Consensus Treatment Plans Research Articles

Approach to Classification and Management of Childhood-Onset Lupus Nephritis

(1) To discuss the relevance of the 2018 International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification system for nephritis in childhood-onset systemic lupus erythematosus (SLE). (2) To highlight current practices not addressed in the 2013 Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plan (CTP) for pediatric lupus nephritis (LN). In addition to class of glomerular involvement, multiple features on kidney biopsy can be assessed for prognosis and therapeutic decision-making. There is pediatric evidence and expertise for optimization of LN classification. Yet, treatment is based primarily on whether nephritis is proliferative or membranous. Moreover, alternative strategies for induction therapy are more commonly prescribed for children. There is a need for pediatric data to evaluate/validate LN classification systems. Updates are needed to CTPs for LN induction therapy. Pediatric nephrologists should be enrolling LN patients into existing prospective registries to build the evidence base for treatment decisions.

Initial Results from a Pilot Comparative Effectiveness Study of 3 Methotrexate-based Consensus Treatment Plans for Juvenile Localized Scleroderma.

To perform a comparative effectiveness feasibility study in juvenile localized scleroderma (LS), using standardized treatment regimens (consensus treatment plans; CTP). A prospective, multicenter 1-year pilot observational cohort study was performed by Childhood Arthritis and Rheumatology Research Alliance (CARRA) LS workgroup members. Patients with active, moderate to severe juvenile LS were treated with one of 3 CTP: methotrexate alone, or in combination with intravenous (30 mg/kg/dose for 3 mos) or oral corticosteroids (2 mg/kg/day tapered by 48 weeks). Fifty patients, with demographics typical for juvenile LS, were enrolled, and 44 (88%) completed the study. Most had extracutaneous involvement. Patients improved in all 3 CTP, with > 75% having a major or moderate level of improvement compared to baseline. Damage accrued in some patients. Major deviations from prescribed regimen resulted from medication intolerance (n = 6; 14%) or treatment failure (n = 11; 25%); failures occurred in all 3 CTP. Significant responses to treatment were demonstrated by LS skin scoring measures and overall physician assessments, with differences in response level identified in some patient subsets. Response differences were associated with baseline disease activity level, LS subtype, skin disease extent, and extracutaneous involvement. This study demonstrates the feasibility of conducting juvenile LS comparative effectiveness studies. The CTP were found to be safe, effective, and tolerable. Our assessments performed well. Because damage is common and may progress despite effective control of activity, we recommend initial treatment efficacy be evaluated primarily by activity measures. Potential confounders for response were identified that warrant further study.

Treatment of juvenile idiopathic arthritis: what's new?

The present review highlights the advances in disease outcome achieved with currently available biologic medications and future perspectives for JIA management. In the last two decades, the management of juvenile idiopathic arthritis (JIA) has been revolutionized by appropriate legislative initiatives, the existence of very large collaborative networks and the increased availability of the novel biologic medications. A more rational approach to the management of JIA is being fostered by the recent publication of therapeutic recommendations, consensus treatment plans and for a treat-to-target strategy.

Physician practices for withdrawal of medications in inactive systemic juvenile arthritis, Childhood Arthritis and Rheumatology Research Alliance (CARRA) survey

BackgroundWe describe a Childhood Arthritis and Rheumatology Research Alliance (CARRA) survey of North American pediatric rheumatologists that assesses physician attitudes on withdrawal of medications in systemic juvenile idiopathic arthritis (SJIA).MethodsA REDCap anonymous electronic survey was distributed to 100 random CARRA JIA workgroup physician-voting members. The survey had three broad sections including: A) demographic information; B) physicians’ opinions on clinical inactive disease (CID) in SJIA and C) existing practices for withdrawing medications in SJIA.ResultsThe survey had an 86% response rate. 88 and 93% of participants agreed with the current criteria for CID and clinical remission on medications (CRM) respectively. 78% thought it necessary to meet CRM before tapering medications except steroids. 76% use CARRA SJIA consensus treatment plans always or the majority of the time. All participants weaned steroids first in SJIA patients on combination therapy, 47% waited > 6 months before tapering additional medications. 35% each tapered methotrexate over > 6 months and 2–6 months; however, 39% preferred tapering anakinra, canakinumab and tocilizumab more quickly over 2–6 months and favored spacing the dosing interval for canakinumab and tocilizumab. When patients are on combination therapy with methotrexate and biologics, 58% preferred tapering methotrexate first while others considered patient/family preference and adverse effects to guide their choice.ConclusionMost CARRA members surveyed use published consensus treatment plans for SJIA and agree with validated definitions of CID and CRM. There was agreement with tapering steroids first in SJIA. There was considerable variability with tapering decisions of all other medications.

