Outcome Of Juvenile Idiopathic Arthritis Research Articles

Effect of joint hypermobility on outcomes of children with juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is common in pediatric rheumatology. Despite treatment, many patients experience persistent disease activity. Joint hypermobility (JH), defined by an excessive range of motion across multiple joints, is prevalent in children and adolescents and may influence disease outcomes in JIA. This study examines the impact of JH on symptoms in youth and young adults with JIA. Data were obtained from the PR-COIN network and included patients under 21years old with a diagnosis of JIA. Patients with JIA and JH were matched with those having JIA-only based on age, sex assigned at birth, JIA subtype, and medication exposure. Clinical data, including disease activity measures, patient well-being, and pain ratings, were collected at baseline and follow-up visits. The sample included 420 patients with JIA + JH and 2100 with JIA only. The JIA + JH group exhibited higher disease activity at baseline, more active arthritis joints, elevated physician global assessment of disease activity scores, and worse patient-reported well-being. These differences persisted over time. The JIA + JH group had a 19-20% greater likelihood of maintaining high disease activity scores and worsening over subsequent visits, indicating a significant impact of JH on disease progression. JH in youth with JIA is associated with higher and persistent disease activity, suggesting that JH significantly contributes to the disease burden in patients with JIA and should be considered in treatment strategies. Future research should further explore the mechanisms by which JH influences disease activity and investigate comprehensive management approaches to improve outcomes for this population. Key Points • Children with JIA and joint hypermobility (JH) exhibit significantly higher disease activity at baseline compared to those with JIA only, including more active arthritis joints and elevated physician global assessment scores. • The presence of JH in JIA patients is associated with poorer patient-reported well-being and higher overall disease activity scores, which persist over time despite treatment. • JIA + JH patients have a 19-20% greater likelihood of maintaining high disease activity and worsening over subsequent visits, indicating a significant impact of JH on disease progression. • The study suggests that JH should be considered an important clinical factor in the management of JIA, with targeted interventions needed to address the increased disease activity and improve overall patient outcomes.

Preterm birth, preeclampsia, gestational hypertension and offspring birth weight in women with active juvenile idiopathic arthritis and healthy controls

ObjectivesThere is insufficient knowledge about pregnancy outcomes in women with juvenile idiopathic arthritis (JIA). Our objective was to explore a possible association of inflammatory active JIA and pregnancy outcomes, including preterm birth, preeclampsia, gestational hypertension, and offspring gestational weight.MethodsWe linked data from the Norwegian nationwide observational register RevNatus with data from the Medical Birth Registry of Norway (MBRN) for the period 2010 to 2019. Singleton births in women with JIA (n = 181) included in RevNatus were cases. After excluding births in mothers with rheumatic inflammatory diseases, the remaining singleton births registered in MBRN, served as population controls (n = 575 798).ResultsPreterm birth was more frequent in women with active JIA (17.6%) and of equivalent frequency in women with inactive JIA (3.1%), compared to population controls (4.9%). Preeclampsia had similar rates in women with JIA and population controls while gestational hypertension was more frequent in women with active JIA (7.2%) and inactive JIA (6.9%) compared to population controls (1.7%). Abnormal fetal growth occurred in similar rates in women with JIA and population controls.ConclusionHaving active JIA in pregnancy increased the risk for preterm birth (risk difference 12.7, 95% CI 4.7 to 25.3) and gestational hypertension (risk difference 6.2, 95% CI 1.4 to 16.8). There was no increased risk for preeclampsia or abnormal fetal growth compared to population controls.

Body mass index is associated with health-related quality of life and disease characteristics in young adults with juvenile idiopathic arthritis

