Juvenile Idiopathic Arthritis Involvement Research Articles

Initial Respiratory System Involvement in Juvenile Idiopathic Arthritis with Systemic Onset Is a Marker of Interstitial Lung Disease: The Results of Retrospective Cohort Study Analysis.

Background: Pulmonary involvement in systemic juvenile idiopathic arthritis (SJIA) is a rare but dangerous complication. The main risk factors are already known, such as macrophage activation syndrome, a refractory course of systemic juvenile arthritis, infusion reaction to interleukin 1 and/or interleukin 6 blockers, trisomy 21, and eosinophilia. However, information about respiratory system involvement (RSI) at the onset of SJIA is scarce. Our study aimed to evaluate the specific features of children with SJIA with RSI and their outcomes. Methods: In a single-center retrospective cohort study, we compared the information from the medical records of 200 children with SJIA according to ILAR criteria or SJIA-like disease (probable/possible SJIA) with and without signs of RSI (dyspnea, shortness of breath, pleurisy, acute respiratory distress syndrome, and interstitial lung disease (ILD)) at the disease onset and evaluated their outcomes (remission, development of chronic ILD, clubbing, and pulmonary arterial hypertension). Results: A quarter (25%) of the SJIA patients had signs of the RSI at onset and they more often had rash; hepato- and splenomegaly; heart (pericarditis, myocarditis), central nervous system, and kidney involvement; hemorrhagic syndrome; macrophage activation syndrome (MAS, 44.4% vs. 9.0%, p = 0.0000001); and, rarely, arthritis with fewer active joints, compared to patients without RSI. Five patients (10% from the group having RSI at the onset of SJIA and 2.5% from the whole SJIA cohort) developed fibrosing ILD. All of them had a severe relapsed/chronic course of MAS; 80% of them had a tocilizumab infusion reaction and further switched to canakinumab. Unfortunately, one patient with Down's syndrome had gone. Conclusion: Patients with any signs of RSI at the onset of the SJIA are required to be closely monitored due to the high risk of the following fibrosing ILD development. They required prompt control of MAS, monitoring eosinophilia, and routine checks of night oxygen saturation for the prevention/early detection of chronic ILD.

Proteinuria and hematuria as early signs of renal involvement in juvenile idiopathic arthritis.

To evaluate glomerular and tubular renal functions and analyze blood pressure in a cohort of pediatric patients with juvenile idiopathic arthritis (JIA). A total of 40 pediatric patients, 20 (50%) with JIA and 20 (50%) healthy control subjects, were studied, and performed the renal function on 24-h collection and the 24-h ambulatory blood pressure monitoring (ABPM). Moreover, we compared renal function and blood pressure trends between the groups of JIA patients with different disease activities. No statistically significant differences were observed between patients with JIA and healthy children in terms of glomerular filtration rate (GFR), fractional excretion of sodium (FENa), tubular reabsorption of phosphate (TRP), and calcium-creatinine urine ratio (CaU/CrU). In contrast, we observed significantly higher values in JIA patients than in controls for the presence of hematuria (p < 0.0001) and proteinuria (p < 0.0001). Compared to the control group there were significantly higher values of hematuria and proteinuria/day in both groups of JIA patients with low disease activity (respectively, p = 0.0001 and p = 0.0002) and moderate disease activity (respectively p = 0.0001 and p = 0.0012). Systolic and diastolic dipping were significantly reduced in patients with JIA compared with healthy controls (p < 0.0001 and p < 0.0001, respectively). Our study showed that children with JIA, already in the early stages of the disease, have higher values of hematuria and proteinuria, which are early warning signs of nephropathy. Therefore, detailed screening of renal function and pressure monitoring in patients are necessary to monitor their evolution over time.

Temporomandibular involvement in children and adolescents with juvenile idiopathic arthritis: a 2-year prospective cohort study

This study aimed to clinically evaluate temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) and the ability to identify and/or predict development of TMJ-deformities over time using cone beam computed tomography (CBCT). The predictive value of self-reported TMJ pain was also assessed. A prospective longitudinal cohort study comprising 54 children with JIA, 39 girls and 15 boys, was performed. All children had active disease at baseline, 50% with the subtype oligoarthritis. Repeated clinical orofacial and CBCT examinations were performed over a two-year period. At baseline, 39% had radiographic TMJ deformities (24% unilateral, 15% bilateral), at 2-year follow-up, 42% (p > 0.05). Both progressing and improving TMJ deformities were observed. An association was found between TMJ-deformities and self-reported TMJ pain at baseline (p = 0.01). Maximum unassisted mouth opening (MUO) was smaller for children with TMJ-deformities (p < 0.05). The prevalence of palpatory muscle pain was high (48–59%) but not predictive of development of TMJ-deformities. TMJ noises increased over time and crepitations were associated with TMJ-deformities (p < 0.05). In conclusion, in children with JIA, self-reported TMJ pain and dysfunction were common and predictive of TMJ deformities. TMJ deformities were associated with smaller MUO and palpatory TMJ pain as well as crepitations. Trial registration. ClinicalTrials.gov Protocol id: 2010/2089-31/2.

