P026 Efficacy and safety of Etanercept for the treatment of Juvenile Idiopathic Arthritis

Background Etanercept (ETN) is the first anti-TNF to have obtained FDA approval in 1999 for juvenile idiopathic arthritis (JIA) refractory to methotrexate. Currently, the indications of ETN cover the polyarticular JIA, the extended oligoarticular, and enthesitis-related arthritis. To assess the efficacy of Etanercept, as well as its tolerance in JIA. Material and methods We carried out a retrospective study of children with JIA according to the criteria of the ILAR classification and treated with Etanercept at the pediatric center of the CHU de Sétif since 2015. Nineteen children were included and considered to 3 months, 6 months, 1 year, 2 years, 3 years, 4 years, 5 years on the criteria epidemiology, the efficiency criteria (joint Scores, uveitis, ESR, CHAQ), and the occurrence possible side effects. We defined the improvement of 30% (ACR 30), 50% (ACR 50), 70% (ACR 70), 90% (ACR 90), and 100% (ACR 100) as the improvement of minus 3 criteria out of 6 of 30%, 50%, 70%, 90%, 100%; patients must not have a worsening of > 30% of any of the 6 criteria. Results The epidemiological features were the following: 12 girls and 7 boys, 10 present polyarticular form, 6 present oligoarticular form, 2 cases with psoriatic arthritis, and a single case of enthesitis-related arthritis. ACR 30 is obtained in 75%, 84%, 88% of cases at 3 months, 6 months, and 1 year, respectively. The strongest responses were obtained in polyarticular, oligoarticular, and enthesitis-related arthritis. Complete remission was maintained in the majority of patients for varied durations depending on the follow-up. Furthermore, no clinical or biological adverse effects were noted. Conclusion The Etanercept has been dramatically effective in children with juvenile idiopathic arthritis, especially in the polyarticular subtype, oligoarticular, and enthesitis-related arthritis. Its overall tolerance is very good.

P018 Functional impairment in enthesitis related arthritis patients

Background Enthesitis related arthritis (ERA) is a subgroup of juvenile idiopathic arthritis. It is characterized by the presence of enthesitis and predominately lower limb arthritis and can affect sacroiliac joint and spine. Recent studies showed that ERA is associated with worse physical status and poorer quality of life (1). The main objective of this study was to compare the aspects of functional status in patients (ERA) and patients with spondyloarthritis (SpA). Methods A retrospective monocentric study was carried out on patients with ERA (ILAR criteria) or SpA (ASAS Criteria). Demographic data and clinical characteristics were obtained from medical records. Disease activity was evaluated by: erythrocyte sedimentation rate (ESR), C-reactive protein rate (CRP) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Functional impairment was evaluated by Bath Ankylosing Spondylitis Functional Index (BASFI) and Ankylosing Spondylitis Quality of life Questionnaire (ASQoL). Global well-being was assessed by Bath Ankylosing Spondylitis Global Index (BASGI). Population was divided into two groups: group 1 (G1) stands for ERA patients and group 2 (G2) stands for SpA patients. P < 0.05 was considered statistically significant. Results A total of 174 patients (40 ERA and 134 SpA) were enrolled. Mean age at disease onset was 12.4 ± 3 years in G1 and 27.8 ± 8 years in G2. Male to female sex ratio was 5.6 in G1 and 3.7 in G2. Morning stiffness (>60 min) was reported by 37.5% of G1 and 49.3%. G1 patients had longer morning stiffness than G2 (61 [0–90] min vs 30 [0–240] min; P = 0.58). Multiple nocturnal awakenings were reported by 45% of G1 patients and 58.2% of G2 patients. Median BASDAI score was 4.9 [1–44] in G1 and 4.5 in G2 [0–10] (P = 0.48). Median BASGI score was 6 [1.5–9.5] in G1 and 6 [0–10] in G2 (P = 0.58). Median ESR was 35 mm/h [8–90] in G1 and 35 mm/h [2–125] in G2. Median CRP was 18.2 mg/l [1–70] in G1 and 13 mg/l [3–180] in G2. The assessment of functional status revealed that G1 patients had higher BASFI scores than G2 patients (5.2 vs 4.5). The association between G1 and BASFI was statistically significant (P = 0.05). Median ASQoL was 12 [2–17] in G1 and 9 [0–18] in G2. No link was noted between G1 and ASQoL score (P = 0.152). Conclusion Our study showed that ERA was associated with higher BASFI scores in comparison with SpA. Treat-to target strategies are mandatory in order to optimize the functional status of children with ERA.