Developing comparative effectiveness studies for a rare, understudied pediatric disease: lessons learned from the CARRA juvenile localized scleroderma consensus treatment plan pilot study

BackgroundWe designed and initiated a pilot comparative effectiveness study for juvenile localized scleroderma (jLS), for which there is limited evidence on best therapy. We evaluated the process we used, in relation to the specific protocol and to the general task of identifying strategies for implementing studies in rare pediatric diseases.MethodsThis was a prospective, multi-center, observational cohort study of 50 jLS patients initiating treatment, designed and conducted by the jLS group of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) from 2012 to 2015. A series of virtual and physical meetings were held to design the study, standardize clinical assessments, generate and refine disease activity and damage measures, and monitor the study. Patients were initiated on one of three standardized methotrexate-based treatment regimens (consensus treatment plans, CTPs) and monitored for 1 year. An optional bio-banking sub-study was included.ResultsThe target enrollment of 50 patients was achieved over 26 months at 10 sites, with patients enrolled into all CTPs. Enrolled patients were typical for jLS. Study eligibility criteria were found to perform well, capturing patients thought appropriate for treatment studies. Minor modifications to the eligibility criteria, primarily to facilitate recruitment for future studies, were discussed with consensus agreement reached on them by the jLS group. There were marked differences in site preferences for specific CTPs, with half the sites treating all their patients with the same CTP. Most patients (88%) completed the study, and 68% participated in the bio-banking substudy.ConclusionsWe demonstrate the feasibility of our approach for conducting comparative effectiveness research in a rare pediatric disease. Multi-center collaboration by dedicated investigators who met regularly was a key factor in the success of this project. Other factors that facilitate these studies include having a sufficient number of investigators to enroll in each regimen, and streamlining study approval and management.

Juvenile dermatomyositis-what's new?

Juvenile dermatomyositis (JDM) is the most common chronic inflammatory myopathy of childhood, which is still frequently characterized by acomplicated disease course. In this review, novel findings relating to JDM are presented based on areview of the literature. Myositis-specific antibodies are often detected and may correlate with clinical phenotypes and disease course. Activation of typeI interferon pathways plays an important pathogenic role and relates to the main clinical manifestations of the disease. This may lead to targeted therapies in the future. Currently, there are no treatments specifically approved for the treatment of JDM. Standard therapy is currently considered to include glucocorticoids and methotrexate based on arandomized controlled study and expert consensus. Several medications are commonly used in cases of refractory JDM, including azathioprine, ciclosporin, intravenous immune globulins, mycophenolate mofetil, and rituximab. An optimal treatment of JDM has not yet been established; however, there are national and international consensus recommendations and treatment plans that may aid in the decision-making process. Several validated tools are available to assess disease activity, disease damage, and treatment responses. Such tools should be routinely used in patients with JDM, and ideally be documented in registries in order to allow comparative effectiveness studies. The PRO-KIND initiative of the German Society for Pediatric Rheumatology has developed adiagnostic and atreat-to-target strategy based on apractice- and consensus-based process.

