JIA Children Research Articles

Infections in children with Juvenile Idiopathic Arthritis, more frequent than in healthy children? Prospective multi-center observational study.

Abstract Background Children with JIA may have a higher risk of infection. Our objectives are to describe and compare infection rates in JIA patients vs non-JIA counterparts. Methods A prospective, multicenter observational study was performed in Spain from 01/2017 to 06/2019. JIA patients from 7 participating hospitals and children without JIA (siblings of JIA patients, and non-JIA children from primary health centers) were followed up with quarterly questionnaires recording infection episodes. Tuberculosis, Herpes Zoster, and infections requiring hospital admission were considered severe infections. Rate of infection (episodes/patient/year) was compared using a Generalized Estimating Equations Model. Results 371 children (181 JIA and 190 non-JIA) were included. Median age was 8.8years (IQR 5.5 – 11.3); 75% of JIA patients received immunosuppressive treatment (24% methotrexate, 22% biologic, 26% both). 667 infections were recorded, 15(2.2%) considered severe. Infection rate was 1.31 (95%CI 1.1 – 1.5) in JIA and 1.12 (95%CI 0.9 – 1.3) in non-JIA participants (p0.19). Age <4 years increased 2.5 times infection rate (2.51 vs. 0.98, p<0.001) in both groups. The most frequent infection sites were upper respiratory (62.6% vs. 74.5%) and gastrointestinal (18.8%vs 11.4%). There were no differences in severe infections (2.5%vs 2%, p0.65) among groups. In JIA children, younger age and higher disease activity (JADAS71) were associated with a higher infection rate. Conclusions Despite being a potential risk group, we found no differences in infection rate, severity, type and antibiotic treatment between patients with and without JIA. Most infections were mild. Age below 4 years increased infection risk in both groups. Higher disease activity was associated with a higher infection rate in JIA patients.

P030 Impact of methotrexate on growth in children with Juvenile Idiopathic Arthritis

Abstract Background Juvenile idiopathic arthritis (JIA) is a heterogeneous group of chronic inflammatory disease which could be responsible for functional impairment and severe growth disturbance. Conventional disease-modifying antirheumatic drugs, such as methotrexate (MTX), may improve growth velocity especially by regulating systemic inflammation. The objective of this study was to evaluate the effect of MTX on growth parameters in pre-pubertal children with JIA and to determine the factors affecting the growth velocity. Methods We assessed height and changes in the height standard deviation score (SDS) at disease onset, at the onset of MTX and at the last follow-up visit in a cross-sectional study of JIA children. All patients were pre-pubertal when MTX began and were followed for at least 6 months afterward. We compared growth parameters (height, growth rate, weight and body mass index (BMI)) in responders and non-responders to MTX. The growth rate was defined as the number of millimeters of height acquired during 1 year. Associations between changes in the height SDS and discrete variables were evaluated using χ2 or Fisher’s exact tests. The significance level was set at 0.05. Results We enrolled 36 pre-pubertal children with JIA (34 boys and 12 girls) who had been treated with MTX orally. Median patient age was 6.2 years [4–13] at the onset of MTX and 8.4 years [6.1–14.9] at the latest follow-up. The median disease duration was 2.7 years [2.5–5.3]. Twenty-one patients (58.3%) had oligoarticular JIA, 2 patients (5.5%) had systemic JIA, 10 (27.7%) had polyarticular JIA and 3 (8.3%) had enthesitis-related arthritis. Nineteen children (52.7%) had received corticosteroids during an average period of 1.7 years [0.6–3] with a mean of 10 mg/day of prednisone or equivalent. The median duration of MTX at the latest follow-up was 3.1 years [0.62–5.5] with a mean MTX dose of 10 mg/m2/week [10–15]. Twenty-eight patients responded to MTX treatment and 8 did not. There were no significant differences between the responders and non-responders for age at treatment initiation, disease duration and mean MTX dose. At MTX onset, no significant differences between the two groups in terms of height (P = 0.52), growth rate (P = 0.08), weight (P = 0.74) and BMI (P = 0.9) were found. One year after MTX therapy, mean height (0.2 vs -1.1; P = 0.03), mean growth rate (0.5 vs –2.9 SDS; P = 0.01), mean weight (0.4 vs -2.3 SDS; P = 0.01) and mean BMI (0.6 vs -1.9; P = 0.04) were significantly higher in the responder group than in non responders, respectively. At the latest follow-up, this increase was significantly maintained for growth rate (P = 0.001) and height (P = 0.002) in the responder group. In the multivariate analysis, patients who required >10 mg/m2/week of MTX, systemic JIA and patients with reliance on steroids had a significantly lower growth velocity after the onset of MTX (P < 001, P = 0.02, P = 0.02 respectively). Conclusion In our study, the increase in growth parameters in pre-pubertal children with JIA was associated with a better control of the disease activity under MTX therapy.