P028 Impact of Juvenile Idiopathic Arthritis on schooling

Background Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children and is one of the major causes of morbidity and physical disability. Due to frequent absences, children with chronic health impairments are also often confronted with educational difficulties. The aims of this study were to assess the impact of JIA on children’s schooling and to determine the factors that influence their school level. Methods This is a cross-sectional study including patients with JIA (ILAR criteria). A detailed questionnaire was completed for each participant by interviewing them or their parents as well as by information obtained from their medical records. Collected data included age, sex, subtype of JIA, disease duration, level of disability according to the Childhood Heath Assessment Questionnaire (CHAQ), visual analogue scale for patient’s overall assessment of disease activity (VASOA), duration of morning stiffness, tender joint counts (TJCs), swollen joint counts (SJCs), erythrocyte sedimentation rate (ESR), C-Reactive Protein (CRP), Disease Activity Score (DAS28) for polyarticular and oligoarticular JIA, Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) for Enthesitis-related arthritis. Medications used for JIA treatment were also documented. Data on the school performance of patients and their siblings were obtained using telephone interviews (educational level, absenteeism, school delay by repetition, drop-out). Results A total of 43 patients with JIA were included, 25 female and 18 male, with a mean age of 26 years [12–51] and a mean disease duration of 237 months (5–496). The average age of the onset of the disease was 7.4 years [1.5–16]. The most common subtype was rheumatoid factor-positive polyarthritis (n = 18) followed by systematic (n = 8), oligoarticular (n = 8), rheumatoid factor-negative polyarthritis (n = 5) and Enthesitis-related arthritis (n = 4). The mean DAS28 was 3.02 [0.76 – 5.55] and the median CHAQ was 0.66 [0–3]. Twenty-nine of the children were receiving corticosteroid. Disease-modifying anti-rheumatic drugs were used by 38 of the 43 patients: methotrexate (n = 27), sulfasalazine (n = 8), leflunomide (n = 7), biotherapies (n = 16). Twenty patients had complications: Hip arthritis (n = 18), growth stunting (n = 14), uveitis (n = 5). Joint replacement was required in 11 cases. Four patients were illiterate, 14 had dropped out of school, 24 reported repeated absences due to illness. A year of schooling was repeated by 50.85% of patients. Eleven out of 32 patients over the age of 20 had an university level. Almost 80% of patients were exempted of physical education. There were no significant associations between the school-related problems, the socio-demographic characteristics and the various parameters of clinical and biological activity studied. Conclusion Our study suggested that JIA negatively affects schooling of children. More studies, with a larger sample of children, are needed to identify the variables associated with school failure in order to ensure the proper management of these patients and to increase their academic performance.

P057 Uveitis in enthesitis-related arthritis: what are the predictive factors?

