Diagnosis Of JIA Research Articles

Joint Manifestations in Children with Familial Mediterranean Fever in Armenia: Clinical and Genetic Characteristics

Familial Mediterranean fever (FMF) is an autosomal-recessive autoinflammatory disease in the group of Hereditary periodic fever syndromes (HPFS), characterized by recurrent, self-limited attacks of fever and polyserositis, which last 2-4 days. It manifests mainly in childhood and often has early onset, resolve without sequels. Genetic testing of FMF is efficient and allows diagnosing of atypical cases. FMF is a significant health care problem in Armenia because of high frequency of carriers of MEFV mutations 1:3 - 4 (0.21) and marked FMF prevalence 54.7 per 10`000 of the total population. Articular symptoms are found in about 45% of cases. They usually manifest as acute recurrent arthritis (ARA) or arthralgia, but more rarely also chronic arthritis can be found. We aimed to investigate clinical and genetic characteristics of the joint manifestations in Armenian children with FMF. The charts of all 715 patients with proven diagnosis of FMF at the National Pediatric Center (NPC) for FMF were reviewed for joint manifestations. The diagnosis of FMF and disease severity were determined according to the Tel-Hashomer criteria and molecular-genetic detection of 12 MEFV mutations common for Armenians. The characteristics of joint manifestations and overall FMF disease were then compared to MEFV mutation analysis. There were 438 boys and 277 girls with an age at diagnosis between 3 months and 17 years (mean age: 8.64±0.17). Joint involvement was observed in 56.4% of all 715 cases. The manifestation was ARA in 30.5%, arthralgia in 21.2% and chronic arthritis (CA) in 4.7%, which would also qualify for a diagnosis of JIA. The frequency of ARA was associated with M694V mutation, mainly M694V homozygous and M694V heterozygous genotypes. The risk of CA depended on MEFV genotype of the FMF patients and was the highest in M694Vheterozygotes and M694Vhomozygotes. M694V heterozygous genotype was noticed significantly more frequently among FMF patients with spondyloarthritis in comparison to those without it. The probability of the development of CA among FMF patients without M694V mutation was significantly higher also in heterozygotes in comparison to compound-heterozygotes. We concluded, that the frequency of joint involvement among Armenian pediatric FMF patients was high - 56.4%, especially for CA. Chronic arthritis may be the first manifestation of FMF and occurred usually in patients with severe M694V mutations. Patients of Armenian origin with refractory arthritis should be asked for isolated febrile attacks, hemorrhagic vasculitis, episodes of pleuritis and family history to rule out FMF.

Clinical significance of interleukin-18 for the diagnosis and prediction of disease course in systemic juvenile idiopathic arthritis.

To investigate the clinical significance of serum IL-18 levels for the diagnosis of systemic JIA (s-JIA) and to predict the disease course of s-JIA. Overall, 116 patients with s-JIA, 151 with other diseases and 20 healthy controls were analysed. Serum IL-18 levels were measured longitudinally in 41 patients with s-JIA from active phase through remission phase. Serum IL-18 levels were quantified via enzyme-linked immunosorbent assay and the results were compared with clinical features and the disease course of s-JIA. The serum IL-18 level cut-off value for differentiation of s-JIA from other diseases was 4800 pg/ml. In patients with a monocyclic course, serum IL-18 levels steadily decreased during the inactive phase and low levels were sustained during remission. In contrast, in patients with a chronic course, elevated serum IL-18 levels were sustained even during the inactive phase. In patients with a polycyclic course, serum IL-18 levels were elevated during disease flares and normalized during the inactive phase. The serum IL-18 level cut-off value for diagnosis of remission in s-JIA was 595 pg/ml. Serum IL-18 levels of >4800 pg/ml may be useful for differentiating between s-JIA and other diseases. Monitoring of serum IL-18 levels might be useful for predicting the disease course and assessing remission in s-JIA.

Genomic risk scores for juvenile idiopathic arthritis and its subtypes

ObjectivesJuvenile idiopathic arthritis (JIA) is an autoimmune disease and a common cause of chronic disability in children. Diagnosis of JIA is based purely on clinical symptoms, which can be variable,...