OBSIDIAN - real-world evidence of originator to biosimilar drug switch in juvenile idiopathic arthritis.

Limited data about use of biosimilars (BIOs) are available in children with JIA. This study therefore aimed to evaluate long-term efficacy and safety of switching from etanercept (ETA) and adalimumab (ADA) originators to their biosimilars (BIOs), in children with JIA, in a real-world setting. This is a retro-prospective non-interventional multicentre Italian comparative cohort study. Medical charts of JIA children treated with biosimilars of ETA or ADA were included. Efficacy and safety of TNF-inhibitors therapy was evaluated at last follow-up during originator and at 3, 6 and 12 months following the switch to biosimilar. A total of 59 children (42 female, median age at onset 88 months) were treated with biosimilar of ETA (21) and ADA (38). Forty-five switched from the originator to the BIO (17 ETA, 28 ADA). At time of switch, 12/17 patients on ETA and 18/28 on ADA were in remission. No significant difference has been found at 3, 6 and 12 months after the switch. Ten patients discontinued biosimilars due to disease remission (4 ETA, 3 ADA), family willing (1 ETA), occurrence of burning at injection site (1 ETA) and persistent activity (1 ADA). No statistically significant difference was observed between originator and BIOs, nor between originator and BIOs, and between ADA and ETA in time to disease remission achievement, time to relapse and number of patients who experienced adverse event (AE). Our real-life results seem to confirm the efficacy and safety profile of switching from originator of ADA and ETA to their respective BIOs, also in paediatric patients with JIA.

Paediatric non-infectious uveitis in Cape Town, South Africa: a retrospective review of disease characteristics and outcomes on immunomodulating treatment

BackgroundNon-infectious uveitis is a well-reported cause of blindness in more developed countries, however data from sub-Saharan Africa is lacking. Here we aim to describe the diseases associated with paediatric non-infectious uveitis and the effect of currently available treatment in this setting.MethodsA retrospective observational analysis of children with non-infectious uveitis from January 2010 to December 2017, attending the tertiary paediatric rheumatology and ophthalmology referral units in Cape Town was conducted. Statistical analysis utilising STATA13 software was performed with p < 0.05 considered significant.ResultsTwenty-nine children were identified: median age at first visit of 74 months (IQR 49–86 months), female to male ratio of 0.9:1, predominantly of mixed ancestry (72.4%). Juvenile idiopathic arthritis associated uveitis (JIAU) (48.3%), idiopathic uveitis (41.4%), sarcoidosis (6.9%) and Behcet’s disease (3.5%) were diagnosed. Chronic anterior uveitis (72.4%) was the most frequent finding. Fifty-five percent had complications at presentation and all children with idiopathic uveitis presented with cataracts.Only 6.5% of the JIA cohort had JIAU. All JIA children had chronic anterior uveitis. There were no differences between JIA children with uveitis and those without uveitis, for sex (p = 0.68) and race (p = 0.58). Significantly, children with uveitis presented at an overall younger age (p = 0.008), had oligo-articular JIA (p = 0.01) and were antinuclear antibody positive (p < 0.001).Children with idiopathic uveitis were predominantly male (66.6%) with chronic anterior uveitis (41.7%).Nineteen children (65.5%) in the cohort had inactive disease on treatment at 12 months from diagnosis, which included 10 on topical corticosteroid therapy. At the last clinical visit 17 (58.6%) on standard initial therapy, 8 (27.6%) on tumour necrosis factor inhibitors and 2 on additional DMARDs were in remission. Five of these children still required topical corticosteroids. Surgery was performed in 41.4%, primarily in the idiopathic group. Visual acuity improved or was maintained on treatment.ConclusionCurrent practice seems to detect children with potentially sight-threatening disease but the high rate of complications and the low percentage of children with JIAU raises concerns of delayed healthcare intervention. Tumour necrosis factor inhibitors have improved outcomes in refractory cases in this cohort, however further studies are needed.

