Pediatric Rheumatology Centers Research Articles

Features of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study.

To evaluate demographic data and clinical and laboratory features at disease diagnosis in 3 different age groups of childhood-onset systemic lupus erythematosus (SLE): group A, early-onset (<6 years); group B, school age (≥6 to <12 years); and group C, adolescent (≥12 to <18 years). This was a Brazilian multicenter cohort retrospective study in 10 pediatric rheumatology centers, including 847 childhood-onset SLE patients. Patients were divided into 3 groups: group A with 39 patients (4%), group B with 395 patients (47%), and group C with 413 patients (49%). Of 39 childhood-onset SLE patients in group A, 3 (8%) were ages <2 years, 4 (10%) were ≥2 to <3 years, and 32 (82%) were ≥3 and <6 years. A total of 74 childhood-onset SLE patients were analyzed for C1q levels, and complete C1q deficiency was observed in 3 of 74 patients (4%), all in group A. Groups were similar regarding high frequencies of female sex, nephritis, neuropsychiatric involvement, Systemic Lupus Erythematosus Disease Activity Index 2000 score ≥8, autoantibody profile, elevated acute phase proteins, and low complement levels (P > 0.05). However, the frequency of fever (78% versus 61% versus 47%; P < 0.0001), hepatomegaly (42% versus 29% versus 14%; P < 0.0001), splenomegaly (28% versus 12% versus 4%; P < 0.0001), and discoid lupus (13% versus 4% versus 4%; P = 0.020) was significantly higher in group A compared to groups B and C. The frequency of weight loss >2 kg (19% versus 28% versus 36%; P = 0.017), photosensitivity (34% versus 41% versus 51%; P = 0.006), leukopenia <4,000/mm3 (14% versus 25% versus 30%; P = 0.048), and lymphopenia <1,500/mm3 (22% versus 41% versus 47%; P = 0.011) was significantly lower in group A. Our large multicenter study identified the finding that the initial appearance of childhood-onset SLE is characterized by comparable high frequency of internal organ involvement and some distinct clinical and laboratory features in early-onset and adolescent groups.

Pancreatitis Subtypes Survey in 852 Childhood-Onset Systemic Lupus Erythematosus Patients.

Pancreatitis is a rare and a life-threatening systemic lupus erythematosus (SLE) manifestation in childhood-onset SLE (cSLE). The objective of this study was to systematically classify pancreatitis in cSLE according to the International Study Group of Pediatric Pancreatitis and determine the overall prevalence, clinical features, laboratory, and first episode outcomes. A multicenter cohort study in 10 pediatric rheumatology centers, including 852 patients with cSLE. Pancreatitis was diagnosed in 22 of 852 (2.6%) patients with cSLE. It was classified as acute pancreatitis in 20 (91%), acute recurrent pancreatitis in 2 (9%), and none of them had chronic pancreatitis. None of them had gallstones, traumatic pancreatitis, or reported alcohol/tobacco use. The comparison of patients with pancreatitis (first episode) and without this complication revealed a shorter disease duration (1 [0-10] vs 4 [0-23] years, P < 0.0001) and higher median of Systemic Lupus Erythematosus Disease Activity Index 2000 (21 [0-41] vs 2 [0-45], P < 0.0001). The frequencies of fever (P < 0.0001), weight loss (P < 0.0001), serositis (P < 0.0001), nephritis (P < 0.0001), arterial hypertension (P < 0.0001), acute renal failure (P < 0.0001), macrophage activation syndrome (P < 0.0001), and death (P = 0.001) were also higher in patients with pancreatitis. The frequencies of intravenous methylprednisolone use (P < 0.0001) and the median of prednisone dose (55 [15-60] vs 11 [1-90] mg/day, P < 0.0001) were significantly higher in patients with pancreatitis. Of note, the 2 patients with acute recurrent pancreatitis had 2 episodes, with pain-free interval of 1 and 4 years. This was the first study characterizing pancreatitis using the International Study Group of Pediatric Pancreatitis standardized definitions in patients with cSLE showing that the predominant form is acute pancreatitis seen in association with glucocorticoid treatment and active severe disease.