Pilot study comparing the childhood arthritis and rheumatology research alliance consensus treatment plans for induction therapy of juvenile proliferative lupus nephritis

BackgroundTo reduce treatment variability and facilitate comparative effectiveness studies, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) published consensus treatment plans (CTPs) including one for juvenile proliferative lupus nephritis (LN). Induction immunosuppression CTPs outline treatment with either monthly intravenous (IV) cyclophosphamide (CYC) or mycophenolate mofetil (MMF) in conjunction with one of three corticosteroid (steroid) CTPs: primarily oral, primarily IV or mixed oral/IV. The acceptability and in-practice use of these CTPs are unknown. Therefore, the primary aims of the pilot study were to demonstrate feasibility of adhering to the LN CTPs and delineate barriers to implementation in clinical care in the US. Further, we aimed to explore the safety and effectiveness of the treatments for induction therapy.MethodsForty-one patients were enrolled from 10 CARRA sites. Patients had new-onset biopsy proven ISN/RPS class III or IV proliferative LN, were starting induction therapy with MMF or IV CYC and high-dose steroids and were followed for up to 24 months. Routine clinical data were collected at each visit. Provider reasons for CTP selection were assessed at baseline. Adherence to the CTPs was evaluated by provider survey and medication logs. Complete and partial renal responses were reported at 6 months.ResultsThe majority of patients were female (83%) with a mean age of 14.7 years, SD 2.8. CYC was used more commonly than MMF for patients with ISN/RPS class IV LN (vs. class III), those who had hematuria, and those with adherence concerns. Overall adherence to the immunosuppression induction CTPs was acceptable with a majority of patients receiving the target MMF (86%) or CYC (63%) dose. However, adherence to the steroid CTPs was poor (37%) with large variability in dosing. Renal response endpoints were exploratory and did not show a significant difference between CYC and MMF.ConclusionsOverall, the immunosuppression CTPs were followed as intended in the majority of patients however, adherence to the steroid CTPs was poor indicating revision is necessary. In addition, our pilot study revealed several sources of treatment selection bias that will need to be addressed in for future comparative effectiveness research.

Glucocorticoid treatment in juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of joints in childhood. Glucocorticoids are being used in JIA treatment effectively for decades. Although systemic glucocorticoid use decreased with the introduction of biologic drugs, intraarticular glucocorticoid injections(IAGI) with nonsteroidal anti-inflammatory drugs and non-biologic disease modifying anti-rheumatic drugs (DMARDs) still remain the primary treatment in JIA, especially in oligoarticular subcategory. Systemic glucocorticoids are used mainly for severe JIA-associated complications such as macrophage activation syndrome (MAS), myocarditis, pericarditis, pleuritis, peritonitis, and severe anemia; as bridging therapy while waiting for the full therapeutic effect of DMARDs; and in certain occasions for patients with severe refractory uveitis. Since glucocorticoid administration is associated with many adverse events, it is important to use glucocorticoids in an optimum way balancing the risks and benefits. The aim of this review is to summarize the current knowledge on glucocorticoid treatment in JIA. A comprehensive literature search was conducted utilizing the Cochrane Library and MEDLINE/PubMed databases. The main topics include mechanism of action, dose, duration, adverse events, vaccination during glucocorticoid treatment, the place of glucocorticoids in JIA treatment guidelines and consensus treatment plans, glucocorticoid use in JIA-associated uveitis, MAS, and IAGI. Data from the literature provide guidance on how to use glucocorticoids in JIA treatment especially for IAGI and systemic use in systemic JIA and MAS. However, there is lack of evidence and need for prospective randomized studies in most parts including the indications in different JIA subcategories, optimum dose/route of administration/duration of treatment, and tapering strategies.

Multidisciplinary approach to the management of pulmonary embolism patients: the pulmonary embolism response team (PERT).

Pulmonary embolism (PE) is a potentially fatal disease with a broad range of treatment options that spans multiple specialties. The rapid evolution and expansion of novel therapies to treat PE make it a disease process that is well suited to a multidisciplinary approach. In order to facilitate a rapid, robust response to the diagnosis of PE, some hospitals have established multidisciplinary pulmonary embolism response teams (PERTs). The PERT model is based on existing multidisciplinary teams such as heart teams and rapid response teams. A PERT is composed of clinicians from the range of specialties involved in the treatment of PE, including pulmonology critical care, interventional radiology, cardiology, and cardiothoracic surgery among others. A PERT is a 24/7 consult service that is able to provide expert advice on the initial management of PE patients and convene in real time to develop a consensus treatment plan specifically tailored to the needs of a particular patient and consistent with the capabilities of the institution. In this review, we discuss the rationale for establishing a PERT and its potential benefits. We discuss considerations in forming a PERT and present case studies of several PERTs currently in operation at different institutions. We also discuss potential difficulties in forming a PERT and review evidence that has been generated by some of the PERTs that have been in operation the longest.