The quality of life in Chinese juvenile idiopathic arthritis patients: psychometric properties of the pediatric quality of life inventor generic core scales and rheumatology module

BackgroundJuvenile idiopathic arthritis (JIA) may seriously affects patients’ quality of life (QoL), but it was rarely focused and studied in China, so we explore JIA children’s QoL using Chinese version of the PedsQL4.0 Generic Core and PedsQL3.0 Rheumatology Module scale, and analyzed the psychometric properties of these two Scales among Chinese JIA children.MethodsWe recruited 180 JIA patients from Children's Hospital Affiliated to Capital Institute of Pediatrics and Hebei Yanda Hospital from July 2018 to August 2019. The questionnaires include information related on JIA, PedsQL4.0 generic core and PedsQL3.0 Rheumatology Module scales. According to the disease type, onset age of and course of JIA, we divided them into different groups, then compared the QoL status among different groups. Moreover, we analyzed the reliability and validity of these two scales in these 180 JIA children.ResultsThe mean score of PedsQL4.0 generic core scale on these 180 patients was 82.85 ± 14.82, for these in active period was 72.05 ± 15.29, in remission period was 89.77 ± 9.23; the QoL score of systemic, polyarticular and oligoarticular JIA patients were 77.05 ± 19.11, 84.33 ± 12.46 and 87.12 ± 10.23. The mean score of PedsQL3.0 Rheumatology Module scale on 180 patients was 91.22 ± 9.45, for these in active period was 84.70 ± 11.37, in remission period was 95.43 ± 4.48; the QoL score of systemic, polyarticular and oligoarticular JIA patients were 89.41 ± 11.54, 89.38 ± 10.08 and 93.71 ± 6.92. In the PedsQL 4.0 Generic Core scale, the α coefficients of total scale and almost every dimension are all greater than 0.8 except for the school activity dimension of 0.589; the correlation coefficients of 22 items’ scores (total 23 items) with the scores of dimensions they belong to are greater than 0.5 (maximum value is 0.864), and the other one is 0.406. In PedsQL3.0 Rheumatology Module scale, except for the treatment and worry dimensions of 0.652 and 0.635, the α coefficients of other dimensions and the total scale are all greater than 0.7; the correlation coefficients of all items’ score were greater than 0.5 (the maximum is 0.933, the minimum is 0.515).ConclusionsThe QoL of Chinese JIA children is worse than their healthy peers, these in active period and diagnosed as systemic type were undergoing worst quality of life. The reliability and validity of PedsQL 4.0 Generic Core and PedsQL3.0 Rheumatology Module scale in Chinese JIA children are satisfactory, and can be used in clinical and scientific researches.

Shared decision-making aid for juvenile idiopathic arthritis: moving from informative patient education to interactive critical thinking.