Initial Benchmarking of the Quality of Medical Care in Childhood-Onset Systemic Lupus Erythematosus.

To assess the quality of medical care in childhood-onset systemic lupus erythematosus (SLE) at tertiary pediatric rheumatology centers as measured by observance of SLE quality indicators (SLE-QIs). International consensus has been achieved for childhood-onset SLE-QIs capturing medical care provision in 9 domains: diagnostic testing, education of cardiovascular (CV) risk and lifestyles, lupus nephritis (LN), medication management, bone health, ophthalmologic surveillance, transition, pregnancy, and vaccination. Using medical record information, the level of performance of these childhood-onset SLE-QIs was assessed in childhood-onset SLE populations treated at 4 tertiary pediatric rheumatology centers in the US, 2 in Brazil, and 1 center in India. A total of 483 childhood-onset SLE patients were assessed. Care for the 310 US patients differed markedly for childhood-onset SLE-QIs addressing LN, bone health, vaccinations, education on CV risk, and transition planning. Performance of safety blood testing for medications was high at all centers. Despite often similar performance on the childhood-onset SLE-QI, access to kidney biopsies was lower in Brazil than in the US. Irrespective of the country of practice, larger centers tended to meet the childhood-onset SLE-QIs more often than smaller centers. The childhood-onset SLE-QIs, evidence-based minimum standards of medical care, are not consistently met in the US or some other countries outside the US. This has the potential to contribute to suboptimal childhood-onset SLE outcomes.

Choosing the right treatment for patients with a severe course of chronic non-bacterial osteomyelitis (CNO) - pamidronate or TNF-α blockade?

Chronic non-bacterial osteomyelitis (CNO) is an inflammatory, non-infectious disorder of the skeletal system with unknown aetiology. Therapeutic options are NSAIDs, steroids and DMARDs (MTX or sulfasalazine). However, a considerable number of patients have a severe disease course and bisphosphonates or TNF-α blockade might be a therapeutic option. We performed a multicentre, retrospective chart review of all patients diagnosed with CNO in two paediatric rheumatology centres in the last 10 years and treated with pamidronate and/or TNF-α blockade. 17 patients were treated with pamidronate and/or TNF-α blockade. Out of these 17 patients, 10 were treated with pamidronate alone and showed clinical improvement. Three of the 17 patients were initially treated with TNF-α blockade; two had a positive response, one patient stopped therapy due to minor side effects. Two or one patients were initially treated with pamidonate or TNF-α blockade, respectively, but did not show clinical improvement. Interestingly, switching from pamidronate to TNF-α blockade or vice versa was associated with clinical improvement in these patients. One patient was treated with a combination therapy using pamidronate and TNF-α blockade and showed a good clinical response. Pamidronate and TNF-α blockade showed a good response in therapy refractory CNO patients. Severe adverse effects were not observed. However, some patients seem to benefit from one of the two therapeutic options in particular. Future studies are needed aiming to identify clinical and/or radiological parameters that might guide the decision to the appropriate treatment in an individual patient.

Identification of type I interferonopathies using blood interferon signature: the experience of a pediatric rheumatology center

Methods We collected blood samples from a cohort of pediatric rheumatologic patients and scored them according to a qPCR based IFN gene signature assay. Expression of 6 type I IFN-related genes (IFI27, IFI44L, IFIT1, ISG15, RSAD2, SIGLEC1) was quantified by standard RTPCR techniques. An IFN score was calculated for each patient using the median fold change of gene expression related to a healthy control. Patients were selected based on the presence of the following features: i) atypical or incomplete SLE-like symptoms occurring in infancy or in preprepubertal age; ii) vasculopathy (skin ulcers, chilblains, strokes) iii) panniculitis with or without lypodystrophy iv) interstitial lung disease in the context of systemic inflammation.