Background Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood. Uveitis is its most common extra-articular manifestation [1]. It is a potentially sight-threatening condition with a considerable risk of morbidity. This study aimed to describe the prevalence and characteristics of JIA-related uveitis in enthesitis-related arthritis (ERA) patients. Methods We conducted a retrospective study including 40 patients with JIA according to the classification criteria of the International League of Associations for Rheumatology (ILAR). All selected patients presented with ERA. A screening of uveitis was conducted in all patients. Clinical, radiologic and, biologic features of JIA were collected. We evaluated the association between these features and the presence of uveitis. Results The mean age of our patients was 24 years, with a sex ratio of 3. The mean age at the onset of JIA was 11 years. Upon screening, fifteen percent of the patients had active uveitis. Nine percent of the patients presented with an acute onset of JIA. Forty-six percent had initial spinal symptoms, while 26% presented with peripheral onset. Upon examination, 4% of the patients presented with monoarthritis, 20.4% with oligoarthritis and 34.8% with polyarthritis. Thirteen percent had enthesitis. Fifty-seven percent of the patients had coxitis. Sixty-eight percent had sacroiliitis. The mean CRP and ESR levels were respectively 16 mg/l and 34. Human leucocyte antigen (HLA) B27 screening came back positive in 11% of the cases. The mean BASDAI and BASFI levels were both at 4.7. The mean MASES was 0.44. Twenty percent of the patients received NSAIDs. Eleven percent received methotrexate, 18% salazopyrine and, 5.6% biologics. A significant association was established between the presence of uveitis and a polyarticular onset of JIA (P = 0.036). However, no significant associations were established with the sex of patients (P = 0.457), age of onset (P = 0.828), activity of the disease as evaluated by BASDAI (P = 0.40) and MASES (P = 0.87), inflammatory markers (CRP P = 0.946, and ESR P = 0.662), the use of NSAIDS (P = 1), methotrexate (P = 0.318), salazopyrine (P = 0.170) and biologics (P = 1). Conclusion In conclusion, uveitis associated with JIA is a serious and sight-threatening disease. Several factors associated with a more severe disease development have been identified [2–4]. Our study showed a significant association between the polyarticular onset of JIA and the occurrence of uveitis. Also, our study showed no significant association with male gender and HLA-B27 in children, unlike previous studies of spondyloarthritis conducted in adults [5–6]. We conclude that the screening of uveitis should be performed in all JIA patients, especially those presenting initially with polyarthritis.

P009 Osteoporosis is a frequent complication in enthesitis related arthritis patients

Background Enthesitis related arthritis (ERA) represents a clinical entity of juvenile idiopathic arthritis. This chronic rheumatic disease may lead to early bone mass loss and increase risk fracture. The aims of this study were to evaluate the prevalence of clinical osteoporosis in patients with ERA and to identify what factors are associated with increased occurrence of osteoporosis. Methods We reviewed the medical records of patients with confirmed ERA. We analyzed their demographic data and the clinical characteristics. Dual-energy X-ray absorptiometry (DEXA) was used to determine bone status. Osteoporosis was defined as Z score <-2.5DS. Disease activity was evaluated by: erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Data were analyzed using the SPSS statistical package. A P-value < 0.05 was considered significant. Results Thirty-three patients (27 male and 7 female) with a mean age at of 23.8 ± 7.5 years were enrolled. The mean age at disease onset was 12 ± 2.6 years. Median disease duration was 108 months [12–408]. The median ESR and CRP levels were 35 mm/h [8–90] and 20 mg/l [1–70] respectively. Median BASDAI score was 4.7 [1–9.7]. At bone densitometry, osteoporosis and osteopenia were found in 44.1% and 23.5% cases respectively. None of the patients had a history of osteoporotic fractures. Long term corticosteroid therapy and sedentarily were noted in 18.2% and 47.1% of patients respectively. On statistical analysis, osteoporosis was associated with these parameters: age at ERA onset (P = 0.035), disease duration (P = 0.04), CRP (P = 0.009), BASDAI score (P = 0.05) and sedentarily (P = 0.031). Neither corticosteroid therapy (P = 0.68) nor high ESR level (P = 0.73) were associated with osteoporosis. Conclusion In this study, osteoporosis was a common extra articular feature during ERA. As adult spondyloarthritis, disease activity, duration and sedentarily seem to be associated with the bone loss.

P029 Assessment of diagnosis delay during enthesitis related arthritis

Background Enthesitis related arthritis (ERA) is a distinct subgroup of juvenile arthritis characterized by male predominance and adolescent onset. Though, ERA patients still experience long diagnosis delays. This may lead to articular damage and functional disability. The aim of this study was to quantify the lag time between ERA symptoms onset and diagnosis and to evaluate its impact on disease activity, functional disability and structural damage. Methods A retrospective monocentric study was carried out on ERA patients. Diagnosis delay was collected from patients’ medical files. Disease activity was evaluated by: erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Functional disability and structural damage were evaluated by Bath Ankylosing Spondylitis Fonctional Index (BASFI) and Bath Ankylosing Spondylitis Radiology Index (BASRI) respectively. Data were analyzed using the SPSS statistical package. A p-value < 0.05 was considered significant. Results Thirty-four patients with a mean age of 23.8 ± 7.5 years were included. Male to female sex ratio was 3.85. Mean age at disease onset was 12 ± 2.6 years. Median disease duration was 108 months [12–408]. Median ERA diagnosis delay was 10 months [3–108]. Median ESR and CRP were 35 mm/h [8–90] and 20 mg/l [1–70] respectively. Median BASDAI score was 4.7 [1–9.7]. Median BASFI and BASRI scores were 4.6 [1.9–10] and 10 [2–16] respectively. Coxitis was found in 38.2% of cases. On statistical analysis, significant positive correlation was found between ERA diagnosis delay and ESR (P = 0.03, r = 0.69) and CRP (P = 0.05, r = 0.456) respectively. No link was noted between ERA diagnosis delay and these parameters: gender (P = 0.58), age at disease onset (P = 0.68), occurrence of coxitis (P = 0.66), BASFI (P = 0.08), BASDAI (P = 0.45) and BASRI (P = 0.12). Conclusion ERA patient’s journey was long in our study. Longer delays were associated with higher ESR and CRP levels. Further studies are required to confirm our results.