The Childhood Arthritis and Rheumatology Research Alliance Consensus Treatment Plans: Toward Comparative Effectiveness in the Pediatric Rheumatic Diseases.

The pediatric rheumatic diseases are a heterogeneous group of rare diseases, posing a number of challenges for the use of traditional clinical and translational research methods. Innovative comparative effectiveness approaches are needed to efficiently study treatment strategies and disease outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed the consensus treatment plan (CTP) approach as a comparative effectiveness tool for research in pediatric rheumatology. CTPs are treatment strategies, developed by consensus methods among CARRA members, intended to reduce variation in treatment approaches, standardize outcome measurements, and allow for comparison of the effectiveness of different approaches with the goal of improving disease outcomes. To date, CTPs have been published for 8 different diseases and disease manifestations. The approach has been successfully piloted for juvenile localized scleroderma, systemic juvenile idiopathic arthritis (JIA), polyarticular JIA, dermatomyositis, and lupus nephritis. Large-scale studies are underway for systemic JIA and polyarticular JIA, with the CARRA patient registry serving as the data collection platform. These studies have been designed with stakeholder involvement, including active input from CARRA providers, patients, and parents, with the goal of increasing feasibility and ensuring the relevance of the outcomes. These studies include ancillary biologic specimen collection intended to support additional translational and mechanistic studies. Data from these ongoing CTP studies will provide more information on the ability of this approach to identify effective treatment strategies and improve outcomes in the pediatric rheumatic diseases.

Recent therapeutic advances in juvenile idiopathic arthritis.

Over the past two decades, the management of juvenile idiopathic arthritis (JIA) has been revolutionized by the increased tendency toward early aggressive interventions and the availability of the novel biologic medications. In 2017, three novel randomized controlled trials have evaluated the effectiveness and tolerability of golimumab and tocilizumab in polyarticular JIA, and shown that methotrexate may increase and prolong the effect of intra-articular corticosteroid injection in children with oligoarthritis. A more rational approach to the management of JIA is being fostered by the recent publication of therapeutic recommendations, consensus treatment plans, and advice for the optimal care. A few months ago, an international consensus effort has led to the development of the recommendations for the treat-to-target in JIA. The application of this strategy in routine care may improve disease outcome. Because the potential of attaining inactive disease in children with JIA has markedly increased, there is an urgent need for randomized controlled trials, analyses of clinical data sets, and expert advice to guide discontinuation of medications once complete disease quiescence has been achieved.

Pilot study comparing the Childhood Arthritis & Rheumatology Research Alliance (CARRA) systemic Juvenile Idiopathic Arthritis Consensus Treatment Plans

ObjectivesTo assess the feasibility of studying the comparative effectiveness of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans (CTPs) for systemic Juvenile Idiopathic Arthritis (JIA) using an observational registry.MethodsUntreated systemic JIA patients enrolled in the CARRA Registry were begun on one of 4 CTPs chosen by the treating physician and patient/family (glucocorticoid [GC] alone; methotrexate [MTX] ± GC; IL1 inhibitor [IL1i] ± GC; IL6 inhibitor [IL6i] ± GC). The primary outcome of clinical inactive disease (CID) without current GC use was assessed at 9 months. Trial registration: clinicaltrials.gov NCT01697254; first registered 9/28/12 (retrospectively enrolled).ResultsThirty patients were enrolled at 13 sites; eight patients were started on a non-biologic CTP (2 GC, 6 MTX) and 22 patients on a biologic CTP (12 IL1i, 10 IL6i) at disease onset. Demographic and disease features were similar between CTP groups. CTP choice appeared to segregate by site preference. CID off GC was achieved by 37% (11 of 30) including 11/22 (50%) starting a biologic CTP compared to 0/8 starting a non-biologic CTP (p = 0.014). There were four serious adverse events: two infections, one appendicitis and one macrophage activation syndrome.ConclusionsThe CARRA systemic JIA CTP pilot study demonstrated successful implementation of CTPs using the CARRA registry infrastructure. Having demonstrated feasibility, a larger study using CTP response to better determine the relative effectiveness of treatments for new-onset systemic JIA is now underway.