BackgroundThere is a growing interest concerning the relationship between obesity and several medical conditions and inflammation. Nevertheless, there is a lack of studies regarding body mass index (BMI) among patients with juvenile idiopathic arthritis (JIA). Our aim was to investigate the impact of BMI on health-related quality of life (HRQoL) measured with a 36-Item Short Form Survey (SF-36), disease activity, and disability in young adults with JIA.MethodsThis study is a part of the population-based Nordic JIA cohort study. All newly diagnosed patients with JIA were recruited consecutively between 1997–2000 in specific regions in the Nordic countries. Patients in this sub-study were enrolled from 434 patients who attended their 18-year follow-up visit. Patients were classified according to the World Health Organization (WHO) into four groups based on their BMI. HRQoL, disease characteristics, disability, fatigue, sleep quality, physical activity, pain, comorbidities, and social status were assessed.ResultsThree hundred fifty-five patients from the original study cohort were enrolled in this study and 72% of them were female. Mean age was 23.9 (± SD 4.4) years. A significant relationship was found between the JIA categories and BMI groups (p = 0.014). A significant relationship was also found between BMI and disease activity scores (DAS28) (p = 0.028), disability (p < 0.001), pain (p = 0.013), fatigue (p = 0.035), and sleep quality (p = 0.044). Moreover, a significant relationship between BMI and HRQoL regarding bodily pain (p = 0.010) and general health (p = 0.048) was revealed when adjusted for sex, age, and JIA subtype.ConclusionWe discovered that BMI was significantly related to HRQoL, disease activity, and disability. BMI deserves more attention considering the treatment options and outcome of JIA in young adults.

Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. The International League of Associations for Rheumatology (ILAR) has defined JIA as "arthritis of unknown etiology persisting for ≥6 wk with an onset at <16 y of age, after excluding other causes of joint inflammation". Synovial inflammation is the result of a complex interplay of aberrant immune systems (both adaptive and innate) in a genetically susceptible individual, with possible external stimuli/triggers. Diagnosis of JIA essentially remains clinical, and laboratory investigations usually help to assess the severity of disease activity. Few investigations like antinuclear antibodies (ANA), human leukocyte antigen (HLA)-B27, and rheumatoid factor (RF) help to categorize or prognosticate a child with JIA. Timely use of effective therapeutic interventions including biological has shown good long-term outcomes of JIA.

Diagnostic and prognostic role of synovial tissue analysis in juvenile idiopathic arthritis: a monocentric study

ObjectivesThis study investigates the diagnostic role of synovial tissue analysis in children presenting with arthritis and assesses its prognostic significance to predict clinical outcome in juvenile idiopathic arthritis (JIA).MethodsSynovial samples...

Current Validated Clinical and Patient Reported Disease Outcome Measures in Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA) is a common chronic illness in childhood and comprises seven categories based on the International League of Associations for Rheumatology (ILAR) criteria. Accurate assessment and measurement of the clinical, functional, and quality of life outcomes of patients with JIA are paramount for understanding the disease course and formulating effective treatment strategies. Interest in the development and use of outcome measurements specifically focused on rheumatologic conditions has greatly expanded over the last two decades, adding to and improving upon the established disease measures. Furthermore, many of these measures have been validated using the widely accepted Outcome Measures in Rheumatology (OMERACT) core principles of instrument validation, allowing researchers and clinicians to gain confidence in these tools. This review summarizes the current validated disease outcome measures in JIA, including clinical, imaging, patient-reported, and functional outcome measurement tools, and highlights ongoing work that continues to refine and improve upon the available tools. The clinical disease outcome measures discussed in this review include physician global assessment (PhGA), American College of Rheumatology (ACR, Wallace) criteria for clinical inactive disease and clinical remission, juvenile arthritis disease activity scores (JADAS), juvenile spondyloarthritis disease activity index (JSPaDA), juvenile arthritis damage index (JADAI), and the ACR pediatric response scores. The imaging outcome measures discussed include the Dijkstra composite scores, childhood arthritis radiographic score of the hip (CARSH), and Poznanski Score. The patient-reported disease outcome measures discussed include patient global assessment (PtGA), patient-reported outcome measurement information system for JIA (PROMIS), juvenile arthritis parent/child centered disease assessment index (JAPAI, JACAI), juvenile arthritis multidimensional assessment report (JAMAR), and the Pediatric quality of life inventory rheumatology module (PedsQL). The functional outcome tools discussed include the Childhood Health Assessment Questionnaire (CHAQ), juvenile arthritis functionality scale and index (JAFS and JASI), and Juvenile Arthritis Functional Assessment Report and Scale (JAFAS and JAFAR).