O14 Transitions in diagnosis and between services in juvenile idiopathic arthritis: living and learning

O14 Transitions in diagnosis and between services in juvenile idiopathic arthritis: living and learning

A new Canadian inception cohort for juvenile idiopathic arthritis: The Canadian Alliance of Pediatric Rheumatology Investigators Registry

The aim was to describe the design, methods and initial findings of a new Canadian inception cohort of children with JIA, The Canadian Alliance of Pediatric Rheumatology Investigators (CAPRI) JIA Registry. The CAPRI JIA Registry was started in 2017 to collect information prospectively on children enrolled within 3 months of JIA diagnosis across Canada. The registry has a non-traditional modular design, with no artificially set times for registry visits to occur, streamlined multi-method data collection that requires 2-4 min per visit, and reports cumulative incidence of treatments, outcomes and adverse events calculated by Kaplan-Meier survival methods. A total of 166 patients, enrolled a median of 6 weeks after JIA diagnosis at 10 centres, were included. The median age at diagnosis was 9 years [interquartile range (IQR) 3, 13], 61% were female and 51% had oligoarticular JIA. The median three-variable clinical Juvenile Arthritis Disease Activity Score was 6.5 (IQR 4, 10) at enrolment, and the median time to first attainment of clinically inactive disease (CID) was 24 weeks (by 1 year, 81%). Within 1 year of diagnosis, 70% of patients had started a DMARD and 35% a biologic agent. The rates of adverse events and serious adverse events were 60 and 5.8 per 100 patient-years, respectively. This streamlined and flexible registry minimizes the burden of data collection and interference with clinic operations. Initial findings suggest that treatments for newly diagnosed patients with JIA in Canada have intensified, and now 81% of patients attain CID within 1 year of diagnosis.

Niveles de vitamina D y su asociación con la actividad de la enfermedad y calidad de vida de pacientes con artritis idiopática juvenil del Hospital Materno Infantil de ISSyM Toluca

Introduction: Juvenile Idiopathic Arthritis (JIA) is one of the most common chronic inflammatory diseases in pediatrics, it is known that vitamin D has a direct effect on bone and muscle health, in the last decade functions have been found on the immune response and inflammation. Studies on chronic inflammatory diseases in pediatric age including JIA. The objective of this work is to determine if there is an association between vitamin D levels and disease activity and quality of life of patients with JiA. Methods: Cohort, observational and analytical study where, prior approval by the Research Ethics Committee of the Hospital, vit D levels were determined and with the result CHAQ and JADAS71 tests were applied, in patients with JiA diagnosis, in the Pediatric Rheumatology consultation, information was collected from 28 patients diagnosed with JiA. Results: An average of vitamin D levels of 21.53 ng / mL (+/- 7.04 ng / mL) was found with a minimum of 6.9 ng / mL and a maximum of 38.9 ng / mL. The results obtained for the quality of life evaluated through the CHAQ Questionnaire reported that 30.8% were with zero limitation, 30.8% mild limitation, 26.9% moderate limitation and 11.5% severe limitation. When using the JADAS test to assess disease activity, 42.3% of patients have inactive disease; 7.7% with low activity, 26.9% with moderate activity and 23.1% with high activity. Conclusion: Although if it is found with an average in the population studied of vitamin D, in ranges of insufficiency, no relationship was found between these levels with the disease activity and quality of life of those of patients with JiA.

Assessment of condylar volume and ramus height in JIA patients with unilateral and bilateral TMJ involvement: retrospective case-control study.

The objectives of the study are to analyze volumetric differences of condylar volumes in patients with unilateral and bilateral JIA and to compare results with control condylar volumes. Forty-six CBCT images were analyzed for all patients affected by JIA, 37 females and 9 males (mean age 10.8 ± 4.2) with TMJ involvement (19 unilaterally, mean age 10.9 ± 4.5; 27 bilaterally, mean age 10.7 ± 4.5), and 25 CBCT of subjects without diagnosis of JIA were selected as controls (mean age 10.8 ± 4.2 years). In the case of unilateral JIA, condylar volumes and ramus lengths were compared with healthy condyle and with the compromised one. In the case of bilateral JIA, condyle volume and ramus lengths were compared with healthy one. The Shapiro-Wilk test was used to assess whether the data was normally distributed. Paired t test was applied to compare affected and non-affected condyle in the same patients (P < 0.05). Independent t test was used to evaluate whether the difference between the groups were comparable or significantly different (P < 0.05). For the unilateral JIA group, significant differences comparing affected and non-affected condyles were found. A statistically significant reduction of the volume of the head, neck, and ramus was found in the affected side (P < 0.01). For the bilateral JIA group, statistically significant differences have been found considering the condylar head and neck, the whole condylar volume, and the ramus length compared with the control group (P < 0.05). Subjects with unilateral JIA have condyles volumetrically smaller than those of the unaffected side and those found in healthy patients. A considerable decrease of the volume of all the anatomical structures considered in the patients with bilateral JIA was found compared with control group. The study presents the effects of JIA on different anatomical structures highlighting their dimensional changes, whose sequelae are irreversible if not diagnosed and treated early.