Childhood Arthritis and Rheumatology Research Alliance consensus clinical treatment plans for juvenile dermatomyositis with skin predominant disease

BackgroundJuvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript.MethodsThe Children’s Arthritis and Rheumatology Research Alliance (CARRA) is a North American consortium of pediatric rheumatology health care providers. CARRA members collaborated to determine consensus on typical treatments for JDM patients with skin findings without significant weakness, to develop CTPs for this subgroup of patients. We used a combination of Delphi surveys and nominal group consensus meetings to develop these CTPs.ResultsConsensus was reached on patient characteristics and outcome assessment, and CTPs were developed and finalized for patients with skin predominant JDM. Treatment option A included hydroxychloroquine alone, Treatment option B included hydroxychloroquine and methotrexate, and Treatment option C included hydroxychloroquine, methotrexate and corticosteroids.ConclusionsThree CTPs were developed for use in children with skin predominant JDM, which reflect typical treatment approaches. These are not considered to be specific recommendations or standard of care. Using the CARRA network and prospective data collection, we will be able to apply statistical methods in the future to allow comparisons of JDM patients following these consensus treatment plans.

Physicians' Perspectives on the Diagnosis and Treatment of Chronic Nonbacterial Osteomyelitis.

Background/Purpose. Understanding the practices of pediatric rheumatologists in diagnosing and treating chronic nonbacterial osteomyelitis (CNO) can provide important information to guide the development of consensus treatment plans. The objectives of this study were to determine physicians' approaches to (1) diagnosing and monitoring CNO, (2) ordering a bone biopsy, and (3) making treatment decisions. Methods. A survey was distributed among members of the Childhood Arthritis and Rheumatology Research Alliance using a web-based questionnaire. Results. 121 of 277 (41%) attending physician members completed the survey. Plain radiographs (89%) were most commonly used followed by regional MRI (78%), bone scintigraphy (43%), and whole-body MRI (36%). The top three reasons for performing a biopsy were constitutional findings (66%), unifocal bone lesions (64%), and nocturnal bone pain (45%). Nearly all responders (95%) prescribed nonsteroidal anti-inflammatory drugs (NSAIDs) as initial therapy. For patients who failed NSAID treatment, methotrexate (67%), tumor necrosis factor inhibitors (65%), and bisphosphonates (46%) were the next most commonly used treatments. The presence of a spinal lesion increased the use of bisphosphonate treatment. Conclusion. The diagnostic approach and disease activity monitoring for CNO varied among surveyed physicians. Our survey findings provided important background for the development of consensus treatment plans for CNO.

Childhood Arthritis and Rheumatology Research Alliance Consensus Clinical Treatment Plans for Juvenile Dermatomyositis with Persistent Skin Rash.

Juvenile dermatomyositis (JDM) is the most common form of idiopathic inflammatory myopathy in children. While outcomes are generally thought to be good, persistence of skin rash is a common problem. The goal of this study was to describe the development of clinical treatment plans (CTP) for children with JDM characterized by persistent skin rash despite complete resolution of muscle involvement. The Childhood Arthritis and Rheumatology Research Alliance, a North American consortium of pediatric rheumatologists and other healthcare providers, used a combination of Delphi surveys and nominal group consensus meetings to develop CTP that reflected consensus on typical treatments for patients with JDM with persistent skin rash. Consensus was reached on patient characteristics and outcome assessment. Patients should have previously received corticosteroids and methotrexate (MTX). Three consensus treatment plans were developed. Plan A added intravenous immunoglobulin (IVIG) if it was not already being used. Plan B added mycophenolate mofetil, while Plan C added cyclosporine. Continuation of previous treatments, including corticosteroids, MTX, and IVIG, was permitted in plans B and C. Three consensus CTP were developed for use in children with JDM and persistent skin rash despite complete resolution of muscle disease. These CTP reflect typical treatment approaches and are not to be considered treatment recommendations or standard of care. Using prospective data collection and statistical methods to account for nonrandom treatment assignment, it is expected that these CTP will be used to allow treatment comparisons, and ultimately determine the best treatment for these patients.