Survey of adolescents’ needs and parents’ views on sexual health in juvenile idiopathic arthritis

BackgroundAlthough the advent of new therapeutics for juvenile idiopathic arthritis (JIA) patients has considerably lessened the impact of the disease and reduced its sequelae, the outcomes of JIA remain important in their lives. Disease repercussions and side effects of treatments may affect sexual health and cause psychological distress. This aim of the study was to determine the expectations of adolescent JIA patients and the perceptions of their parents regarding knowledge and communication with healthcare providers (HCPs) in the field of sexual health (SH).MethodsIn France, from September 2021 to April 2022, a survey was conducted, using anonymous self-administered questionnaires, among JIA patients (adults (aged 18–45 years) to provide insights from their recollection of their adolescence) and their parents in nine rheumatology centers and three patient associations.ResultsThe responses to the 76 patient questionnaires and 43 parent questionnaires that were collected were analyzed. Half of the patients thought JIA impacted their romantic relationships, but the results were less clear-cut for their sexual activity; and 58.7% of the patients said they would be comfortable discussing the subject with HCPs, but only 26.3% had done so, mainly regarding biomedical issues. The patients and their parents thought that ideally, the topic should be addressed in an individual patient education session at the hospital (51.3% and 34.9%, respectively), in a regular consultation (47.4% and 53.5%), or in a dedicated consultation requested by the adolescent without the adolescent’s parents being informed (38.2% and 20.9%). Most of the respondents thought HCPs should be proactive in SH (77.6% of the patients and 69.8% of their parents). More patients than parents said the following digital information tools must be used: videos (29.0% vs. 9.3%, p = 0.0127) and smartphone applications (25.0% vs. 9.3%, p = 0.0372).ConclusionHCPs should consider addressing the unmet need for SH discussions during their patient encounters. To meet this need, we propose concrete actions in line with the wishes of patients and parents.Clinical trial registration numberNCT04791189.

Presence of uveitis as indicator of juvenile idiopathic arthritis severity: results of a retrospective cohort study

BACKGROUND: Juvenile idiopathic arthritis is the most common rheumatic disease in children. A frequent extra-articular manifestation of juvenile idiopathic arthritis is uveitis, which is a serious clinical diagnostic problem in routine pediatric practice. Among the known risk factors for uveitis are the early age of the juvenile idiopathic arthritis onset, oligoarticular subtype, seropositivity by antinuclear factor.&#x0D; AIM: to evaluate the influence of presence of uveitis on the course of juvenile idiopathic arthritis.&#x0D; MATERIALS AND METHODS: A single-center retrospective study included 520 patients with uveitis. The analysis was carried out among patients who developed (n = 116) and did not develop (n = 404) uveitis. The minimum follow-up period was 2 years, for patients who did not develop uveitis.&#x0D; RESULTS: Uveitis was diagnosed in 116 (22.3%) children with juvenile idiopathic arthritis. Most often, uveitis occurred in patients with oligoarthritis and psoriatic arthritis. When comparing the features of the articular status of patients with juvenile idiopathic arthritis who developed and did not develop uveitis, a lower frequency of involvement of the cervical spine, temporomandibular, shoulder, elbow, wrist, proximal and distal interphalangeal joints, hip, talus-heel joint, as well as a smaller number of active joints in children with uveitis was found. Patients with uveitis received methotrexate therapy more often, cumulative doses of corticosteroids were lower, the frequency of prescribing genetically engineered biological drugs was approximately the same in both groups. Remission of arthritis was achieved more often, but the proportion of children who developed an exacerbation was higher. When calculating the risk factors of uveitis by binary logistic regression, it was found that the main predictors of uveitis were oligoarthritis, the number of active joints 8, seropositivity by antinuclear factor and recurrent course of arthritis. The difference in the frequency of achieving remission may be due to more aggressive systemic therapy in the presence of uveitis.&#x0D; CONCLUSIONS: Children with uveitis have a more severe course of juvenile idiopathic arthritis and may require more aggressive immuno-suppressive therapy. Further studies are required to determine the prognostic role of uveitis in the course and outcomes of juvenile idiopathic arthritis.

Musculoskeletal Ultrasound and the Assessment of Disease Activity in Juvenile Idiopathic Arthritis.