A randomized study of local anesthesia for pain control during intra-articular corticosteroid injection in children with arthritis.

BackgroundIntra-articular corticosteroid injections (IACI) are routinely used by pediatric rheumatologists in the treatment of chronic arthritis. Frequently, topical anesthetics are used to control procedural pain, but their relative efficacy has not been reported. In this study, we evaluated the level of pain associated with different anesthetic methods, Numby® 900 Iontophoretic Drug Delivery System, or EMLA® cream, with or without subcutaneous buffered lidocaine (SQBL), during IACI of the knee in children with arthritis.MethodsWe conducted a prospective study of patients, ages 4 to 21 years old, followed at three pediatric rheumatology centers who were undergoing IACI of a knee joint. Patients were randomized into two treatment groups: 1) topical anesthetic only (EMLA® or Numby® (E/N)), or 2) topical anesthetic (E/N) and SQBL. Pain was assessed at baseline, during topical anesthetic placement, and following the IACI (post-procedure). The Faces Pain Scale-Revised (FPS-R), the Face, Leg, Activity, Cry, Consolability (FLACC) behavioral scale and the parental global assessment (PGA) (0 = best experience, 10 = worst experience) were determined.ResultsSixty-three patients (44 females) with a median [IQR] age of 10.8 [IQR = (8.2–14.4)] years (range 4.7–20 years) with active knee arthritis were consented. FPS-R post-procedure (P = 0.03), FLACC (P = 0.02) and PGA (P = 0.01) scores were significantly lower in females treated with E/N plus SQBL compared to patients treated with E/N only. Females in the E/N only group had a significant worsening of their baseline pain (p < 0.0004) and a greater magnitude of change in their baseline FPS-R scores (p < 0.001) from the procedure compared to females in the E/N plus SQBL group who had no worsening of their baseline pain. No significant change in pain level or PGA score was found among males in either treatment group. Pain scores overall were similar to the oligoarthritis patients, a more homogeneous group of patients. Both EMLA® (n = 33) and Numby® (n = 29) were equally well tolerated with no significant difference in median FPS-R administration scores overall.ConclusionOur results suggest that a topical anesthetic plus SQBL is more effective for injection pain control than topical anesthesia only. Further studies addressing pain and anxiety will help determine the optimal method of pain control for IACI.

Long-term outcome of children with pediatric-onset cutaneous and visceral polyarteritis nodosa

ObjectiveTo assess the prognostic impact of clinical presentation in children with polyarteritis nodosa (PAN). MethodsChildren diagnosed between 1986 and 2006 in a tertiary care pediatric rheumatology center were classified as “cutaneous PAN” (group 1), “cutaneous PAN with significant extra-cutaneous features” (group 2) or “visceral childhood PAN” (group 3). Outcome measures: (1) clinical remission off-therapy at last follow-up, (2) requirement and length of glucocorticoid therapy, (3) presence of disease-related sequelae. ResultsTwenty-nine children were included. Sixteen met the Ankara criteria for PAN. Nine patients were qualified as group 1, 11 as group 2, and 9 as group 3. At last follow-up, 15 children were in clinical remission off-therapy: 4 from group 1 (44%), 4 from group 2 (36%) and 7 from group 3 (78%). Glucocorticoid therapy was required for 8 (89%), 7 (64%) and 7 (78%) patients from groups 1, 2 and 3, respectively. Seven children did not require any glucocorticoid therapy. Time-dependent probability of achieving glucocorticoid-free clinical remission was similar between the three groups. Three patients (one from each group) had digital ischemia leading to amputation. There were no significant between-group differences in outcome based on the three outcome measures addressed. ConclusionOutcome was not strikingly predictable from initial presentation in children with PAN. The organ distribution-based distinction between cutaneous and visceral PAN had little prognostic power in this series.