Progress and prognosis in juvenile dermatomyositis

With the advent of corticosteroids as a treatment option for autoimmune disease in the early 1960s, the course of juvenile dermatomyositis was altered from one of high mortality to one with various degrees of morbidity. Prior to treatment with corticosteroids, juvenile dermatomyositis resulted in recovery, recovery with chronic disability or death. Corticosteroids significantly decreased mortality and by modifying disease course, morbidity but introduced additional complications. Biomarkers of disease activity, as well as predictors of disease course and severity, are lacking but are a focus of current investigation. Improved understanding of pathogenesis has expanded medication choices to treat both new-onset and refractory disease. Published consensus treatment plans are being implemented, facilitating studies of comparative effectiveness and toxicity.

Adding Canakinumab to the Childhood Arthritis and Rheumatology Research Alliance Consensus Treatment Plans for Systemic Juvenile Idiopathic Arthritis: Comment on the Article by DeWitt et al

Adding Canakinumab to the Childhood Arthritis and Rheumatology Research Alliance Consensus Treatment Plans for Systemic Juvenile Idiopathic Arthritis: Comment on the Article by DeWitt et al

Childhood Arthritis and Rheumatology Research Alliance Consensus Treatment Plans for New‐Onset Polyarticular Juvenile Idiopathic Arthritis

There is no standardized approach to the initial treatment of polyarticular juvenile idiopathic arthritis (JIA) among pediatric rheumatologists. Understanding the comparative effectiveness of the diverse therapeutic options available will result in better health outcomes for polyarticular JIA. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans (CTPs) for use in clinical practice to facilitate such studies. A case-based survey was administered to CARRA members to identify the common treatment approaches for new-onset polyarticular JIA. Two face-to-face consensus conferences employed modified nominal group technique to identify treatment strategies, operational case definition, end points, and data elements to be collected. A core workgroup reviewed the relevant literature, refined plans, and developed medication dosing and monitoring recommendations. The initial case-based survey identified significant variability among treatment approaches for new-onset polyarticular JIA. We developed 3 CTPs based on treatment strategies for the first 12 months of therapy, as well as case definitions and clinical and laboratory monitoring schedules. The CTPs include a step-up plan (nonbiologic disease-modifying antirheumatic drug [DMARD] followed by a biologic DMARD), an early combination plan (nonbiologic and biologic DMARD combined within a month of treatment initiation), and a biologic only plan. This approach was approved by 96% of the CARRA JIA Research Committee members attending the 2013 CARRA face-to-face meeting. Three standardized CTPs were developed for new-onset polyarticular JIA. Coupled with data collection at defined intervals, use of these CTPs will enable the study of their comparative effectiveness in an observational setting to optimize initial management of polyarticular JIA.

A22: Consensus Treatment Plans for Induction Therapy in Childhood Proliferative Lupus Nephritis—Status of Use in Daily Clinical Care