Proposal for a definition for response to treatment, inactive disease and damage for JIA associated uveitis based on the validation of a uveitis related JIA outcome measures from the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC)

BackgroundJIA-associated uveitis (JIAU) is a serious, sight-threatening disease with significant long-term complications and risk of blindness, even with improved contemporary treatments. The MIWGUC was set up in order to propose specific JIAU activity and response items and to validate their applicability for clinical outcome studies.MethodsThe group consists of 8 paediatric rheumatologists and 7 ophthalmologists. A consensus meeting took place on November 2015 in Barcelona (Spain) with the objective of validating the previously proposed measures. The validation process was based on the results of a prospective open, international, multi-centre, cohort study designed to validate the outcome measures proposed by the initial MIWGUC group meeting in 2012. The meeting used the same Delphi and nominal group technique as previously described in the first paper from the MIWGUC group (Arthritis Care Res 64:1365–72, 2012). Patients were included with a diagnosis of JIA, aged less than 18 years, and with active uveitis or an uveitis flare which required treatment with a disease-modifying anti-rheumatic drug. The proposed outcome measures for uveitis were collected by an ophthalmologist and for arthritis by a paediatric rheumatologist. Patient reported outcome measures were also measured.ResultsA total of 82 patients were enrolled into the validation cohort. Fifty four percent (n = 44) had persistent oligoarthritis followed by rheumatoid factor negative polyarthritis (n = 15, 18%). The mean uveitis disease duration was 3.3 years (SD 3.0). Bilateral eye involvement was reported in 65 (79.3%) patients.The main findings are that the most significant changes, from baseline to 6 months, are found in the AC activity measures of cells and flare. These measures correlate with the presence of pre-existing structural complications and this has implications for the reporting of trials using a single measure as a primary outcome. We also found that visual analogue scales of disease activity showed significant change when reported by the ophthalmologist, rheumatologist and families.The measures formed three relatively distinct groups. The first group of measures comprised uveitis activity, ocular damage and the ophthalmologists’ VAS. The second comprised patient reported outcomes including disruption to school attendance. The third group consisted of the rheumatologists’ VAS and the joint score.ConclusionsWe propose distinctive and clinically significant measures of disease activity, severity and damage for JIAU. This effort is the initial step for developing a comprehensive outcome measures for JIAU, which incorporates the perspectives of rheumatologists, ophthalmologists, patients and families.

P13 Real-life single-centre experience of tocilizumab-associated neutropenia in children with JIA