To determine the frequency of subclinical synovitis on musculoskeletal ultrasonography (MSUS) in juvenile idiopathic arthritis (JIA) and correlate patient- and provider-reported outcome measures with MSUS synovitis. JIA patients with an active joint count (AJC) of >4 underwent a 42-joint MSUS performed at baseline and 3 months. B-mode and power Doppler images were obtained and scored (range 0-3) for each of the 42 joints. Outcomes evaluated included physician global assessment of disease activity (PhGA), patient global assessment of disease activity (PtGA), patient pain, Childhood Health Assessment Questionnaire (C-HAQ), and AJC. Subclinical synovitis was defined as synovitis detected by MSUS only. Generalized estimation equations were used to test the relationship between clinical arthritis (positive/negative) and subclinical synovitis (positive/negative). Spearman's correlation coefficients (rs ) were calculated to determine the association between MSUS synovitis and patient- and physician-reported outcomes. In 30 patients, subclinical synovitis was detected in 30% of joints. Clinical arthritis of the fingers, wrists, and knee joints was significantly associated with MSUS synovitis in these joints. PtGA and the C-HAQ had a moderate (rs =0.44, P= 0.014) to weak (rs =0.37, P=0.045) correlation with MSUS synovitis. There was a statistically significant strong correlation between MSUS synovitis and PhGA (rs =0.61, P=0.001), but a weak correlation with AJC (rs =0.37, P=0.048) at the follow-up visit. Subclinical synovitis was commonly observed in this cohort of JIA patients. The fair-to-moderate correlation of MSUS synovitis with patient- and provider-reported outcomes suggests that MSUS assesses a different, possibly more objective, domain not determined by traditional JIA outcome measurements.

Nutritional Aspects of Juvenile Idiopathic Arthritis: An A to Z for Dietitians.

Juvenile idiopathic arthritis (JIA) represents a chronic, autoimmune, rheumatic musculoskeletal disease with a diagnosis before 16 years of age. Chronic arthritis is a common manifestation in all JIA subtypes. The nature of JIA, in combination to its therapy often results in the development of nutrition-, gastrointestinal (GI)- or metabolic-related issues. The most-common therapy-related nutritional issues involve methotrexate (MTX) and glucocorticosteroids (GCC) adverse events. MTX is a folic acid antagonist, thus supplementation with folic acid in required for improving GI side effects and correcting low serum levels. On the other hand, long-term GCC administration is often associated with hyperglycemia, insulin resistance and growth delay. This relationship is further aggravated when more joints are affected and greater doses of GCC are being administered. Apart from stature, body mass index z-scores are also suboptimal in JIA. Other signs of malnutrition include decreased phase angle and muscle mass, especially among patients with polyarthritis JIA. Evidence also points to the existence of an inverse relationship between disease activity and overweight/obesity. Specific dietary patterns, including the anti-inflammatory diet, might confer improvements in selected JIA outcomes, but the level of available research is yet insufficient to draw safe conclusions. The majority of patients exhibit suboptimal vitamin D status; hence, supplementation is recommended. Collectively, the evidence indicates that, due to the age of onset and the complexity of the disease, along with its pharmacotherapy, children with JIA are prone to the development of several nutritional problems, warranting expert monitoring. Vitamin deficiencies, oral and GI-problems limiting dietary intake, faltering growth, overweight and obesity, physical inactivity, or impaired bone health are among the many nutritional issues in JIA requiring dietitian support.

Epidemiology, Treatment, and Outcomes of Juvenile Idiopathic Arthritis-Associated Uveitis: A Multi-Institutional Study in Taiwan

ABSTRACT Purpose To assess the epidemiology, treatment, and outcomes of juvenile idiopathic arthritis (JIA)–associated uveitis (JIA-U) in Taiwan. Methods Retrospective, multicenter database. Results Totally, 44 (6.1%) of the 722 JIA patients had uveitis. The mean ages of JIA and JIA-U diagnosis were 10.7 and 11.1 years, respectively. JIA-U patients had more antinuclear antibody presence. Among JIA-U patients, 25 (56.8%) were male, 11 (25.0%) experienced any ocular complication, and 8 (18.2%) received ocular surgery. Inactivity lasting ≥6 months was achieved in 17 (38.6%) patients; however, 11 (25.0%) of those experienced reactivation with a mean of 624.7 days after achieving inactivity. Female had more ocular complications and surgeries. Conclusion Late age at JIA-U diagnosis and male predominance were distinctive in Taiwan. For patients with inactivity lasting ≥ 6 months was achieved, they still required close follow-up due to the high reactivation rate within 2 years. Female had poorer ocular outcomes.