Background/Purpose:Consensus treatment plans (CTPs) serve as an alternative strategy to randomized controlled trials for assessing treatment effects. The CTPs for induction therapy of childhood proliferative lupus nephritis (cLN) were developed for use in clinical care; they are based on best available evidence and endorsed by CARRA Pediatric Rheumatologists. They specify two options for immunosuppressive therapy (IV Cyclophosphamide (CP) and oral Mycophenolate (MMF)) and three options for steroid therapy (primarily oral regimen, primarily IV regimen and mixed oral/IV regimen).The purpose of this pilot study was to assess the use of and adherence to the cLN‐CTPs, and to estimate rates of renal response and safety and tolerability. Preliminary results are presented here.Methods:Providers at 14 participating CARRA sites agreed to utilize the induction cLN‐CTPs for eligible patients. Eligible patients were <20 years old and had biopsy‐proven newly diagnosed proliferative cLN; however the CTPs were not intended for patients with concurrent infections, pregnancy or other severe SLE manifestations that would dictate specific therapies different from the cLN‐CTPs. Patients were invited to enroll in the CARRA registry for periodic collection of standard clinical SLE and renal outcome measures.Results:To date, 71 potential SLE patients with newly diagnosed proliferative cLN were cared for at 13 sites (87% female, mean age 14.6 yrs, mixed ethnicity [37% Hispanic] and race [41% Caucasian, 30% African American, 9% Asian, 20% other/unknown]). 41 (58%) patients were enrolled in the CARRA registry, a prerequisite for study participation. Reasons for non‐enrollment in the registry included failure of being approached in a timely fashion (34%), patients/parents declined participation (4%), and failure to complete consent due to not speaking English (1%). Among the 41 patients who were enrolled in the registry, 29 (43%) were treated according to one of the cLN‐CTPs. For induction immunosuppressive therapy, providers selected the CP CTP for 61%, and the MMF CTP for 39% of patients. For induction steroid therapy, providers selected the primarily IV regimen for 43%, mixed IV/oral regimen for 35%, and primary oral regimen for 17% of the patients. Reasons for not using one of the options specified in the CTPs included patients not meeting eligibility characteristics for their use (35%), providers choosing not to use the cLN‐CTP (13%), treatment initiated at another institution (7%), and the prescribing physician being unaware of the cLN‐CTPs (1.5%).Conclusion:A minority of patients with newly diagnosed proliferative cLN received therapy according to the cLN‐CTPs, which are considered the best therapeutic regimen based on available scientific evidence and expert opinion. Important outcome data from many patients was missed by patients not being approached for enrollment in the registry. This suggests that additional efforts are necessary to promote implementation of the cLN‐CTPs as we endeavor to perform comparative effectiveness studies and improve the prognosis for children with cLN.

A70: Medication Use in the Treatment of Juvenile Idiopathic Uveitis Patients Enrolled in the Childhood Arthritis and Rheumatology Research Alliance Registry

Background/Purpose:There is no clear standard of care for the treatment of juvenile uveitis when initial steroid therapy is insufficient. We sought to assess current prescribing practices in the treatment of this condition with steroid‐sparing medications. For this study, we focused on children with idiopathic, non‐infectious uveitis.Methods:This is a cross‐sectional cohort study of idiopathic uveitis patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) data registry. Clinical characteristics, including demographic information, presenting features, disease complications, and medications were collected. Descriptive statistics were used to characterize the cohort. Chi‐square and Fisher's exact t‐test analyses were used to assess for associations between medications and clinical characteristics.Results:There were 92 children with idiopathic uveitis identified, of which 41 (45%) were male. The majority (62%) had anterior uveitis, followed by panuveitis (21%), intermediate (13%), and posterior (4%) uveitis. Average age of onset was 8.4 years. Caucasians represented 77% of the cohort; 13% were Hispanic. Among the 76 patients (83%) who had been treated with a non‐biologic DMARD, 70 had received methotrexate (MTX). The initial form of administration (oral versus subcutaneous) was inferred based on categorization of medication use as “current,” “past,” or “never.” Of the 67 patients for whom timing of administration could be determined, 36 received subcutaneous MTX as initial therapy, while 31 initially received oral MTX. Caucasian patients were more likely to receive oral MTX first (p = 0.046) as compared to non‐Caucasians, and there was a trend towards subcutaneous administration in younger patients (mean 7.4 versus 9.1 years, p = 0.06). In addition, 49 patients (53%) had received a biologic agent, all TNF inhibitors: infliximab (n = 36), adalimumab (n = 18), or golimumab (n = 1). Of these, all but 2 had also received MTX. Use of a biologic agent was positively associated with cataract development (p = 0.003), although rates of vision loss and overall complications were not higher in patients treated with biologics. Patients who had received a biologic agent were more likely to have a positive ANA, although this association was not statistically significant (p = 0.07). Age, race, and sex were not associated with the use of biologics.Conclusion:Most children with idiopathic uveitis enrolled in the CARRA registry were treated with methotrexate, ith the form of administration divided equally between oral and subcutaneous dosing. Factors associated with initial use of oral versus subcutaneous MTX include race and possibly age. Patients who received a biologic agent were more likely to have cataracts, suggesting that biologics might be used more often in patients with more severe disease or those requiring chronic corticosteroids. However, the overall uveitis complication rate was not higher in patients treated with biologics. Many variables likely contribute to the selection of medications used to treat juvenile uveitis; further elucidation of these factors will be necessary for the development of consensus treatment plans.