Algorithm for Growth Evaluation in Juvenile Idiopathic Arthritis.

Juvenile Idiopathic Arthritis (JIA) includes a range of inflammatory conditions that exhibit chronic arthritis with various clinical presentations. The disease's heterogeneity leads to different impacts on children's health, both short and long-term. Compromised growth, seen as growth retardation and delayed puberty, is a common complication in children with JIA, severely impacting their quality of life. This impairment is linked to disease duration and activity, with severe cases in systemic and polyarticular subtypes. Literature reports growth retardation incidence from 8% to 41%, but data on pubertal impairment is lacking. Growth in children is influenced by systemic and local mechanisms. Chronic inflammation, prolonged glucocorticosteroid (GCS) use, and nutritional issues contribute to growth stunting and pubertal delays. Chronic inflammation in JIA flattens growth curves, while steroid treatment impairs growth and causes weight gain. Disruption of the GH/IGF1 axis is known, but data on systemic hormonal resistance in JIA are insufficient. Optimizing JIA treatment, including biological therapies, is expected to improve growth velocity and reduce long-term impacts by better disease control and reduced GCS doses. Thyroid function also influences growth and puberty, but comprehensive studies on thyroid involvement in JIA are lacking. Given the early onset of chronic inflammatory consequences, preventive auxological screening measures are necessary for children with JIA. Early detection of developmental disorders can enhance therapeutic management. This article summarizes information from a cohort study on growth in children with JIA and proposes a diagnostic algorithm for clinical use.

A rare case of severe isolated temporomandibular joint involvement in juvenile idiopathic arthritis

Background: Juvenile idiopathic arthritis is an inflammatory condition of unknown etiology. Early temporomandibular joint involvement may be asymptomatic or with mild symptomatology, despite radiological signs of substantial joint damage. Early diagnosis and management is of substantial importance, because orofacial symptoms due to temporomandibular joint arthritis may be disabling and interfere with impaired quality of life or even dentofacial abnormalities. When temporomandibular joint involvement is the first and only manifestation - a very rare event – it is easy to remain undiagnosed. It is noteworthy that the current body of published material on isolated arthritis of the temporomandibular joint is limited to 2 case reports and a multinational cohort of pediatric rheumatology centers.

The childhood arthritis radiographic score of the hip: the proposal cut-off value using cluster analysis.

Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease that affects children. It is crucial to detect and treat hip involvement in JIA early to prevent functional impairment and reduced quality of life. The Childhood Arthritis Radiographic Score of the Hip (CARSH) is a validated radiographic scoring system used to assess hip involvement in JIA. In this study, we aimed to determine cut-off values for CARSH scores using cluster analysis. The study was conducted as a cross-sectional analysis and included JIA patients with hip involvement who underwent a pelvic radiograph. The same pelvic radiograph was interpreted by two experienced pediatric rheumatologists at baseline and after 3weeks by both readers for reliability. The CARSH scores were calculated for each hip four times (twice by each reader). For the 50 hips, a total of 200 interpretations of the CARSH score were obtained. Model-based clustering was employed to identify distinct groups of CARSH score interpretations and characterize the phenotype of each cluster. Twenty-five children with hip involvement were included. The mean age was 13.9 ± 4.6years. JIA subtypes were as follows: ERA in 64%, oligoarthritis in 16%, psoriatic arthritis in 12%, polyarthritis RF + in 4%, and RF - in 4% of patients. For the 200 hip interpretations, three clusters based on the level of the CARSH were identified by model-based clustering. Cluster 1 consisted of 17 CARSH score interpretations with a median score of 7 ± 3 (ranging from 1 to 15). This group primarily comprised patients with enthesitis-related arthritis (ERA) and psoriatic arthritis. Patients in cluster 1 were generally older, experienced longer diagnostic delays, and had a longer disease duration compared to the other clusters. Cluster 2 exhibited a moderate CARSH score, with an average score of 4 ± 3 (1 to 15). Patients in this group had significantly higher body weight compared to the other clusters. Cluster 3 represented the group with the least severe hip involvement, characterized by CARSH scores of 2 ± 1 (ranging from 0 to 9). This cluster had a higher proportion of male patients and higher C-reactive protein (CRP) levels than the other clusters. Regarding the individual items of the CARSH score, cluster 1 showed higher percentages of hip radiograph abnormalities such as joint space narrowing, erosions, growth abnormalities, and subchondral cysts. Cluster 2 was characterized by a high rate of acetabular sclerosis, with little to no abnormalities in other CARSH score items. Cluster 3 was the only group that exhibited hip subluxation, with minimal abnormalities in the other score items. In conclusion, this study identified three distinct groups of CARSH scores, representing varying levels of severity in hip involvement in JIA. These findings provide valuable insights for clinicians in assessing and managing JIA patients with hip involvement, enabling tailored treatment strategies based on the severity of the condition. Key Points • While a Childhood Arthritis Radiographic Score of the Hip (CARSH) is a valid and reliable tool in hip-related juvenile idiopathic arthritis, its use is limited in daily practice due to the lack of available cut-off values. • The cluster analysis defined three clusters based on the CARSH levels. • Cluster 1 exhibited the highest score with more damage and disability. Cluster 2 involved a moderate score and more overweight patients. Cluster 3 included the least level of the score but with an active disease parameter.