Abstract Background Tocilizumab, approved for treatment of children &gt;2years with SoJIA and polyarticular JIA (pJIA) is efficacious and well-tolerated. Transient neutropenia as a side-effect of treatment has been reported. Our aim was to perform a retrospective chart review of children treated with Tocilizumab and report our experience of tocilizumab-associated neutopenic episodes. Methods Retrospective review of patients receiving tocilizumab (January 2010-January 2019) performed and baseline demographics recorded. Patients with a diagnosis of extended-oligoarticular JIA (ExO-JIA), pJIA and SoJIA were reviewed in more detail to ascertain frequency of neutropenia and related-factors. For the purpose of analysis, ExO-JIA and pJIA were combined to create an ExO/pJIA group. Neutropenia was defined as neutrophils&lt;1.5 and severe neutropenia as &lt; 1.0. Statistical analysis was performed using the Mann-Whitney-U and Chi-Square tests. Results Sixty-eight children (60% female) attending the rheumatology department at GOSH are receiving tocilizumab. Of these, 53 (78%) have a diagnosis of ExO-JIA (n = 10), pJIA (n = 12) or SoJIA (n = 31). Median age at diagnosis was 4.4years (0.2–14.7). Disease duration pre-commencement of tocilizumab was 2.9years (0.1–11). Children with SoJIA were commenced on tocilizumab significantly (p &lt; 0.001) earlier in their disease course than children with ExO/pJIA (1.5 years, 0.1–7.4 versus 6.5 years, 0.4-11 respectively). However, there was no significant difference in number of alternative treatments received pre-tocilizumab between groups. Concurrent methotrexate administration was similar between groups. In contrast, concurrent use of steroids in SoJIA (27/31, 87%) was significantly more frequent (p &lt; 0.00001) than in ExO/pJIA (2/22, 9%). All children commenced on tocilizumab had a normal preceding white cell and neutrophil count. Median time to first episode of neutropenia was 0.5years (0.1-3.2). No significant pattern in time from commencing tocilizumab to developing first episode of neutropenia was observed (40% developed neutropenia after &gt;6months on tocilizumab). Recurrent neutropenia (i.e. &gt;2 episodes of neutropenia) was observed in 38% (20/53) and was significantly more common in children with SoJIA compared to ExO/pJIA (p &lt; 0.05). All children with ExO/pJIA experienced a total of 2-3 neutropenic episodes, significantly less (p &lt; 0.05) than the recurrence rate in children with SoJIA (median 7 episodes, 2-17). Severe neutropenia was observed in 65% (n = 13), occurring significantly (p &lt; 0.001) more frequently in SoJIA (n = 11, 85%) than ExO/pJIA. In those with recurrent neutropenia, no significant correlation was identified between risk of neutropenia and interval between doses, dose (mg/kg) received, concurrent administration of methotrexate, duration on tocilizumab or number of alternative treatments received pre-tocilizumab. Finally, 17 children (32%) taking tocilizumab had a documented infection, but no relationship between neutropenia and risk of infection was identified. Conclusion Tocilizumab-associated neutropenia is more frequently observed, more severe and more often recurrent in children with SoJIA. Neutropenia can occur at any stage in the treatment course, therefore clinicians should consider continuing blood-monitoring pre-tocilizumab long-term, with particular attention paid to those diagnosed with SoJIA. Conflicts of Interest The authors declare no conflicts of interest.

Prevalence and course of lower limb disease activity and walking disability over the first 5 years of juvenile idiopathic arthritis: results from the childhood arthritis prospective study.

ObjectiveThe aim was to investigate the time course of lower limb disease activity and walking disability in children with JIA over a 5-year course.MethodsThe Childhood Arthritis Prospective Study is a longitudinal study of children with a new JIA diagnosis. Childhood Arthritis Prospective Study data include demographics and core outcome variables at baseline, 6 months and yearly thereafter. Prevalence and transition rates from baseline to 5 years were obtained for active and limited joint counts at the hip, knee, ankle and foot joints; and walking disability, measured using the Childhood Health Assessment Questionnaire walking subscale. Missing data were accounted for using multiple imputation.ResultsA total of 1041 children (64% female), with a median age of 7.7 years at first visit, were included. Baseline knee and ankle synovitis prevalence was 71 and 34%, respectively, decreasing to 8–20 and 6–12%, respectively, after 1 year. Baseline hip and foot synovitis prevalence was <11%, decreasing to <5% after 6 months. At least mild walking disability was present in 52% at baseline, stabilizing at 25–30% after 1 year.ConclusionLower limb synovitis and walking disability are relatively common around the time of initial presentation in children and young people with JIA. Mild to moderate walking disability persisted in ∼25% of patients for the duration of the study, despite a significant reduction in the frequency of lower limb synovitis. This suggests that there is an unmet need for non-medical strategies designed to prevent and/or resolve persistent walking disability in JIA.