To develop and evaluate an illustrated, stand-alone, interactive evidence-based shared decision making (SDM) aid for JIA children; its ability to produce positive perceived involvement of JIA patients in their own management and its impact on their adherence to therapy, school absenteeism and treatment outcomes. The SDM aid was developed to offer information about the disease, risks and benefits of treatment. A multidisciplinary team defined SDM criteria based on international standards (IPDAS). Eight categories emerged as highly important for SDM. Each category was supported by simple illustrations in an interactive style. At the end of each category, the child is asked to make a decision in view of the information given. Ninety-four JIA children were provided with the tool, in a randomised controlled study, in comparison to a control group of 95 JIA patients treated according to standard protocols. A total of 97.5% of the study children reported comprehensibility of more than 90%. The patients' adherence to therapy was significantly (p < 0.01) higher in the SDM group, whereas stopping DMARDs for intolerability was significantly higher in the control group at 12months of treatment. There was a significant improvement in the patient-reported outcomes in the SDM group, and absence from school was significantly higher in the control group (p < 0.01). The developed SDM aid offered the children evidence-based information about the pros and cons of treatment options and improved their understanding of the disease and their ability to make an informed decision that is reflected on their adherence to therapy and better treatment outcomes. Key Points • This work represents the second generation of shared decision-making tools. • The developed tool adopts an interactive style and enhances critical thinking, giving the patients the facility of making their own decision regarding their management. • The work gives an example of core domain set of outcomes which can be used for shared decision-making interventions.

Platelet microparticles level in juvenile idiopathic arthritis: A pediatric population-based cross-sectional study in a tertiary care center

Background: Platelet-derived microparticles (PMPs) are small vesicles that are released from the plasma membrane upon platelet activation, which are then involved in haemostasis, vascular health, and have recently been shown to be intimately involved in immune responses. Aims and Objectives: We aimed to evaluate the level of plasma Platelet-derived micro particles in children with JIA and to assess the relationship between PMP levels and disease activity in JIA. Materials and Methods: Children with JIA who fulfilled the International League of Associations for Rheumatology (ILAR) classification criteria for juvenile idiopathic arthritis were included. They were categorised into active disease group and inactive disease as assessed by Wallace criteria. Samples were run in Navios flow cytometer (Beckman Coulter). Platelet microparticles were identified by MPs positive for both Annexin V and CD41 antibodies. Results: Out of 26 children with JIA, 12 had active disease group and 14 had inactive disease as assessed by Wallace criteria. Mean PMP level was 83507 cells/μl and 34904 cells/μl in active and inactive disease respectively (P = 0.06). There was no significant correlation between PMP and CRP levels (P = 0.75 and r = 0 .102) or PMP and ESR levels (P = 0.56 and r = -0.186) in JIA children with active disease. Conclusion: PMP levels were significantly elevated in disease activity of JIA and could represent a new biomarker reflecting the state of cell activation in JIA. PMP role in the inflammatory processes needs to be further elucidated.

Body composition and phase angle as an indicator of nutritional status in children with juvenile idiopathic arthritis

BackgroundJuvenile idiopathic arthritis (JIA) is the most common chronic, systemic autoimmune connective tissue disease diagnosed in children and adolescents. An important aspect of monitoring of children with JIA is a precise assessment of the nutritional status to identify children and adolescents at risk of malnutrition. The aim of the study was to assess the body composition and phase angle in children diagnosed with JIA in comparison to age and sex matched healthy children since there are scarce reports in paediatric patients.MethodsA total of 46 children and adolescents aged 4–18 years, with JIA were included in the cross-sectional study. Controls were selected from the group of healthy children and adolescents. Children with diagnosed JIA and healthy children were strictly matched for age and gender. In both groups BIA with phase angle calculation was performed.ResultsPhase angle score was significantly lower in the study group compared to control group (5.45 ± 0.64 vs. 5.85 ± 0.80, p = 0.010). Also lower percentage of body cell mass (50.63 ± 3.46 vs. 52.70 ± 4.06, p = 0.010) and muscle mass (46.02 ± 6.32 vs. 49.53 ± 6.67, p = 0.005) were revealed. In the analysis of subtypes of JIA we found significant differences between children and adolescents with polyarthritis compared to control group, while no significant differences were found between patients with oligoarthritis and control group.ConclusionsThe obtained results indicate a higher risk of malnutrition in children and adolescents with JIA compared to healthy peers, predominantly in patients with polyarthritis.