Ultrasound and MRI of the foot in children and adolescents newly diagnosed with juvenile idiopathic arthritis.

To evaluate the spectrum of inflammatory features in foot joints which may be detected on routinely performed ultrasound (US) and magnetic resonance imaging (MRI) in children newly diagnosed with juvenile idiopathic arthritis (JIA). Two groups of children hospitalized in a reference center for rheumatology, newly diagnosed with JIA and suspected of foot involvement in the course of JIA were included in this retrospective study. In the first group of 47 patients aged 1-18 years, the imaging was restricted to US. The second group of 22 patients aged 5-18 years underwent only non-contrast MRI of the foot. The most frequent pathologies seen on US included effusion and synovial thickening in the first metatarsophalangeal joint (MTP1), followed by the tibiotalar joint. Synovial hyperemia on color Doppler US images was present most frequently in the Chopart and midtarsal joints (64%; 7/11 cases), followed by the tibiotalar joint (45%; 5/11), and MTP2-5 joint synovitis (40%; 4/10). Grade 3 hyperemia was present only in four cases; grades 1 and 2 were detected in the majority of cases. On MRI, bone marrow edema was the most frequent pathology, found mostly in the calcaneus (45%; 10/22 cases), while alterations of the forefoot were rare. No cases of bursitis, enthesitis, cysts, erosions or ankylosis were diagnosed in either of the analyzed groups. Routine US of the foot is recommended for early detection of its involvement in JIA in daily clinical practice. Although MRI can identify features of various JIA stages, it is particularly useful for the detection of bone marrow alterations.

E10 Temporo-mandibular joint involvement risk factors in juvenile idiopathic arthritis

Abstract Introduction Recent recommendations suppose temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) is a risk factor of poor outcomes and severe disease course. Objectives To compare the clinical characteristics of patients with juvenile idiopathic arthritis with and without TMJ involvement. Methods We analyzed the data of 753 patients with juvenile idiopathic arthritis aged 2–17 years, depending on TMJ arthritis (n = 43; 5.7%) or no TMJ arthritis (n = 710; 94.3%). Clinical, laboratory characteristics and treatment regimens were compared. Odds ratio (OR) analysis of sensitivity (Se) and specificity (Sp) was performed to obtained the predictors of TMJ involvement. Results Despite the similar age of onset, TMJ arthritis was associated with longer course of the disease, polyarticular JIA category, use of systemic corticosteroids and longer achievement of remission with similar rate of biologic administration. TMJ arthritis was associated with involvement of cervical spine, hip and shoulder arthritis. The main risk factors, associated with TMJ involvement in JIA were active joints &amp;gt; 8 (OR = 14.9 (5.8; 38.3), P = 0.0000001), delayed remission &amp;gt; 7 years (OR = 3.1 (1.6; 5.8), P = 0.0004), delayed hip involvement (OR = 4.6 (0.9; 22.6), P = 0.041), hip osteoarthritis (OR = 4.0 (1.2; 13.0), P = 0.014), and avascular necrosis of the hip. Cervical spine arthritis (OR = 10.3 (5.4; 19.8), P = 0.000001), and corticosteroid treatment (OR = 2.3 (1.2; 4.4), P = 0.0007) were associated with TMJ arthritis. Patients with TMJ arthritis required more biologics (OR-3.2 (1.6; 6.2), P = 0.0006). Oligoarticular JIA category and uveitis were protective against TMJ involvement. Data are in the table. Conclusion TMJ involvement is marker of poor disease prognosis. Early initiation of biologics and avoidance of corticosteroids might improve the risk of TMJ involvement. Ethics Written consent was obtained according to the declaration of Helsinki. This retrospective study's protocol was approved by the local Ethical Committee of Saint Petersburg State Pediatric Medical University (protocol number 11/10 from 23.11.2020).