Time to diagnosis in juvenile idiopathic arthritis: a french perspective

BackgroundJuvenile idiopathic arthritis (JIA) is a rare disease that is not widely known by paediatricians and general practitioner (GP) leading to diagnostic error and delayed care provision. We aimed to analyse patient’s journey and time to diagnosis of JIA (delay from the first symptom to the diagnosis of JIA).We performed a retrospective cohort study of 67 patients diagnosed with JIA and seen in the paediatric rheumatology department of the Kremlin Bicêtre Hospital, between July 2002 and January 2015. Patients were selected for analysis in order to represent an equal distribution of five JIA subtypes: oligoarticular onset (21), polyarticular onset (13), enthesitis-related arthritis (17), and systemic onset (16).ResultsSixty-seven patients were finally analysed (42 girls). Before JIA diagnosis was made, patients had visited a mean of three physicians (3.6 ± 1.4 (mean; SD)). Emergency room physicians (52%) were the first patient’s referral before GP (42%). Paediatric rheumatologists were mostly seen as third referral (52% versus 3% at first referral). Reactive arthritis (34%) and septic arthritis (24%) represented both the most common initial diagnosis. JIA was suspected after an average median time delay of 3 months (0.26–81.2) except for 25 patients (37%): SJIA (n = 9), ERA (n = 7), OAJIA (3) and POJIA (n = 6) for whom diagnosis was suspected straightaway. In most cases (88%), JIA was established by paediatric rheumatologists.Surprisingly, the median total time to diagnosis in our population was rather short (3 months). Paediatric rheumatologist played a major role in making the diagnosis but the journey to reach them was long and complex with multiple referrals. These results reinforce the necessity of improving GP and emergency physician’s awareness and education on paediatric rheumatic diseases as the importance of a strong network in paediatric rheumatology to improve patient’s level of care.ConclusionWe highlighted the complex patient’s journey to diagnosis in children with JIA and made assumptions that reference center might reduce time to diagnosis although not statically proven. Further analysis with a larger number of patients might be needed to better investigate this probability.

Analysis of health care claims during the peri-transfer stage of transition from pediatric to adult care among juvenile idiopathic arthritis patients.

BackgroundTo investigate the utilization of health care services before and after transfer from pediatric to adult rheumatology care in clinical practice.MethodsUsing US commercial claims data from January 2005 through August 2012, we identified individuals with a JIA diagnosis code from a pediatric rheumatologist followed by any diagnosis code from an adult rheumatologist. Individuals had 6 months observable time before the last pediatric visit and 6 months after the first adult visit. Medication, emergency room, physical therapy use, and diagnosis codes were compared between the pediatric and adult interval using McNemar’s test. The proportion of days covered (PDC) of TNFi for the time between last pediatric and first adult visit was calculated.ResultsWe identified 58 individuals with JIA who transferred from pediatric to adult rheumatology care after the age of 14. The median age at the last pediatric rheumatology visit was 18.1 years old and the median transfer interval was 195 days. 29 % of patients received NSAIDs in the adult interval compared to 43 % in the pediatric interval (p = 0.06). In the pediatric interval, 71 % received a JRA and 0 % received an RA physician diagnosis code compared to 28 and 45 %, respectively, in the adult interval. The median PDC for patients receiving a TNFi was 0.75 during the transfer interval.ConclusionIndividuals with JIA who transferred to adult care were more likely receive a diagnosis of RA instead of JRA and were less likely to receive NSAIDs, but had no significant immediate changes to other medication use.

Impact of disease modifying anti‐rheumatic drugs (DMARDs) on the uveitis risk in juvenile idiopathic arthritis

PurposeTo analyze the influence of methotrexate (MTX), Tumour necrosis factor (TNF) inhibitors, and their combination on uveitis occurrence in juvenile idiopathic arthritis.MethodsData of a nation‐wide prospective rheumatological and ophthalmological database in Germany were evaluated for the years 2002 to 2013. Patient with JIA disease duration of less than 12 months at initial documentation and with a follow‐up of ≥ 2 years were included in this analysis. Discrete‐time survival analysis was performed to evaluate the impact of disease modifying anti‐rheumatic drugs (DMARDs) on the occurrence of uveitis.ResultsA total of 3,512 JIA patients fulfilled the inclusion criteria (mean age 8.3 ± 4.8 years, female 65.7%, ANA‐positive 53.2%, mean age at arthritis onset 7.8 ± 4.8 years, mean follow‐up time 3.6 ± 2.4 years). Uveitis manifested in 180 patients (5.1%) within one year after JIA onset, and in further 251 patients (7.1%) after the first year. MTX (HR 0.63, p = 0.022), TNF inhibitors (HR 0.56, p &lt; 0.001) and a combination of the two (HR 0.10, p &lt; 0.001) in the year before uveitis onset significantly reduced the risk for uveitis. MTX treatment within the first year after JIA diagnosis revealed the lowest uveitis risk (HR 0.29, p &lt; 0.001).ConclusionsDMARDs in JIA patients significantly reduce the risk for uveitis onset. A relevant protective effect was found especially for early MTX use within the first year of arthritis onset and for the combination of MTX with a TNF inhibitor.