Share Decision Making Aid for Juvenile Idiopathic Arthritis: Moving from Informative Patient Education to Interactive Critical Thinking

Background: Shared decision making (SDM) is an emerging trend in paediatrics. Currently available SDM interventions are mere information on the disease or medication and often fail to engage the children in the medical decision process. It has been suggested that future decision aids should endorse better children engagement. Objectives: To develop, and evaluate an illustrated and interactive evidence-based SDM aid for JIA children, 2. 2.Positive perceived involvement of JIA patients in their own management that would be reflected on treatment outcomes. Methods: A multidisciplinary team defined SDM criteria based on the international standards (IPDAS). Eight categories emerged as highly important for SDM. Each category was supported by simple illustrations in an interactive style. At the end of each category the child is asked to make a decision in view of the information given. 94 JIA children evaluated the tool, in a randomized controlled study, in comparison to control group of 95 JIA patients treated according to standard protocols. Results: The SDM aid was developed to offer information about the disease, risks and benefits of treatment.97.5% of the children included reported comprehensibility of > 90/100. The patients' adherence to therapy was significantly (p<0.01) higher in the SDM group, whereas stopping DMARDs for intolerability, was significantly higher in the control group at 12-months of treatment. There was significant improvement in the patient reported outcomes in the SDM group, whilst absence from school was significantly higher in the control group (p< 0.01) Conclusions: This Illustrated-interactive SDM aid for JIA children was found to be a simple, user-friendly tool which can be implemented in standard practice. The developed SDM offered the children evidence-based information about the pros and cons of treatment options, improved their understanding of the disease, communication with their treating clinician and their ability to make an informed decision. Funding Statement: Authors declare none. Declaration of Interests: Nothing to declare. Ethics Approval Statement: Local ethical and methodological protocols for approval of the study were followed. All patients or their parents/ caregivers who participated in the study signed an informed consent according to the declaration of Helsinki.

Population-based cohort study on the risk of malignancy in East Asian children with juvenile idiopathic arthritis.

BackgroundTo investigate the association and magnitude of risk between JIA, its associated treatment and cancer development in Taiwanese children.MethodsNationwide population-based 1:4 age- and gender-matched retrospective cohort study was designed using the National Health Insurance Research Database of Taiwan. A cohort of 2,892 children <16 years old with JIA was formed as well as a non-JIA cohort of 11,568 in year 2003 to 2005. They were followed up till a diagnosis of malignancy or up to 8 years until 2010. Relative risk (RR), incidence rate ratio (IRR), and adjusted hazard ratio (aHR) of developing malignancy were calculated.ResultsThe female to male ratio was 0.79:1. There were 3 cases of incident cancer in the “MTX use, biologics-naïve” group, only 1 in the anti-TNF biologics-containing group and 29 in the “both MTX- and biologics-naïve” group, in comparison, there were 50 cases of cancer in the non-JIA comparator group. During a 16114.16 patient-years follow-up, the RR and IRR for developing a malignancy in both methotrexate- and anti-tumor necrosis factor (TNF) biologics-naïve JIA children were 2.75 (95% confidence interval, 1.75 – 4.32) and 3.21 (2.01 – 5.05), respectively. For leukemia, the IRR was 7.38 (2.50 – 22.75); lymphoma, 8.30 (1.23 – 69.79); and soft tissue sarcoma, 11.07 (0.84 – 326.4). The IRR of other cancers was 2.08 (1.11 – 3.71). The aHR on cancer risk was 3.14 (1.98 – 4.98) in methotrexate- and biologics-naïve group. There were no statistically significant increased risk in JIA patients treated with methotrexate and/or anti-TNF biologics.ConclusionsCompared with children without JIA, children with JIA have 3-fold increase of risk on malignancy in East Asia. Seemingly neither methotrexate nor anti-TNF biologics increases the risk further.