E13 Cervical spine involvement in juvenile idiopathic arthritis: frequency and associated factors

Abstract Background Juvenile idiopathic arthritis (JIA) is a pediatric rheumatism including several subgroups. The cervical spine involvement is poorly studied in JIA compared with adult rheumatism. Objectives This study aimed to assess the frequency and associated factors with cervical spine involvement in JIA. Methods We conducted a retrospective study including adults with long-standing JIA according to International League of Associations of Rheumatology (ILAR) criteria over a 28-year period (1994 – 2022). We retrieved radiographs of the cervical spine in anteroposterior, lateral, and lateral flexion views in search of an atlantoaxial dislocation (AAD). Demographic, clinical, biological, and radiological data were compared according to the presence or absence of AAD. Results A total of 29 Patients were enrolled (12 men and 17 women), the mean age was 35.69 ± 11.72 [18–61] years. The mean age of disease onset was 11.10 ± 4.25 [2–16] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months. The average disease duration was 24.48 ± 12.76 [1–47] years. The mean body mass index (BMI) was 21.20 ± 4.88 [14.17–27.55] kg/m2. Most patients had a polyarticular form (55.2%, n = 16). Mean C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were 42.74 ± 63.37 [2–218] mg/l and 69.67 ± 36.23 [36–108] mm/h. A biological inflammatory syndrome was present in 19 cases. Rheumatoid factor, ACPA and anti-nuclear antibodies were observed in 12, 7 and 5 cases respectively. At least one extra-articular involvement was noted in 16 cases. Bone erosions and hip involvement were noted in 18 and 14 cases, respectively. AAD was noted in 4 patients (13.8%). It was diagnosed on average 4.28 ± 10.07 [0–27] years after disease onset. AAD was only associated with extra-articular involvement in our study (75% vs 13.6%; P = 0.047). On the other hand, our study showed that age, gender, age at onset, diagnostic delay, duration of symptoms, BMI, JIA sub form, inflammation markers, rheumatoid factor, ACPA, antinuclear antibodies, bone erosions, and hip involvement were not associated with AAD. Conclusion The cervical spine involvement was not negligible in our study and was associated with extra-articular involvement. This condition is serious and can be life threatening. Therefore, it is important to perform more studies to better evaluate its frequency, clinical presentation, and imaging.

E16 Extra-articular manifestations and hip involvement in juvenile idiopathic arthritis: what is the link?

Abstract Introduction Juvenile idiopathic arthritis (JIA) is a complex pediatric rheumatism. The systemic and structural involvement of the disease is both functional and life threatening. Hip involvement can reach 50% of cases, especially in severe and destructive cases. Objectives The objective of this work is to study the relationship between systemic and hip involvement in JIA. Methods This was a retrospective study including adults with long-standing JIA according to the International League of Associations for Rheumatology (ILAR) criteria over a period of 28 years (1994–2022). Socio-demographic, clinical, biological, and radiographic data were collected. Results There were 29 patients (17 females and 12 males), the mean age was 35.69 ± 11.72 [18–61] years. The mean age of disease onset was 11.10 ± 4.25 [2–16] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months. The average disease duration was 24.48 ± 12.76 [1–47] years. At least one extra-articular manifestation was noted in 20 patients. An ocular dry syndrome was present in 6 cases, with a delay of onset of 132.14 ± 134.18 [0–312] months. Skin involvement was present in 4 cases with a mean onset delay of 24.25 ± 47.83 [0–96] months. Three patients had a pulmonary interstitial syndrome, the onset delay of this pulmonary involvement was 228.00 ± 74.94 [0–312] months. Valvular involvement was present in 3 cases and pericarditis in only one case, the onset delay of cardiac involvement was 211.33 ± 129.40 [70–324] months. Renal involvement was noted in 2 cases: one case of proteinuria and one case of renal failure, the time of onset of renal involvement was 12.50 ± 16.26 [1–24] months. Furthermore, hematological damage such as anaemia was noted in 20 of the patients included. No patients had macrophage activation syndrome in our study population. Hip involvement was noted in 14 patients. It was bilateral in 9 cases. Twenty-three hips were affected in total. Hip involvement was diagnosed 14.5 ± 9.37 [1–28] years after disease onset. Our statistical study identified a single correlation between hip involvement and cardiac involvement during JIA (r = 0.413; P = 0.026). In contrast, no other correlation was identified between hip involvement and the rest of the extra-articular involvement in JIA. Conclusion Extra-articular and hip involvements are common in JIA. Both have a prognostic impact and must be systematically screened throughout the follow-up. In our study, hip involvement was correlated with cardiac involvement.

Foot-related impairment in children with juvenile idiopathic arthritis.