P038 Let’s look at coxitis features in enthesitis-related arthritis population

Background Enthesitis-related arthritis (ERA) is a defined juvenile idiopathic arthritis subtypes, which presents with enthesitis, arthritis and axial skeleton involvement. ∼20–50% of JIA patients have hip involvement within 1–6 years of diagnosis onset [1]. The frequency of coxitis in ERA category is not recognized. The aim of this study was to assess coxitis features and its management in ERA population. Methods A retrospective study including children with ERA according to the International League of Associations for Rheumatology (ILAR). Data recorded included sociodemographic features, disease characteristics (disease duration, extra-articular manifestations, and presence of HLAB27) as well as treatment modalities. Regarding coxitis, we collected radiographs, ultrasound (US) and magnetic resonance imaging (MRI) of the hip when performed. Coxitis was defined by clinical (limited range of motion) and/or radiographic findings (destruction, synovitis, bone marrow oedema). Results The study included 51 patients with ERA. There was a male predominance (78.4%). The mean age of onset of the disease was 12.2 years [6–16]. The mean current age was 24.3 years old [9–59]. A family history of spondyloarthritis was found in 26.8% of cases. A positive HLAB27 was reported in 85.7% of cases. The distribution of extra-articular manifestations (37.5%) was as follows: ocular (n = 8), cardiovascular (n = 2), gastrointestinal (n = 1), pulmonary (n = 4). A peripheral onset was found in 39% and a peripheral and axial onset was reported in 42% of patients. Hip involvement was found in 78.4% of the patients and revealed the disease in 43% of cases. The mean delay between disease onset and coxitis was 4.5 years [0–34]. Coxitis was bilateral and destructive in 82.5% and 51% of cases respectively. The most limited range of motion was the internal rotation (68%), followed by hip flexion (48%) and the external rotation (43.2%). In patients with normal hip radiography (n = 8), US or MRI depicted early changes in 75% of cases. Hip replacement was noted in ten patients and was bilateral in 70% of cases. Regarding treatment modalities, NSAIDs and csDMARD (MTX n = 14, SLZ n = 8, biologics n = 2) were prescribed in 76.4% and 52% respectively. Most of the patients had physical therapy (88%) and 23.5% of them had intra-articular corticoid injection. Twenty-six percent of the patients had hip replacement. Conclusion Our study showed a high prevalence of coxitis among ERA patients. There is a need to further optimize therapeutic strategies for such patients.

P022 Genetic background predicts the extra-articular involvement in patients with enthesitis related arthritis