Multicenter retrospective study of biological tolerance in juvenile idiopathic arthritis (jir-cohort)

Ten years after the introduction of biologics in children, we assess the tolerance of these therapies among patients with juvenile idiopathic arthritis (JIA) in pediatric rheumatology centers.

A controlled trial of intra-articular corticosteroids with or without methotrexate in oligoarticular juvenile idiopathic arthritis

In contrast with the numerous controlled trials conducted in polyarticular or systemic juvenile idiopathic arthritis (JIA), little evidence-based information is available for oligoarticular JIA. As a result, the management of children with this subtype, which is the most prevalent in Western countries, is largely empiric. Intra-articular corticosteroid (IAC) injection is the therapy of first choice for oligoarthritis in many pediatric rheumatology centers. However, although IAC injections are usually highly efficacious, relapses of synovitis are common and sometimes occur only a few months after the procedure. It is still unclear whether concomitant administration of methotrexate (MTX) may increase and prolong the effectiveness of IAC injections.

Intra-articular steroid injections: 5 years' experience in a tertiary paediatric rheumatology centre

Intra-articular steroid (IAS) injections are a safe and effective therapy in the management of Juvenile Idiopathic Arthritis (JIA). Timely access to joint injections, including appropriate anaesthesia, is now considered standard of care in the treatment of JIA. Despite this, little is known about the resource burden created by IAS provision. Published data is also lacking to inform families of the likely role that IAS will play in the management of their child’s disease.

Chronic recurrent multifocal osteomyelitis: experience from a single pediatric rheumatology center over the past ten years

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare inflammatory disorder that primarily affects children. Its hallmark is recurring episodes of sterile osteomyelitis. The clinical presentation is insidious bone pain with or without fever. Pathogenesis is still unknown and there is no any effective treatment.

Frequency of joint involvement in juvenile idiopathic arthritis during a 5-year follow-up of newly diagnosed patients: implications for MR imaging as outcome measure.

To assess the sequence and type of active joints in a cohort of newly diagnosed juvenile idiopathic arthritis (JIA) patients with full access to current treatment at first visit and during a follow-up period of 5-years, in order to identify an index joint/group of joints for magnetic resonance imaging in JIA. Patient charts of all consecutive newly diagnosed JIA patients with a follow-up duration of at least 5 years were analyzed. Patients were derived from two tertiary pediatric rheumatology centers. Patient characteristics and data concerning the presence of joints with arthritis and the use of medication were recorded. Findings from 95 JIA patients [39 (41 %) oligoarticular and 56 (59 %) polyarticular] were analyzed. At first visit, distribution of active joints among patients was as follows: knee (n = 70, 74 %), ankle (n = 55, 58 %), elbow (n = 23, 24 %), wrist (n = 23, 24 %), metacarpophalangeal (MCP) (n = 20, 21 %), proximal interphalangeal (PIP) (n = 13, 14 %), hip (n = 6, 6 %), shoulder (n = 5, 5 %), and distal interphalangeal (DIP) (n = 4, 4 %) joints. After a follow-up period of 5 years, the cumulative percentage of patients with specific joint involvement changed into: knee (n = 88, 93 %), ankle (n = 79, 83 %), elbow (n = 43, 45 %), wrist (n = 38, 40 %), MCP (n = 36, 38 %), PIP (n = 29, 31 %), shoulder (n = 20, 21 %), hip (n = 17, 19 %), and DIP (n = 9, 10 %) joints. Despite changes in treatment strategies over the years, the knee remains the most commonly involved joint at onset and during follow-up in JIA, followed by the ankle, elbow, and wrist. For the evaluation of outcome with MRI, the knee appears the most appropriate joint in JIA.

Interventions for morphea

Interventions for morphea

FRI0547 Predictive Factors of TMJ Involvement in 230 Consecutive JIA Patients Regularly Screened from 2000 to 2014 in A Single Pediatric Rheumatology Centre

BackgroundJuvenile Idiopathic Arthritis (JIA) has been shown to involve frequently the temporo-mandibular joint (TMJ). An important prognostic aspect is to diagnose and treat it promptly.ObjectivesTo evaluate in a consecutive JIA...