Foot involvement is present in approximately 60%-90% of children with Juvenile idiopathic arthritis (JIA). It is a major cause of disability, which can lead to deterioration in daily activities and quality of life. However, it is often overlooked and can compromise patient management. Our objective was to describe the ankle and foot involvement in JIA and to appraise the functional impact of this damage on the child's performance and quality of life by using validated scores. The cross-sectional study included patients with JIA. Functional impairment was assessed through the Juvenile Arthritis Functionality Scale (JAFS) and the Oxford Ankle and Foot Questionnaire for children. Twenty-three patients aged 12.75±3.9 and with 41months of disease duration. After completing the Oxford score, the physical domain appeared to be the most altered. A long delay in diagnosis was associated with an alteration in the emotional domain of the Oxford score. Higher disease activity was significantly associated with impairment in both the physical activity and the footwear domains of the Oxford score. As regards clinical examination data, the presence of foot pain as well as the presence of tendinopathy were associated with an alteration of all Oxford score domains. The presence of flat feet significantly affected all domains of the Oxford score. The JAFS was reported to affect the child's performance ability and was associated with impairment in physical activity, school and play, and emotional domains. Ankle and foot involvement was common in our study. Functional ability was most impaired in the lower limbs. High disease activity, foot and ankle pain, tenosynovitis, and flat feet were associated with poorer quality of life and higher functional impact.

Incidental findings involving the temporomandibular joint on computed tomography and magnetic resonance imaging.

The temporomandibular joint (TMJ) is frequently imaged in head and neck computed tomography (CT) and magnetic resonance imaging (MRI) studies. Depending on the indication for the study, an abnormality of the TMJ may be an incidental finding. These findings encompass both intra- and extra-articular disorders. They may also be related to local, regional or systemic conditions. Familiarity with these findings along with pertinent clinical information helps narrow the list of differential diagnoses. While definitive diagnosis may not be immediately apparent, a systematic approach contributes to improved discussions between clinicians and radiologists and better patient management.

06 Flat foot impairs physical activity in children with juvenile idiopathic arthritis

BackgroundChildren with juvenile idiopathic arthritis (JIA) are less active compared with the general population due to pain and deformities, mainly of the lower limbs. Indeed, only 23% of children with JIA meet the public health recommendations of one h of moderate to vigorous physical activity daily [1]. In this context, foot involvement in JIA is a considerable limiting cause of physical activity.ObjectivesOur study aimed to investigate the types of static foot disorders that impede physical activity in children with JIA.MethodsWe conducted a cross-sectional study of patients with JIA according to the revised ILAR criteria. Socio-demographic and disease-related data were recorded. All patients underwent podoscope examination for varus or valgus deformity of the hindfoot and plantar footprint abnormality (flat foot or hollow foot). Patients with ankle or foot involvement due to congenital malformation or any other cause besides JIA were excluded. The impact of foot involvement on physical activity was assessed by the Oxford ankle foot questionnaire for children (OxAFQ-C). A higher score represents better functioning. We looked for the effect of foot abnormalities and their impact on physical activity.ResultsA total of 23 patients were collected. The mean age was 12.7 ± 3 [6–18] years and the mean age at diagnosis was 9.3 ± 3 [3–16] years. There was a female predominance with a sex ratio of 0.42. The majority of patients had secondary education (52%). The distribution of the different subtypes was dominated by the oligoarticular form (30%) and the enthesitis-related arthritis form (26%), followed by the polyarticular FR + (n = 1), polyarticular FR- (n = 3), psoriatic arthritis (n = 3), systemic (n = 1) and undifferentiated (n = 1) forms. The mean physical activity score assessed by the Oxford score was 73.52 ± 35.8 [0 – 100]. Plantar footprint abnormalities of the hollow and flat foot were found in 39% and 30% of cases respectively. These abnormalities were unilateral and bilateral in 7 and 9 patients respectively. Eleven patients (48%) had a hindfoot abnormality and 30% of them had a limitation of joint movement range. There was no statistically significant association between the physical domain of the Oxford score and the presence of a limited range of the talocrural joint (p> 0.05). A significant reduction in physical activity was associated with hindfoot pain (5.37 vs 89.56; p< 0.001) but was not associated with the presence of hindfoot abnormalities (p = 0.05). The presence of flat feet was significantly associated with impairment in all domains of the Oxford score, particularly in the physical domain (36.79 for the flat foot group vs 89.2 for the group without flat feet, p = 0.001). However, the presence of a hollow foot did not lead to an alteration of the physical activity of JIA patients (p> 0.05).ConclusionOur study showed that the flat foot was associated with a reduction in physical activity. Early detection of this abnormality and adapted podiatric care could improve the function of young patients with JIA.