The role of sleep in Juvenile idiopathic arthritis patients and their caregivers.

Juvenile idiopathic arthritis is a chronic disease that may lead to various consequences for patients and caregivers, especially in relation to sleep quality. Sleep is an essential process for homeostasis of the organism. In general, caregivers of children with JIA are more susceptible to these sleep disorders and a lower quality of life. This impairment in sleep can potentially affect the health of caregiver. For this reason, it is very important to continue to evaluate the quality of sleep in caregivers and how to support these JIA children’s caregivers more effectively.

THU0170 Efficacy and Safety of Infliximab in Juvenile Idiopathic Arthritis and Juvenile Ankylosing Spondylitis

ObjectivesThis study will provide evidence-based medical evidence in the application of infliximab for pediatric rheumatology.MethodsThe 45 cases of this study were allocated to treatment group and control group. The treatment...

Dento-alveolar Changes And Need Of Orthodontic Treatment In JIA Patients Followed From Childhood To Adulthood

Objectives: To determine the occlusal traits and the objective orthodontic treatment in a group of JIA children, with and without radiographic TMJ involvement, and compare with the objective orthodontic treatment in adulthood. Methods: Fifty-three JIA children (mean age 11.2 years) were studied; 30 had radiographic TMJ involvement (TMJ+ group) and 23 had no involvement (TMJ- group). Measurements were performed on dental study casts using the Need of Orthodontic Treatment Index, with 106 gender and age matched controls. Of the 53 JIA patients, 41 had available dental study casts in adulthood that were compared to childhood study casts. Results: In childhood, TMJ+ subjects had an overjet ≥9 mm more frequently than controls (p<0.037). More TMJ+ subjects were categorized with “great treatment need” than controls, 23.5% versus 9.5% (p<0.042), respectively. As adults, 34% had a history of orthodontic treatment; 7.3% with orthognathic surgery. Only 5% could be categorized with “great treatment need” compared to 24% in childhood (p = 0.012). Conclusions: JIA children with TMJ involvement were more susceptible to a large overjet, leading to great treatment need more frequently than controls. In adulthood the occlusion had improved due to orthodontic treatment and orthognathic surgery and probably, due to craniofacial growth and development. In growing JIA patients it is important to monitor the occlusal development.

THU0300 Reduced articular cartilage thickness in joints without a history of active arthritis in children with juvenile idiopathic arthritis

BackgroundThe functional disability experienced in juvenile idiopathic arthritis is primarily caused by degeneration of the osteocartilaginous structures due to the inflammatory process in the synovium. It is therefore essential for...

FRI0341 Temporomandibular joints (TMJ) involvement in juvenile idiopathic arthritis (JIA): Longitudinal evaluation after orthopaedic treatment

BackgroundTMJ involvement has been largely observed in all subsets of patients with JIA. The reported prevalence of detectable X-ray changes varies from 30% to 63%. From 50 to 80% of...

Correlation between clinical indices and findings of joint ultrasonography in juvenile idiopathic arthritis with adalimumab therapy

Methods 4 polyarticular JIA (p-JIA) children diagnosed as JIA according to ILAR criteria were enrolled. All were female. The median age was 12 years (range; 10 to 17 years), and median disease duration was 5.6 years (range; 2.5 to 7.5 years). Two patients were administered prednisolone and methotolexate, One prednisolone only, and one drug free, as preceeding therapy. ADA was administered biweekly at a dose of 40mg per body. Demographic data were collected. Joint US examination was demonstrated at 0 (prior to administration of ADA), 4, and 24 weeks.