Background Enthesitis-related arthritis (ERA) represents 20% of all juvenile idiopathic arthritis subtype. Among the genetic risk factors for the development of ERA, HLA-B27 has been implicated as a major contributor. The frequency of HLA-B27 varies among population. HLA-B 27 status in ERA may influence the clinical phenotype and prognosis of the disease. The main objective of this study is to determine whether genetic background including HLA B27 and familial history of spondylarthritits (SpA) may influence the clinical features of ERA patients. Methods We conducted a retrospective study including patients with ERA, all fulfilling the International League of Associations for Rheumatology (ILAR) criteria. For all patients, we collected the following data: Age, family history of rheumatic inflammatory diseases, inflammatory bowel diseases (IBD), the presence of HLA-B27 antigen, the inflammatory biomarkers: Erythrocyte sedimentation rate (ESR) C-reactive protein (CRP), the disease activity assessed by morning stiffness, night awakenings, the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and the current treatment. We assessed, as well, the functional impact using the Bath Ankylosing Spondylitis Functional Index (BASFI) the Lequesne index. The population was divided into two groups: a group including patients positive to HLA-B27 antigen and/or with family history of rheumatic inflammatory diseases, psoriasis or inflammatorybowel diseases. The second group was defined as control group with patients negative to HLA-B27 antigen and without any family history of the diseases above. Results We included 40 ERA patients, mean age of onset 12,43 ± 3,003 years (6–16). The majority of them were male (n = 34). Twenty-eight patients had a genetic background. Among them, 7,5% of patients had a positive family history, 42,5% were positive to HLA-B27 antigen and 20% of them met both criteria. As shown in table 1, clinical manifestations were similar between the 2 groups. Enthesitis was more frequent in patients with HLAB27 without a significant difference. Regarding the disease activity, the number of night awakenings and the morning stiffness duration were comparable in the two groups. Six patients had a BASDAI score > 4 with no difference between the two groups. Extra-articular manifestations were present in 15 patients. Among them 14 had a genetic background, reaching the significance threshold with P = 0,013. We counted 8 cases of uveitis, one case of IBD, 5 cases of lung disease and 1 case of cardiac involvement. Inflammatory markers were higher in the group with familial history of SpA and/or positive HLAB27. Indeed, the mean ESR value was 42,73 vs 29.9, P = 0,01. There were no correlations between BASFI score and a positive genetic background (P = 0,283). Only one patient was put on biologics. He has no family history and is negative to HLA-B27 antigen. Conclusion The frequency of HLAB27 was in line with the literature data. The genetic background did not influence the disease activity or the functional impairment in our population. However, a positive correlation was found between a positive familial history of SpA, HLAB27, and the presence of extra-articular manifestations as well as with a higher ESR value.

Enthesitis Related Arthritis: A Single Center Experience

Objective: Enthesitis-related arthritis is a subtype of juvenile idiopathic arthritis category, characterized by enthesitis, arthritis, and the risk of axial involvement. We aimed to summarize the demographics, clinical, and laboratory findings of enthesitis-related arthritis patients and to identify the distinguishing features of enthesitis-related arthritis patients with HLA B27 positive compared to the patients who were HLA B27 negative. Materials and Methods: This retrospective study included patients with Enthesitis-related arthritis who followed up between 2015 and 2018. Demographical, clinical, and laboratory data were retrospectively reviewed from the patient files and computerized medical charts. Results: A total of 72 patients diagnosed with enthesitis-related arthritis were included in the study. The male/female ratio was 2.1/1. Fifty-three (73%) of them presented with peripheral arthritis. The most commonly affected joint was knee (81.1%), followed by the ankle (43%), hips (32%), and wrist (5%). HLA B27 was positive in 36 (50%) patients. During follow-up, the number of patients who developed enthesitis-related arthritis -associated uveitis was 8 (11.1%). During follow-up, 56 patients with inflammatory back pain and/or sacroiliac tenderness underwent spinal MRI. Ten (17.8%) patients had only thoracal and/or lumbar involvement, 18 (32%) had only sacroiliitis, and 9 (16%) patients had both of them on spinal MRI. In comparison with HLA-B27-negative children, HLA-B27-positive patients were more likely to have enthesitis (16 (44.4%) vs 8 (22.2%), p=0.046), MRI proven sacroiliitis (19 (52.7%) vs 8 (22.2%), p=0.031), MRI proven spinal involvement (13 (36.1%) vs 6 (16.6%), p=0.031), and uveitis (8 (100%) vs 0(0%), p=0.014). During follow up, 65/72 (90.2 %) of them needed disease-modifying antirheumatic drugs (DMARD), and 51/72 (70.8%) needed anti-tumor necrosis factor-α (TNF-α) therapy. Conclusion: We found that patients who were HLA-B27- positive had significantly more enthesitis, MRI-proven sacroiliitis, MRI-proven spinal involvement, and acute anterior uveitis, in comparison to patients who were HLA B27 negative. It is crucial to carefully assess those patients with concern for enthesitis-related arthritis to determine the expected prognosis and make therapeutic decisions appropriately.