Meta-analysis: diagnostic accuracy of anti-cyclic citrullinated peptide antibody for juvenile idiopathic arthritis.

Objective. To estimate the diagnostic accuracy of the anti-CCP test in JIA and to evaluate factors associated with higher accuracy. Methods. Two investigators performed an extensive search of the literature published between January 2000 and January 2014. The included articles were assessed by the Quality Assessment of Diagnostic Accuracy Studies tool. The meta-analysis was performed using a summary ROC (SROC) curve and a bivariate random-effect model to estimate sensitivity and specificity across studies. Results. The bivariate meta-analysis yielded a pooled sensitivity and specificity of 10% (95% confidence interval (CI): 6.0%–15.0%) and 99.0% (95% CI: 98.0%–100.0%). The area under the SROC curve was 0.96. Sensitivity estimates were highly heterogeneous, which was partially explained by the higher sensitivity in the rheumatoid factor-positive polyarthritis (RF+ PA) subtype (48.0%; 95% CI: 31.0%–65.0%) than in the other subtypes (17.0%; 95% CI: 14.0%–20.0%) and the higher sensitivity of the Inova assay (17.0%; 95% CI: 14.0%–20.%%) than the other assays (0.05%; 95% CI: 2.0%–11.0%). Conclusions. Anti-CCP antibody test has a high specificity for the diagnosis of JIA. The sensitivity of this test is low and varies across populations but is higher in RF+ PA than in other JIA subtypes.

Proinflammatory S100 proteins as clinical markers of juvenile idiopathic arthritis

Objectives. Proteins of the S100 family (S100A8/9, or calprotectin) are known to be useful diasease activity biomarkers of JIA. We hypothesize that titres calprotectin in serum and synovial fluid of children with acute and unconfirmed inflammatory joint symptoms will identify patients who will progress to JIA and our aim is to examine its prognostic value. Methods. A standardized diagnostic evaluation and analysis of serum S100A8/A9 was performed in 115 children with acute (symptoms up to 6 weeks) arthritis as well as in 59 healthy individuals. 47 arthritis patients underwent joint fluid aspiration and samples were collected at the time of presentation. Proteins in serum and synovial fluid were measured by ELISA and compared by the unpaired t test. Arthritis outcome (chronic or transient arthritis) was recorded at 1- and 2-year follow-up periods. JIA diagnosis, disease activity and remission were established according to ILAR and ACR recommendations. Results. The levels of serum and synovial fluid S100A8/A9 were significantly higher in patients with arthritis compared to the levels in healthy individuals (p

Uveitis outcome and complication

Anterior uveitis is a well-known threatening comorbid condition of JIA and affects around 10 to 20 % of the patients depending on JIA subtype. A large proportion of children with JIA develop uveitis in the first year of disease and 73 to 90% do so after four years of the arthritis onset. Uveitis can progress into the adulthood and usually occur as “white uveitis” which is not associated with symptoms like redness and pain as opposed to JIA related to the enthesitis subtype that is symptomatic. Factors associated to lower uveitis remission rate are: JIA diagnosis, findings of 1+ or more vitreous cells at presentation and initial visual acuity of 20/200 or worse. The Standardization of Uveitis Nomenclature (SUN) Group took the first step to define outcome measures for uveitis, but it was established just for adults. The Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC) proposed outcome measures for JIA associated uveitis incorporating the Standardization of Uveitis Nomenclature (SUN) criteria in 2011.