The outcomes of juvenile idiopathic arthritis in children managed with contemporary treatments: results from the ReACCh-Out cohort

ObjectiveTo describe clinical outcomes of juvenile idiopathic arthritis (JIA) in a prospective inception cohort of children managed with contemporary treatments.MethodsChildren newly diagnosed with JIA at 16 Canadian paediatric rheumatology centres...

G358 The performance of the new SLICC criteria for the classification of SLE in children

AimsThe Systemic Lupus International Collaborating Clinics (SLICC) have recently suggested a new set of criteria for the classification of SLE. We aimed to compare the sensitivity and specificities of the...

A8: Juvenile Idiopathic Arthritis with Dry Synovitis: Clinical and Imaging Aspects in a Cohort of 10 Patients

Background/Purpose:Dry synovitis is considered to be a rare form of JIA, incompletely understood and following a potentially destructive course. Since its first description only a few reports mention this entity within the spectrum of JIA. We aimed to describe the clinical and radiological manifestations, and the course of dry synovitis and to compare it with classical RF negative polyarticular JIA.Methods:A retrospective study of 10 patients in 3 pediatric rheumatology centers who presented with 1. progressive, symmetric polyarticular stiffness with limitations and without clinical synovitis, 2. radiologic signs of articular damage and 3. Absence of inflammatory parameters (CRP, ESR). Neuromuscular conditions and metabolic storage/bone disorders were excluded. This cohort was compared with 35 consecutive patients with classical polyarticular RF negative JIA. Clinical, laboratory and imaging (X‐rays, MRI) data of both groups were reviewed.Results:The dry synovitis cohort included 4 boys and 6 girls. The median (range) age at first manifestation and diagnosis were respectively 4.3 yrs (1.1–8.9) and 7.3 yrs (2.8–14). Presenting signs comprised delayed motor development and/or progressive articular stiffness. Pain complaints emerged with time. Clinical examination showed a symmetric polyarticular involvement without clinical signs of synovitis. CRP was normal and ESR &lt; 8mm/h. In 9/10 patients ANA were absent. Radiological imaging (wrist, ankles and/or pelvis) showed osteopenia, advanced bone age, cartilage loss and/or bone erosions in all patients. MRI revealed synovial thickening in 9/10 patients; bone edema was variably present and bone erosions were demonstrated in 8/10 patients. Treatments comprised NSAIDs (n = 8/10), corticosteroids (n = 8/10), methotrexate (n = 9/10), anti‐TNF (n = 6/10), IL‐1 receptor antagonist (n = 1/10) and resulted in some subjective improvement. However articular limitations persisted and imaging showed progressive (osteo)articular damage. The comparison of the dry synovitis with polyarticular JIA patients showed that the age at disease onset was comparable in the 2 groups. Significant differential features found in the dry synovitis cohort were higher diagnostic delay, increased number of joint involvement and contractures, lack of swelling and tenderness, and lack of increased acute phase response (CRP, ESR) and ANA.Conclusion:From our data, we conclude that ‘dry synovitis’ can be considered as a clinical descriptive term referring to the development of progressive symmetric polyarticular limitations. We show that MRI is usefull in demonstrating underlying subclinical inflammation and in combination with clinical features and standard X‐rays helpful in the differential diagnosis with mucopolyssaccharidosis, neuromuscular disorders and bone dysplasia. Clinical as well as radiological evolution seem to be refractory to the current JIA therapy and to be different among patients diagnosed with ‘dry synovitis,’ suggesting that this clinical manifestation may represent a heterogeneous spectrum of joint and/or bone disorders. The exact nature of this entity and a possible contribution of a metabolic or intrinsic bone disorder into its pathogenesis remain to be determined.

