longitudinal telephone survey of individuals with confirmed SLE. Respondents were included in the analysis if they had taken immunosuppressive medications in the past year. We assessed any prior receipt of pneumococcal vaccine and influenza vaccine in the past year, and then elicited reasons for not receiving vaccination. We used bivariate statistics and multivariate logistic regression to assess frequency and predictors of reported reasons for not obtaining influenza or pneumococcal vaccines. RESULTS: Among 508 respondents who received immunosuppressants, 485 reported whether they had received vaccines. Among the 175 respondents who did not receive an influenza vaccine, the most common reason was lack of doctor recommendation (55%), followed by efficacy or safety concerns (21%), and lack of time (19%). Reasons for not receiving pneumococcal vaccine (N = 159) were similar: lack of recommendation (87%), lack of time (7%), and efficacy or safety concerns (4%). Younger, less-educated, non-white patients with shorter disease duration, as well as those immunosuppressed with steroids alone, were at the greatest risk for not receiving indicated vaccine recommendations. CONCLUSIONS: The most common reason why individuals with SLE did not receive pneumococcal and influenza vaccines was that physicians failed to recommend them. Data suggest that increasing vaccination rates in SLE will require improved process quality at the provider level, as well as addressing patient concerns and barriers.

Educational and vocational outcomes of adults with childhood- and adult-onset systemic lupus erythematosus: nine years of followup.

OBJECTIVE: To compare educational and vocational outcomes among adults with childhood-onset systemic lupus erythematosus (SLE) and adult-onset SLE. METHODS: We used data derived from the 2002­2010 cycles of the Lupus Outcomes Study, a longitudinal cohort of 1,204 adult subjects with SLE. Subjects ages 18­60 years living in the US (n = 929) were included in the analysis and were classified as childhood-onset SLE if age at diagnosis was <18 years (n = 115). Logistic regression was used to assess the unadjusted and adjusted effect of childhood-onset SLE, sex, race/ethnicity, baseline age, urban or rural location, and US region on the likelihood of completing a bachelor's degree. Generalized estimating equations were used to assess the effect of childhood-onset SLE, demographics, education, and disease-related factors on the odds of employment, accounting for multiple observations over the study period. RESULTS: Subjects with childhood-onset SLE were on average younger (mean ± SD 29 ± 10 years versus 44 ± 9 years), with longer disease duration (mean ± SD 15 ± 10 years versus 11 ± 8 years). Subjects with adult-onset SLE and childhood-onset SLE subjects were equally likely to complete a bachelor's degree. However, subjects with childhood-onset SLE were significantly less likely to be employed, independent of demographic and disease characteristics (odds ratio 0.62, 95% confidence interval 0.42­0.91). CONCLUSION: While subjects with SLE are just as likely as those with adult-onset SLE to complete college education, childhood-onset SLE significantly increases the risk of not working in adulthood, even when controlling for disease and demographic factors. Exploring reasons for low rates of employment and providing vocational support may be important to maximize long-term functional outcomes in patients with childhood-onset SLE.

Atypical cases of scleroderma en coup de sabre.

Scleroderma en coup de sabre typically presents with a cutaneous induration, but involvement of the underlying bone, eye, and brain is common. We report on 4 pediatric cases with atypical initial clinical presentations. All cases were seen at the University of California San Francisco. Patients 1 and 2 presented to the Pediatric Rheumatology Clinic with uveitis and orbital pseudotumor in December 2009 and March 2010, respectively. Patients 3 and 4 were mimicking acquired demyelinating disorders of the brain, acute demyelinating encephalomyelitis and multiple sclerosis, and were referred to the Pediatric Multiple Sclerosis center in 2008.

Perception of transition readiness and preferences for use of technology in transition programs: teens' ideas for the future.

BACKGROUND: Efforts to facilitate transition of care to adult providers for adolescents with chronic disease are not uniformly successful and many patients encounter challenges. The goal of this study was to assess transition readiness and preferences for tools to aid in the transition process with an emphasis on technology and social media. METHODS: We surveyed and performed focus groups on patients aged 13-21 years from a pediatric university-based rheumatology and general pediatric practice. Demographics and transition readiness were assessed using a questionnaire. Transition readiness was assessed by examining patient knowledge and independence with care. Focus groups were conducted to elicit perspectives about desirable features of a transition program and useful tools. RESULTS: Thirty-five patients completed surveys; and 20 patients and 13 of their parents participated in a focus group. The median patient age was 17 years and 74.3% were female. A Likert scale (0-10, 10=most) was used to evaluate concern over changing to an adult medical provider, (mean=6.4, SD=2.6), preparedness for disease self-management (mean=6.0, SD=2.8), and perceived importance of self-managing their condition (mean=7.1, SD=3.1). Themes that emerged from focus groups included a desire for support groups with other teens, a preference for using text messaging for communication and a desire for an online health management program. CONCLUSIONS: Teens with chronic disease are able to identify health maintenance tasks and strategies that will aid in developing independence with healthcare management. These findings support the idea that developing engaging applications and support groups will assist teens in the transitioning.