07 Predictor factors of hip involvement in juvenile idiopathic arthritis

BackgroundJuvenile idiopathic arthritis (JIA) is one of the commonest rheumatic diseases in children. Hip involvement is a common problem in JIA patients and is associated with functional disability and poor outcomes. Intensive therapy is required to avoid joint replacement surgery. Little studied in the literature, the predictors of hip involvement are still unknown.ObjectivesOur study aims to identify the clinical, biological characteristic of patients with hip involvement and determine the associated risk factors.MethodsA cross-sectional study including children with JIA according to the International League of Associations for Rheumatology (ILAR). The recorded data included sociodemographic features, disease characteristics (subtype disease, duration, and juvenile arthritis disease activity score (JADAS)) 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).ResultsThirty-five patients (20 females) with a median age of 12 years (5–18) and disease duration of 3 years (0.25–15) were recruited. The patient's distribution of JIA subtypes were oligoarticular (n = 13), enthesitis-related arthritis (n = 9), polyarticular (n = 4) (negative rheumatoid factor in 3 patients), undifferentiated (n = 4), psoriatic-arthritis (n = 4) and systemic-onset (n = 1). ESR and CRP median values were 15 mm/h (0–63) and 2 mg/l(0–47) respectively. Sixteen patients were under DMARDs (Methotrexate (n = 10), biological agent (n = 3), biological agent and methotrexate (n = 2), salazopyrine (n = 1)).Sixteen patients (45.71%) developed coxitis (radiographic (n = 8), MRI (n = 5) and US findings (n = 3)) with eight (50%) presenting limited range of motion and 10 (62.5%) developing radiological evidence of hip damage. Hip involvement was associated with a longer disease duration (p = 0.051). JADAS score value of patients with coxitis was higher (Mean 8.35 vs 7.05) but not significantly (p = 0.565). Higher CRP and ESR values were found in patients with coxitis (mean 8.39 mg/l vs 6.83 mg/l, 22.29 mm/h vs 16.37 mm/h respectively) but not significantly (p = 0.718, p = 0.287 respectively). No associations were found between hip involvement and BMI (p = 0.233), age-onset (p = 0.496), JIA subtype (p = 0.509), nor sex (p = 0.767).ConclusionOur study shows that long disease duration exposes to a higher risk of hip involvement in children with JIA. Active disease and biological inflammatory syndrome could be associated risk factors. Studies with larger sample sizes are needed to draw definite causal associations.

05 Methotrexate improve the foot functional impairment in juvenile idiopathic arthritis

BackgroundFoot involvement in juvenile idiopathic arthritis (JIA) is common and affects about 60% of children [1]. Often overlooked by patients and practitioners, ankle and foot disability has been poorly studied and there are no accepted clinical practice guidelines for the diagnostic approach, as well as for the therapeutic management.ObjectivesOur study aimed to evaluate the effect of medical treatments on functional disability related to foot involvement in JIA.MethodsWe conducted a cross-sectional study including patients with JIA according to the revised ILAR criteria, collected from the rheumatology department of the Kassab National Institute. Sociodemographic and disease data (activity assessed by the Juvenile Arthritis Disease Activity Score 10 (JADAS-10) and therapeutic modalities) were recorded. Ankle and foot involvement were investigated by questioning and physical examination. We used the Oxford ankle foot questionnaire for children (OxAFQ-C); a validated, simple and reproducible score to assess the impact on the quality of life of children with foot problems [2]. This questionnaire encompasses 14 items corresponding to three dimensions: physical, school and play, and emotional. Higher scores represent better functioning. We searched for an association between the different drug treatments for JIA and the OxAFQ-C score.ResultsThe study included 23 patients. The mean age was 13 ± 4 [6–18] years. The sex ratio was 0.42 with a female predominance. The age of the disease was 49 ± 40 months [6–180 months]. The distribution of the different forms of JIA was as follows: oligoarticular (n = 7), enthesitis-related arthritis (n = 6), polyarticular FR + (n = 1), polyarticular FR- (n = 3), psoriatic arthritis (n = 3), systemic (n = 1) and undifferentiated (n = 1). Pain on walking and limitation of the talocrural joint was found in 39% and 30% respectively. The mean JADAS-10 score was 6.72 ± 6.1 [0–20]. Ten patients had high activity. Eighteen patients (78%) were taking level I analgesics and 14 patients (61%) were on non-steroidal anti-inflammatory drugs, 12 of them on demand. Naproxen was the most commonly used drug, followed by diclofenac. Eleven patients (48%) were on a disease-modifying anti-rheumatic drug (csDMARD). Methotrexate (MTX) was prescribed in 30% of cases with a mean dose of 7.91 mg/week [7.5–10]. Only one patient was on sulfasalazine. Four patients (17%) were treated with biologics: Etanercept (n = 3) and Tocilizumab (n = 1). The mean scores of the different domains of (OxAFQ-C) were as follows: 73.52 ± 35.89 [0—100] in the physical domain, 84.2 ± 30 [6.25—100] in the school and play domain, 88.75 ± 24.71 [12.5 – 100] in the emotional domain. There was no statistically significant association between the different domains of the Oxford score and the use of analgesics or NSAIDs (p> 0.05). Similarly, there was no statistically significant association between the different domains of the Oxford score and treatment with biotherapy (p> 0.05). However, patients on MTX had less functional impairment of the feet with a significant improvement in the physical domain (99.26 on MTX vs 62.5 without MTX, p = 0.02, r = 0.6).ConclusionOur work showed that only methotrexate was associated with an improvement in functional foot outcomes in JIA. Further studies are needed to highlight the effect of other therapies, especially biologics.