A151: Pediatric Rheumatology Care and Outcomes Improvement Network Demonstrates Performance Improvement on Juvenile Idiopathic Arthritis Quality Measures

Background/Purpose: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).Methods: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.Results: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.Conclusion: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.

A133: Initial Benchmarking of the Quality of Medical Care of Children and Adolescents with Lupus

Background/Purpose:Recently, 26 quality indicators (QI) categorized into 9 domains for childhood‐onset systemic lupus erythematous (cSLE) have been developed based on international consensus and scientific evidence. QI are defined as minimum standards of medical care in support of optimal disease outcomes. The current level at which these QI are followed has not been well documented. Hence, the objective of this study is to assess the current quality of medical care received by patients with cSLE at tertiary pediatric rheumatology centers.Methods:Cross‐sectional data pertaining to the QI were acquired via chart review and analyzed collectively in 290 cSLE patients followed at four tertiary pediatric rheumatology centers—two in the United States and two in Brazil.Results:Adherence to the QI differed by QI domain, ranging from 41 to 100%. The QI domain with the highest adherence 100% was Laboratory Testing at the time of diagnosis, and the lowest domain 41% was Medication Management. The recommended kidney biopsies for newly diagnosed lupus nephritis were done in only 67% of the patients and 85% of the patients received at least one of the recommended vaccinations (Table ). Cardiovascular risks factors were discussed in 86% of cases when patient was 13 years or older. Adherence to the QI differences between countries and insurance status will be presented, supporting that the set of current QI are suitable for international use. Adherence to QI by Domain Quality Indicators by Domain Results Lab testing at diagnosis &amp; screening Obtained diagnostic/confirmatory labs within first two visits 100% Obtained lab surveillance of CBC, renal, LFT every 12 months 85% General prevention Prescribed influenza and/or encapsulated organisms vaccination, unless contraindicated 85% Discussed and documented education on sun avoidance at least once in the medical record (e.g., wearing protective clothing, applying sunscreens whenever outdoors, and avoiding sunbathing) 89% Discussed transition plan to appropriate adult healthcare providers with patient age ≥14 years. 78% Lupus nephritis (LN) and hypertension management Kidney biopsy discussed/ordered/performed if developed proteinuria &gt;500 mg/day, or worsening GFR, or urinary sediment 60% Evaluated by a nephrologist in the last year of care for lupus nephritis (LN) and of hypertension 65% Evaluated by rheumatologist every 3 months in last year if a patient has known LN regardless of disease activity. 96% Received kidney biopsy when diagnosed with LN 67% Class III/IV, treated with immunosuppressive + glucocorticoids within 1 month 92% Angiotensin receptor blocker (ARB) or, Angiotensin–converting enzyme (ACE) inhibitor were prescribed if ongoing proteinuria &gt;500 mg/day or worsening GFR in last year of care. 96% Check assessments (Kidney function, urine sediment, and proteinuria) every three months. 99% Medication Management If started new meds, discussed risk vs. benefit of therapy 97% Currently prescribed any antimalarial therapy 92% Attempted to taper a dose of steroids not acceptable for chronic use. 89% Attempted to taper and unable to decrease steroid; added/increased steroid sparing agent. 41% Surveillance for medication safety done at regular intervals. 99% Bone Health Received at least one bone mineral density testing DEXA scan 72% Repeat bone mineral density testing if baseline testing outside normal limits (Z score ≤2) 62% Prescribed calcium/vitamin D if a patient is on any steroid therapy. 78% Ophthalmological surveillance Receives eye exams annually while on anti‐malarial therapy 82% Receives eye exams annually while on glucocorticoids 85% Education on cardiovascular risk factors Education on cardiovascular risk factors (smoking, hypertension, high BMI) every 1 year with patient and parent if patient is 13 years or older. 86% Discussed lifestyle modifications (smoking cessation, weight control, exercise) every 2 years with parent and patient 13 years or older. 87% Pregnancy Anti‐SSA, anti‐SB and anti‐phospholipid antibodies have been assessed during pregnancy 100% CNS health Prescribed Immunosuppressive therapy if patient has major neuropsychiatric manifestations in last year of care (Optic neuritis, coma, psychosis … etc.) 94%Conclusion:Based on this initial benchmarking effort, the medical care of patients with cSLE at tertiary pediatric rheumatology centers is very good. Systematic planning and documentation of patient education on lifestyle modifications seems warranted and is expected to improve the self‐management skills of cSLE patients. Furthermore, increased focus on bone health, eye exams annually, and adequate vaccination of cSLE patients are areas to continue to monitor.