Reasons for stopping methotrexate treatment in patients with juvenile idiopathic arthritis

We included 98 patients (median age 15.4 ± 7.5 years) who had a definitive diagnosis of JIA and used MTX. Seventy-six (78%) patients were females. The JIA subtype most frequently observed was polyarticular. Routes of administration of MTX observed were subcutaneous (55%) and oral (13%), but many patients throughout the treatment used both adminis tration (32%) in order to improve adherence to treatment. After a mean follow-up time of 5 years, 51 (52%) of these patients discontinued treatment and 47 (48%) of them were still on treatment, but only 35 (74%) were adherent. Sixty-eight (69%) JIA patients reported the presence of sporadic episodes of adverse reactions during treatment. Episodes of adverse reactions related to MTX was mainly gastric intolerance (abdominal discomfort, nausea and vomiting). There were three cases of elevated transaminase levels and one case of neutropenia. The reasons for JIA patients discontinue treatment with MTX was presence of side effects (35%), interruption on their own decision (27%), treatment abandonment (12%) and other reasons such as pregnancy, chickenpox and lack of medication in the public hospitals (12%), improvement of the disease (8%), and inefficacy (6%). All patients who discontinued MTX on their own decision had additionally the presence of side effects. Conclusion

AB0904 Anti-Mullerian Hormone in A Cohort of Young Adult Women with Juvenile Idiopathic Arthritis

BackgroundJuvenile idiopathic arthritis (JIA) represents one of more common chronic disease of the childhood, affects young people before sixteen and persists into their reproductive years. These patients sometimes experience significant...

FRI0538 Juvenile Idiopathic Arthritis: from Childhood to Adult Life

BackgroundJuvenile Idiopathic Arthritis (JIA) is not a single disease but a term used to classify a group of inflammatory rheumatic diseases (Arthritis) of unknown etiology (Idiopathic) diagnosed before the patient...

A104: Aggressive Erosive Synovitis in Juvenile Idiopathic Arthritis

Background/Purpose:Plain radiographs (x‐rays) are useful in the evaluation of children with JIA. Bone erosions are one of the finding that can be seen on x‐rays. However, they are most often reported in the polyarticular RF+ subtype of JIA and are rare in most other subtypes.Methods:Herein 3 patients with JIA are presented whose diagnoses were questioned after plain x‐rays were obtained. MRI later revealed significant erosive disease in these patients.Results:Case 1: A 14 yo male presented with a monoarthritis of the left wrist. Initial x‐rays were normal. His work‐up was unremarkable. His inflammatory markers were normal. He was thus diagnosed with JIA–oligoarthritis. After 6 months in remission, after the use of an NSAID, he flared in the same joint. Repeat x‐rays showed a radiolucent area with associated sclerosis in the scaphoid of unclear etiology. An MRI was thus requested. It revealed synovitis associated with erosive disease. Post gadolinium injection, a geode in the scaphoid bone with marked synovial enhancement visible within this erosion was detected. He underwent a joint injection which put him into remission. Repeat x‐rays one year later, revealed unchanged findings likely corresponding to the non‐healed erosion.Case 2: An 11 yo HLA B27 + male diagnosed with ERA with polyarthritis of the lower limbs and sacroiliac joints was treated with methotrexate and an NSAID. Investigations ruled out IBD and the arthritis was felt to be well controlled despite persistently elevated inflammatory markers of unclear etiology. He presented with a severe flare in his knees and ankles. X‐rays revealed well‐defined lytic lesions in the anterior aspect of the proximal tibial metaphysis just below the growth plate bilaterally. Given the concern of an infiltrative process, an MRI was obtained. Post gadolinium injection, a large erosion of the tibial plateau with marked synovial enhancement within this erosion was seen, as well as a knee synovitis. Etanercept was started.Case 3: An 8 yo male presented with a monoarthritis of the right wrist evolving for five months after an initial minor trauma. X‐rays revealed advanced bone maturation of the right wrist as well as a sclerotic lesion of the hamate. Given the initial trauma, an MRI was obtained which demonstrated very extensive bone marrow edema, synovitis, tenosynovitis and a large erosion within the hamate. A diagnosis of JIA–oligoarthritis was made. He underwent a joint injection with good disease control.Conclusion:Large erosions are not typical for some JIA subtypes such as oligoarthritis and ERA. Findings on x‐rays led the treating physicians to question the underlying diagnosis however, MRI revealed very aggressive synovitis of the wrist and knee in these patients. MRI was useful to demonstrate the presence of synovial enhancement within the eroded bone and helped rule out another diagnosis. Awareness of this type of aggressive arthritis is needed so that patients are not over‐investigated and prompt treatment can be offered.