Explore the vast range of titles available.

ObjectivesThis study on juvenile SLE patients aimed to evaluate retrospectively the impact of a tertiary center’s management policy of the disease severity on its long-term progression and cumulative damage development as well as provision of quality-driven medical care (QmC).MethodsDisease activity was assessed by the Physician Global Assessment and SLEDAI-2K, flares by SELENA/SLEDAI, and damage by the pediatric SLICC/DI at diagnosis, 6 months post-diagnosis, and annually thereafter. At the same time, QmC was evaluated by relevant indices and quality of life was captured by the Greek version of the General Health Questionnaire only at the last visit.ResultsA total of 35 patients (25/35 females) aged at diagnosis 5.5–15.16 years (median 11.83) with a median lag time to diagnosis 1.8 months had a follow-up of 5 (35/35) and 10 years (13/35), respectively. The predominant baseline manifestations were consistent with those previously reported. Out of 35 patients, 24 (68.5%) were clinically inactive at year 5, and 5/13 (38%) at year 10. All patients received immunosuppressives and 7/35 biologics in addition. At the end of their follow-up, damage was found in 9/35 patients, but none of them had a neuropsychiatric disorder. Over the study, 28/35 patients were compliant with the QmC recommendations.ConclusionsAn early diagnosis combined with a longitudinal quantitative assessment of the disease activity and severity contributes to the continuous evaluation of the disease state. They are the key determinants for the selection of an early, targeted, and personalized management; they restrict the cumulative damage development and contribute to an optimal outcome.Key Points• Juvenile SLE has a heavier introductory profile than in adults and an unpredictable trajectory.• The application of contemporary metric tools for assessing the disease state leads to an objective assessment and regimen selection.• An early diagnosis combined with longitudinal quantitative assessment is a key determinant for an optimal management and a minimal damage development.

To describe the profile of Enthesitis Related Arthritis’ (ERA) patients, in the era of biologic DMARDs (bDMARDs). This retrospective cohort study included patients with ERA monitored on a 3-month schedule for at least 1 year. Their metric assessment included the disease status and damage by applying the contemporary tools clinical-Juvenile Arthritis Disease Activity Score (c-JADAS), Juvenile Spondyloarthritis Disease Activity Index (JSpADA), clinical remission (CR) on/off medication and Juvenile Arthritis Damage Index (JADI). 43 patients (males 26) were enrolled, with a mean disease onset of 10.75 years. Median lag time from diagnosis to bDMARDs was 8.5 months. Patients with sacroiliitis received earlier bDMARDs (hazard ratio, HR 3.26). 36/43 patients achieved CR on medication (median time 11 months), which was correlated with compliance (HR: 3.62). The percentage of CR in patients with or without sacroiliitis was 35% and 63% respectively (p = 0.02). Twenty patients (47%) experienced a flare following CR (75%). The median flare-free survival following CR on/off medication was 42 and 34 months, respectively. At the last evaluation, both median baseline cJADAS and JSpADA dropped to 0, 13/43 patients had a persistent disease activity, while 17/43 and 13/43 patients were in CR on/off medication, respectively. The median patient percentage of CR was 54% and no patient had a JADI > 0. Increased lag time to bDMARDs was associated with increased CR (Odds ratio: 1.48). Early administration of bDMARDs and compliance improved long-term outcome of ERA. Sacroiliitis was a negative prognostic factor with an increased need for bDMARDs and diminished rates of CR.

Objectives To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch–Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria. Methods The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES - supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis ≤18 years. Step 2: blinded classification by consensus panel of a subgroup of 280 cases (128 difficult cases, 152 randomly selected) enabling expert diagnostic verification. Step 3: Ankara 2008 Consensus Conference and statistical evaluation (sensitivity, specificity, area under the curve, κ-agreement) using as ‘gold standard’ the final consensus classification or original treating physician diagnosis. Results A total of 1183/1398 (85%) samples collected were available for analysis: 827 HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms, laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides. A representative subgroup of 280 patients was blinded to the treating physician diagnosis and classified by a consensus panel, with a κ-agreement of 0.96 for HSP (95% CI 0.84 to 1), 0.88 for c-WG (95% CI 0.76 to 0.99), 0.84 for c-TA (95% CI 0.73 to 0.96) and 0.73 for c-PAN (95% CI 0.62 to 0.84), with an overall κ of 0.79 (95% CI 0.73 to 0.84). Conclusion EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.