A44: High Levels of DEK Autoantibodies May Predict Early Flare Following Cessation of Anti‐TNF Therapy

Background/Purpose:The nuclear oncoprotein DEK is a biochemically distinct protein, modulating heterochromatin integrity, chemoattractant of neutrophils and T‐cells and vital for the formation of neutrophil extracellular traps (NETs). NETs are important for resolution of inflammation suggesting that DEK contributes to the development of autoimmune diseases. High levels of DEK autoantibodies have been found in several autoimmune diseases including juvenile idiopathic arthritis (JIA) but their role in disease pathogenesis is not clear. Since DEK and DEK autoantibodies can contribute to the development of immune complexes and NET formation we suggest that DEK antibody levels can predict flare with the discontinuation of anti‐TNF therapy.Methods:In16 pediatric rheumatology centers, sera samples were collected from 137 children with polyarticular JIA with clinically inactive disease (CID) on anti‐TNF therapy. The therapy was stopped and disease activity was monitored for at least 8 months or until disease flare. DEK antibody levels were measured in sera collected at time of enrollment and 6 months (when anti‐TNF therapy was stopped) by ELISA. DEK antibody levels relative to healthy controls were calculated by area under the curve (AUC), expressed as unit‐free ratios.Results:103 females and 34 males patients were enrolled. Mean age 11.3 years and disease duration 5.0 years. JIA included: 13% extended oligoarthritis, 74% polyarthritis rheumatoid factor (RF) negative and 12 % polyarthritis RF positive and 46% positive ANA. 77% were taking etanercept, 18% adalimumab, 5% infliximab and 40% methotrexate. 31 patients (23%) discontinued the study prior stopping the therapy for various reasons, including loss of CID. Of 106 subjects who stopped the therapy, 39 (37%) flared within 8 months (mean of 104.8 days). 67 subjects (63%) had no flares within 8 months after stopping the therapy. 71 out of 106 patients samples were analyzed thus far for DEK antibody levels. DEK antibody level ratios compared to healthy controls was −0.36 (some patients had lower antibody levels than did healthy controls) to 1.41, median ratio of 0.11 (Q1–Q3 of 0.09–0.24) and 0.13 (SD, 0.3). High levels of DEK antibodies, mean and SD of 0.209 ± 0.36 were detected in the 21 patients that flared within 8 months compared to lower levels of DEK antibodies (0.09 ± 0.27) in 50 patients with CID for at least 8 months (ANOVA P= 0.1832). Negative correlation was observed between the days to flare and DEK antibody levels (spearman rho = −0.31, P = 0.07), suggesting that when therapy stopped, patients with higher levels of DEK antibodies will flare sooner with estimated odds ratio of 3.3 (95% CI, 0.61, 18.0), suggesting that each unit increase in DEK antibodies is associated (P = 0.17) with more than a 2‐fold increased risk of flare within 8 months.Conclusion:In children with JIA extended oligoarthritis and polyarthritis on anti‐TNF therapy that maintain CID for at least 6 months while on therapy, high DEK antibody levels may be correlated with flare within the first 8 months after stopping the therapy. This study suggests that DEK antibody levels can predict the outcome of discontinuation of anti‐TNF therapy, although more patient samples need to be analyzed from this study and future studies.