Genetic polymorphism of COL1A1 gene and bone mineralisation in juvenile idiopathic arthritis

Methods We included 196 JIA children, 81 boys and 115 girls. Bone mineralization parameters were detected by dual-energy X-ray absorptiometry of lumbar spine L1-L4. Bone biochemical markers were osteocalcine, C-terminal telopeptides (CTT), parathyroid hormone (PTH), Ca, Ca, P, total alkaline phosphatase (TAP) activity. We have detected Sp1 (rs1800012) and -1997G/T (rs1107946) polymorphisms in type I alpha-1 chain collagen gene (COL1A1).

Performance of IFN-Gamma release assays for detection of tuberculosis infection in juvenile I JIA children in biological therapy

Severe TB has recently been described in association with TNF-α blocker therapy. Furthermore conventional tuberculin skin test (TST) may give false-negative results in patients receiving biological therapy. New in vitro test, based on the detection of IFN-γ release by T-cell stimulated with M. tuberculosis specific antigens (IGRA), are available for LTBI diagnosis, promising to be more sensitive and specific than TST. Nevertheless no sufficient evidence on IGRA performance are available in children. The performance of two commercially available IGRAs, ELISPOT-TB (TS-TB) and Quantiferon-TB gold in-Tube (QFT-GIT), were evaluated in comparison with TST in children with rheumatic diseases. In children with positive result clinical and radiological evidence of TB was sought. Nineteen children with JIA were enrolled since November 2006 (median age 13.5 years; range 2–18). Seventeen children were on long term etanercept therapy plus methotrexate (15 mg/m2/week), 1 on infliximab and 1 received adalimubab plus prednisone (0,2 mg/Kg/die). Five children (26%) showed positive TS-TB, QFT-GIT and TST were negative in all children. Both QFT-GIT and TS-TB showed a high percentage of indeterminate results (21% and 16% respectively). None of the 5 patients positive at TS-TB developed active disease, as judged by clinical and radiological findings, at least in a median follow-up period of 16 months (range 9–17 months). Our data suggest that high rate of positive results by TS-TB is expression of non-specific immune reactivity in rheumatic children.

Current perspectives of autologous stem cell transplantation for severe juvenile idiopathic arthritis

The majority of children with Juvenile Idiopathic Arthritis can nowadays be treated adequately. However despite the use of combinations of antirheumatic drugs, corticosteroids and the newer so called biologicals (blocking the TNF, Interleukin 1 or Interleukin 6 pathways) a proportion of children with arthritis remain resistant also to these therapies and suffer from a very severe, debilitating and potentially fatal disease. For such children autologous stem cell transplantation (ASCT) is successfully performed since 1997. Here we describe the long term outcome of the initial cohort of children with resistant Juvenile Idiopathic arthritis, treated with ASCT. The initial cohort of children was treated with a conditioning regimen containing Cyclophosphamide, anti thymocyte globulins and low dose Total Body irradiation. Overall favourable responses were seen, with a drug free remission rate of 50–55 %. In the more recent years late relapses were noted with lower percentages for drug free long term outcome. Special emphasis is given on 2 cases showing very late relapses, occurring after 7 and 9 years. The observed relapses are often less severe compared to the situation before SCT and can be treated successfully with conventional drugs in the majority of cases. More recently, ASCT was performed in 4 JIA children with a fludarabin containing regimen in stead of low dose TBI. With a 4 to 5 year follow up, these 4 patients are all in drug free full remission. Allogeneic transplant with an HLA matched family donor was reported in 2 JIA cases. Follow up of 1 and 3 year is sofar show clinical disease remission and tapering of medition. In conclusion, given the favourable long term outcome, SCT remains a valuable treatment option for children with drug resistant JIA.