12 Hip involvement in juvenile idiopathic arthritis: frequency and associated factors

BackgroundJuvenile idiopathic arthritis (JIA) is a pediatric rheumatic disease with several subgroups. The hip is frequently affected. The frequency of this involvement can reach 50% especially in the severe and destructive forms. Arthroplasty may be indicated in advanced cases.ObjectivesTo assess the frequency and associated factors with hip involvement in (JIA).MethodsWe conducted a retrospective study including adults with long-standing JIA according to the International League of Associations for Rheumatology (ILAR) criteria over a period of 28 years (1994–2022). Demographic, clinical, biological, and radiographic data were collected. These parameters were compared according to the presence or absence of hip involvement.ResultsA total of 29 Patients were enrolled (12 men and 17 women), the mean age was 35.69 ± 11.72 [18–61] years. The mean age of disease onset was 11.10 ± 4.25 [2–16] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months. The average disease duration was 24.48 ± 12.76 [1–47] years.Sixteen patients had a polyarticular form. Mean CRP values were 42.74 ± 63.37 [2–218] mg/l, a biological inflammatory syndrome was present in 19 cases. Rheumatoid factor, ACPA and anti-nuclear antibodies were observed in 12, 7 and 5 cases respectively. At least one extra-articular manifestation was noted in 16 cases.Hip involvement was noted in 14 patients (48.3%). It was bilateral in 64.3% of cases (n = 9). Twenty-three hips were affected in total (56.9%). Hip involvement was diagnosed 14.5 ± 9.37 [1–28] years after disease onset. Arthroplasty was performed on 10 hips with a delay of 201.60 ± 104.75 [108–348] months between diagnosis of JIA and surgery.Hip involvement was associated with male gender in our study (75% vs 29.4%; p = 0.016). On the other hand, our study showed that age, age at onset, diagnostic delay, symptoms duration, smoking, BMI, extra-articular manifestations, CRP, rheumatoid factor, antinuclear antibodies, ACPA and erosive character were not associated with hip involvement.ConclusionOur study showed that hip involvement is common in JIA, mainly in male patients. It usually occurs late in the disease course. Since hip involvement is a cause of disability and functional impairment, it should be assessed regularly.

23 Predictive factors of hip involvement in juvenile idiopathic arthritis

Abstract Background Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Hip involvement is a common problem in JIA patients and is associated with functional disability and poor outcomes. Intensive therapy is required to avoid replacement surgery. With few studies in the literature, the predictive factors of hip involvement are still unknown. Objectives To identify the clinical, biological characteristic of patients with hip involvement and determine the risk factors associated. Methods A cross-sectional study including children with JIA according to the International League of Associations for Rheumatology (ILAR). The recorded data included sociodemographic features, disease characteristics (subtype disease, duration, and juvenile arthritis disease activity score (JADAS) 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 Thirty-five patients (20 females) with a median age of 12 years (5–18) and disease duration 3 years (0.25–15) were recruited. The patient's distribution of JIA subtypes were oligoarticular (n = 13), enthesitis-related arthritis (n = 9), polyarticular (n = 4) (negative rheumatoid factor in 3 patients), undifferentiated (n = 4), psoriasis-arthritis (n = 4) and systemic-onset (n = 1). ESR and CRP median values were 15 mm/h (0–63) and 2 mg/l (0–47); respectively. DMARDS was admitted to 16 patients Sixteen patients [Methotrexate (n = 10), biological agent (n = 3), biological agent and methotrexate (n = 2), salazopyrine (n = 1)]. Sixteen patients (45.71%) developed coxitis (radiographic (n = 8), MRI (n = 5) and US findings (n = 3)) with limited range of movement in eight (50%) and radiological evidence of hip damage in 10 (62.5%). Hip involvement was associated with a longer disease duration (p = 0.051). JADAS of patients with coxitis was higher (Mean 8.35 vs 7.05) but not significant (p = 0.565). Higher CRP and ESR values were found in patients with coxitis (mean 8.39 mg/l vs 6.83 mg/l, 22.29 mm/h vs 16.37 mm/h respectively) but not significant (p = 0.718, p = 0.287 respectively). No associations were found between hip involvement and BMI (p = 0.233), age-onset (p = 0.496), JIA subtype (p = 0.509) and sex (p = 0.767). Conclusion Our study shows that long disease duration exposes to a higher risk of hip involvement in children with JIA. Active disease and biological inflammatory syndrome could be associated risk factors. Studies with larger sample size are needed to draw definite causal associations.