Progress in the pediatric Auto-inflammatory Diseases (AIDs) has led to improved long-term outcome and the increased pool of pediatric patients who require lifelong monitoring. Implementation of a successful stepwise transition in patients with AIDs denotes the presence of a structured flexible and individualized policy that ensues the stepwise move from family-based pediatric care to adult patient one. This process aims to equip the young adult with self-management skills and the ability to enjoy life even under the burden of a chronic disease. Transition, thus, is a continuously evolutionary process that assists adolescents and young adults with an AID to move into a future that their predecessors with similar diseases never needed to experience. This review, using the myth of Daedalus and Icarus as a scaffold, presents the contemporary profile of the adolescent patient, comments on the evidence derived from Transition recommendations, and emphasizes the need of periodic quantitative assessments to assess the efficacy of the Transition plan. Upon the completion of the transfer to the Adult Center, monitoring of the patient's active participation will support his/her engagement in the new setting.

To assess longitudinally the course and outcome of juvenile idiopathic arthritis (JIA) in patients diagnosed and followed-up exclusively in the biologic era; also, to define possible predictors of the disease progression and need for early implementation of biologicals. Prospective and retrospective, monocentric cohort study of 120 JIA patients, diagnosed between 2001 and 2010, and followed-up for ≥ 4 years (median 8.04). Disease activity, cumulative articular/extra-articular damage and quality of life were evaluated by the assessment tools Juvenile Arthritis Disease Activity Score (JADAS71), Juvenile Arthritis Damage Index (JADI) and Childhood Health Assessment Questionnaire (CHAQ), respectively. Moreover, potential predictors of the disease progression and their relation to biologic therapy were investigated. High JADAS71 score (> 9) at diagnosis was indicative of progression to polyarticular course and the need for early introduction of biologic treatment. Other independent predictors of progression to polyarthritis, were: involvement of upper limb, hip and ankle within 6 months following JIA diagnosis and percentage of cumulative time with active disease > 35% within the first year. At the end of the study, both the median JADAS71 score and the Disability Index were significantly lower than the initial (p < 0.001) and remission off medication was achieved in 25% of the patients. Articular and extra-articular (only ocular) cumulative damage was demonstrated only in 5 and 7.5% of patients, respectively. Physical functional ability was found normal/mildly restricted in 93.3% and moderately restricted in 6.7% of the patients. We believe that these findings, fit in with a picture of JIA course and outcome under current conditions of objective “disease status” evaluation and of tightly controlled follow-up. Predictors emerged from our study could contribute to the identification of patients who will need early implementation of biologic treatment.

To investigate the immunoregulatory role of the Programmed-cell-Death-protein-1 (PD1) pathway, an inhibitory immune checkpoint, in Juvenile Idiopathic Arthritis (JIA). The PD1 expression on CD4+ and CD8+ T-cells was determined by flow cytometry and the PD1 soluble form (sPD1) levels by ELISA, in peripheral blood (PB)/serum and synovial fluid (SF) samples of JIA patients and healthy controls (HCs). We searched for any association in-between the biomarkers and with JIA activity. 101 Caucasian patients (69 female), aged 12 (8-15) years, and 20 HCs participated in this study. The PB PD1 expression on T-cells was higher in: a. JIA patients HCs (CD4: 1.24% 0.32%, p=0.007, CD8: 1.6% 0.4%, p=0.002). b. active inactive JIA (CD4: 1.44% 0.87%, p=0.072, CD8: 2.1% 0.93%, p=0.005). The SF PD1 expression on T-cells correlated strongly and positively with the disease activity (CD4: ρ=0.55, p=0.022, CD8: ρ=0.555, p=0.026). The SF PD1 expression on CD8 T-cells was higher in patients on-treatment those off-treatment (21.3% 5.83% p=0.004). The sPD1 levels were higher in the SF the serum (801pg/ml 367.2, p=0.013), without an association with disease activity. These results indicate an up-regulation of the PD1-pathway in JIA, at least quantitatively, especially in active disease. sPD1 is compartmentally produced at the inflamed joints. Further investigation in a larger sample of JIA patients may verify these observations and contribute to unravelling the precise role of the PD1 pathway in the pathogenesis and persistence of the joint inflammation.