Do adult disease severity subclassifications predict use of cyclophosphamide in children with ANCA-associated vasculitis? An analysis of ARChiVe study treatment decisions.

OBJECTIVE: To determine whether adult disease severity subclassification systems for antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are concordant with the decision to treat pediatric patients with cyclophosphamide (CYC). METHODS: We applied the European Vasculitis Study (EUVAS) and Wegener's Granulomatosis Etanercept Trial (WGET) disease severity subclassification systems to pediatric patients with AAV in A Registry for Childhood Vasculitis (ARChiVe). Modifications were made to the EUVAS and WGET systems to enable their application to this cohort of children. Treatment was categorized into 2 groups, "cyclophosphamide" and "no cyclophosphamide." Pearson's chi-square and Kendall's rank correlation coefficient statistical analyses were used to determine the relationship between disease severity subgroup and treatment at the time of diagnosis. RESULTS: In total, 125 children with AAV were studied. Severity subgroup was associated with treatment group in both the EUVAS (chi-square 45.14, p < 0.001, Kendall's tau-b 0.601, p < 0.001) and WGET (chi-square 59.33, p < 0.001, Kendall's tau-b 0.689, p < 0.001) systems; however, 7 children classified by both systems as having less severe disease received CYC, and 6 children classified as having severe disease by both systems did not receive CYC. CONCLUSION: In this pediatric AAV cohort, the EUVAS and WGET adult severity subclassification systems had strong correlation with physician choice of treatment. However, a proportion of patients received treatment that was not concordant with their assigned severity subclass.

Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis.

OBJECTIVE: Granulomatosis with polyangiitis (Wegener's; GPA) and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare in childhood and are sometimes difficult to discriminate. We compared use of adult-derived classification schemes for GPA against validated pediatric criteria in the ARChiVe (A Registry for Childhood Vasculitis e-entry) cohort, a Childhood Arthritis and Rheumatology Research Alliance initiative. METHODS: Time-of-diagnosis data for children with physician (MD) diagnosis of AAV and unclassified vasculitis (UCV) from 33 US/Canadian centers were analyzed. The European Medicines Agency (EMA) classification algorithm and European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) and American College of Rheumatology (ACR) criteria for GPA were applied to all patients. Sensitivity and specificity were calculated (MD-diagnosis as reference). RESULTS: MD-diagnoses for 155 children were 100 GPA, 25 microscopic polyangiitis (MPA), 6 ANCA-positive pauciimmune glomerulonephritis, 3 Churg-Strauss syndrome, and 21 UCV. Of these, 114 had GPA as defined by EMA, 98 by EULAR/PRINTO/PRES, and 87 by ACR. Fourteen patients were identified as GPA by EULAR/PRINTO/PRES but not by ACR; 3 were identified as GPA by ACR but not EULAR/PRINTO/PRES. Using the EMA algorithm, 135 (87%) children were classifiable. The sensitivity of the EMA algorithm, the EULAR/PRINTO/PRES, and ACR criteria for classifying GPA was 90%, 77%, and 69%, respectively, with specificities of 56%, 62%, and 67%. The relatively poor sensitivity of the 2 criteria related to their inability to discriminate patients with MPA. CONCLUSION: EULAR/PRINTO/PRES was more sensitive than ACR criteria in classifying pediatric GPA. Neither classification system has criteria for MPA; therefore usefulness in discriminating patients in ARChiVe was limited. Even when using the most sensitive EMA algorithm, many children remained unclassified.

Healthcare quality in systemic lupus erythematosus: using Donabedian's conceptual framework to understand what we know.

Healthcare quality improvement has the potential to reduce the striking disparities in health outcomes among patients with systemic lupus erythematosus (SLE). Donabedian's framework for assessment of healthcare quality, which divides factors impacting quality into structures, processes and outcomes, provides a theoretical framework for research and interventions in quality improvement. This review applies Donabedian's model to current research describing quality of care in SLE, highlighting structures and processes that may lead to improved outcomes. Work remains to be done to develop meaningful metrics to assess quality and to understand the structures and processes that improve outcomes. Quality indicators have emerged as an important tool to measure quality, but further validation is required to define their validity and feasibility in clinical practice, as well as their association with improved outcomes. Implementation science also shows promise as a means to create meaningful systematic improvements in healthcare quality for patients with SLE.