Role of Ultrasound Evaluation of Temporomandibular Joint in Juvenile Idiopathic Arthritis: A Systematic Review.

Background: Juvenile idiopathic arthritis (JIA) is childhood’s most frequent chronic rheumatic disease. JIA is a broad term that includes all arthritides starting before 16 years, lasting at least six weeks, and of unknown cause. The temporomandibular joint (TMJ) could be involved in JIA both at onset and during the disease course. The presence of TMJ synovitis might severely impair dentofacial maturation in pediatric patients. The ultrasound (US) application to detect early signs of TMJ synovitis in children with JIA has provided contradictory results. We sought to assess the current role of TMJ US in JIA through a systematic literature review. Methods: The systematic review was conducted according to the recommendations of the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA). Results: The literature search found 345 records. After duplicates removal, 253 records were screened, 20 full-text articles were reviewed to assess their eligibility, and 7 of them were included in the qualitative analysis. Joint effusion was the most recorded parameter, followed by bony condylar abnormalities. Compared to contrast enhancement MRI, the capability to detect signs of active synovitis of TMJ by US is low, especially at the early stages. Conclusion: Understanding how US may help diagnose and manage children with JIA is advisable for several reasons. MRI cannot be frequently repeated, may need sedation, and is expensive. The constant technical improvement of US will undoubtedly allow for better evaluation of what, in the past, was not clear or not even captured by sonography. So far, the role of US in the assessment of TMJ involvement in JIA is indubitably secondary to the MRI. Even so, we think that a baseline MRI of TMJ and the repetition of the sonography over time might both help the interpretation of US images and intercept significative changes.

Electromyographic Activity of Masticatory Muscles in Subjects with Juvenile Idiopathic Arthritis: A Case—Control Study

The aim of this study was to evaluate the effect of juvenile idiopathic arthritis (JIA) on the activity of masticatory muscles using surface electromyography (sEMG). Forty-one JIA subjects (ten males, thirty-one females; average age 13 years ± 3) and thirty-two healthy control subjects (twenty-seven females, five males; average age 14 years ± 2) were recruited. sEMG of anterior temporalis (TA), masseter (MM), and sternocleidomastoid (SCM) muscles was performed by using the occlusal contact analyzer software called Teethan (BTS S.p.A., Garbagnate Milanese, Milan, Italy). Comparisons between groups were assessed with unpaired t-tests for non-normally distributed data and with Mann–Whitney U tests for normally distributed parameters. The JIA group showed a significant increased percentage overlapping coefficient of TA (POC TA) (p = 0.01) and impact index (IMP) (p = 0.003). No significant differences were observed for the POC MM, POC SCM, percentage overlapping coefficient between posterior and anterior teeth contact (BAR), the torsion index (TORS), and the asymmetry index (ASIM). Masticatory muscles seemed to be slightly affected by JIA. sEMG could be an effective aid in the early clinical detection of TMJ involvement in JIA. Further research is needed to confirm its validity.

Hyaluronic Acid Therapy in Hip OA Does Not Perform Equally in Osteoarthritis Secondary to Juvenile Idiopathic Arthritis When Compared to Primary Osteoarthritis: A 2-Year Preliminary Evaluation.

Hip involvement in juvenile idiopathic arthritis (JIA) is one of most important causes of pain and disability. Total hip arthroplasty (THA) is considered the standard when medical approaches fail to relieve pain. However, THA is problematic for many reasons. As current literature lacks studies valuating medical management of osteoarthritis (OA) secondary to JIA, we assessed the long-term pain relief effect of US-guided intra-articular viscosupplementation in hip osteoarthritis secondary to JIA versus primary OA under different etiological conditions. Patients in both groups received intra-articular Hylan G-F 20 2ml once a month for 3 consecutive months and every 6 months for 2years as maintenance. Effectiveness (VAS and WOMAC), NSAID/analgesic consumption, tolerability, withdrawals and reason for discontinuation were collected at each time point. An inverse probability weighting was used to balance the two groups. We retrospectively retrieved data of 14 JIA patients and 26 primary OA. Weighting successfully accounted for differences between the disease groups supporting the results. Viscosupplementation led to an early and significant improvement of pain and function and concomitant decrease in NSAIDs consumption, while the response diverged over 1 year with loss of benefits in JIA. The worst outcome was observed in active JIA. Duration of symptom relief after intra-articular injection of hyaluronic acid depends on the nature of arthritis. Multiple courses of viscosupplementation are required to maintain low-dose NSAIDs consumption in patients responsive to treatment while shortening the time between consecutive injections might provide persistent positive results in patients suffering from JIA.