This review aims to depict the profile of the childhood onset-SLE (c-SLE) and the related challenges that need to be managed in order to achieve an uneventful programmed transition from the family-oriented Pediatric Rheumatology Outpatient Clinic to the adult patient-focused one. Transition is a dynamic, continuous and interactive process where a triad is involved: the medical staff, the pediatric patient and the family. Their mutual collaboration and interaction start at the announcement of the diagnosis and is continuously supported by the ongoing multi-dimensional patient’s and family’s education. A prerequisite of a successful transition is the stepwise consciousness of c-SLE per se as well as the development of essential skills for the management of medical, psychosocial and educational/professional consequences derived during the course of the disease. The ideal time point of the final phase of transition (moving to adults’ care) requires disease remission and the patient’s confirmed dexterity for life self-management and coping with his/her difficult and unpredictable chronic disease.

Our aim was to study the effect of anti-TNF treatment on immunogenicity and safety of the 7-valent conjugate pneumococcal vaccine in children with juvenile idiopathic arthritis. Thirty-one children (mean age:12.9 ± 4.6 years) treated with anti-TNFs plus Disease Modifying Anti-Rheumatic Drugs (DMARDs) and 32 age-matched children treated only with DMARDs were vaccinated with two doses of PCV7. After the first vaccine dose geometric mean titers (GMTs) were significantly increased for all vaccine serotypes ( p < 0.0001) in both groups and were found to be protective (>0.35 μg/ml) in 87–100% of all children, depending on the serotype. Children receiving anti-TNFs achieved a significantly lower GMTs against serotypes 4, 14 and 23F ( p < 0.05). A ≥4-fold increase of the baseline titers to ≥5 vaccine serotypes was observed in 50% and 75% of the anti-TNF and control patients, respectively ( p = 0.0697). No patient developed vaccine-associated serious adverse events or disease flares.

The programmed cell death protein-1 (PD-1) and its ligands (PD-L 1 and 2) suppress immune responses, thus promoting self-tolerance. Among the immunomodulatory cells, acting through the PD-1 pathway, are the B-regulatory cells (Bregs). The role of the PD-1 pathway in Juvenile Idiopathic Arthritis (JIA) has not been adequately studied. To investigate the immunophenotypic profile of T- and B-cells and the activity of the PD-1 pathway in JIA patients. More specifically, we will examine the levels of: a) the soluble form of PD-1 (sPD-1), b) Bregs; and the expression levels of: c) PD-1 on CD4+ and CD8+ T-cells, d) PD-L1 on Bregs and CD19+ B-cells, in blood and synovial fluid samples, at various stages of the disease (onset, relapse, remission, on or off treatment). The above biomarkers will be investigated for correlation with JIA activity. A case-control study of JIA patients (expected number: 60) and healthy controls (n: 20). Total expected number of samples: 100 of peripheral blood, 120 of serum (solely for soluble markers) and 60 of synovial fluid. The patients' demographic data and treatment will be recorded. JIA will be classified according to the ILAR and the recently proposed PReS/PRINTO criteria. JIA activity will be assessed using the JADAS-10 tool. The biomarkers will be determined using multiparametric-polychromatic flow cytometry (quintuple fluorescence protocol) and immunoenzymatic assay ELISA. Further elucidation of the immunophenotypic expression and variation of the abovementioned molecules and cells during active inflammation and remission in JIA. Thereby, the present study is expected to contribute to: a) the modern research and understanding of the confirmed immune dysfunction at the cellular level, which leads to the development of serious autoimmune diseases in childhood, such as JIA, and b) the search for biomarkers that could be targets of early \"intelligent\" treatment and thereby could support the implementation of precision-medicine. The early diagnosis and targeted treatment of JIA are crucial for the maintenance of normal physical functioning and the psychosocial balance of the still growing adolescent/child.