Dosing Variation at Initiation of Adalimumab and Etanercept and Clinical Outcomes in Juvenile Idiopathic Arthritis: A Childhood Arthritis and Rheumatology Research Alliance Registry Study.

To determine the dose-response relationship of tumor necrosis factor (TNF) inhibition in the treatment of juvenile idiopathic arthritis (JIA). Participants of the Childhood Arthritis and Rheumatology Research Alliance Registry were eligible for inclusion in the analyses if they started TNF inhibition treatment for JIA. The primary treatment response was determined 3 to 7 months after the start of treatment, based on the JIA American College of Rheumatology Pediatric criteria for improvement, clinical Juvenile Arthritis Disease Activity Score, and persistence of treatment after 6 months. Subsequently, pooled logistic regression models were performed to include long-term follow-up data. The models were adjusted for risk factors associated with poor treatment response. Dosing was expressed by body weight, body surface area, ideal body weight, fat free mass, and lean body mass. Participants treated with adalimumab (n=328) and etanercept (n=437) were included in the analyses (median dose 0.82 mg/kg body weight [interquartile range (IQR) 0.66-1.04] and 0.83 mg/kg body weight [IQR 0.75-0.95], respectively). The majority of analyses did not show a relationship between dose and outcome. Where associations were found, results were conflicting. Alternative dosing characteristics based on ideal body weight, fat free mass, and lean body mass did not result in stronger or more consistent associations. This study was not able to confirm our hypothesis that increased dosing of TNF inhibitors results in improved treatment outcomes. Although adjustment was performed for risk factors of impaired treatment response, residual confounding by indication likely explains the negative associations found in this study.

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.

Vitamin D Supplementation in Patients with Juvenile Idiopathic Arthritis.

Vitamin D has been implicated in the pathogenesis of skeletal disorders and various autoimmune disorders. Vitamin D can be consumed from the diet or synthesized in the skin upon ultraviolet exposure and hydroxylation in the liver and kidneys. In its bioactive form, vitamin D exerts a potent immunomodulatory effect and is important for bone health. Juvenile idiopathic arthritis (JIA) is a collection of inflammatory joint diseases in children that share the manifestation of inflamed synovium, which can result in growth arrest, articular deformity, bone density loss, and disability. To evaluate the potential effect of vitamin D on JIA disease manifestations and outcomes, we review the role of vitamin D in bone metabolism, discuss the mechanism of vitamin D in modulating the innate and adaptive immune systems, evaluate the clinical significance of vitamin D in patients with JIA, and summarize the supplementation studies.

Examining Health Outcomes in Juvenile Idiopathic Arthritis: A Genetic Epidemiology Study.

ObjectiveJuvenile idiopathic arthritis (JIA) is the most common pediatric rheumatic disease; however, little is known about its wider health impacts. This study explores health outcomes associated with JIA genetic liability.MethodsWe used publicly available genetic data sets to interrogate the genetic correlation between JIA and 832 other health‐related traits using linkage disequilibrium score regression. Two‐sample Mendelian randomization (2SMR) was used to examine four genetic correlates for evidence of causality.ResultsWe found robust evidence (adjusted P [P adj] < 0.05) of genetic correlation between JIA and rheumatoid arthritis (genetic correlation [r g] = 0.63, P adj = 0.029), hypothyroidism/myxedema (r g = 0.61, P adj = 0.041), celiac disease (CD) (r g = 0.58, P adj = 0.032), systemic lupus erythematosus (r g = 0.40, P adj = 0.032), coronary artery disease (CAD) (r g = 0.42, P adj = 0.006), number of noncancer illnesses (r g = 0.42, P adj = 0.016), paternal health (r g = 0.57, P adj = 0.032), and strenuous sports (r g = −0.52, P adj = 0.032). 2SMR analyses found robust evidence that genetic liability to JIA was causally associated with the number of noncancer illnesses reported by UK Biobank (UKBB) participants (increase of 0.03 noncancer illnesses per doubling odds of JIA, 95% confidence interval 0.01‐0.05).ConclusionThis study illustrates genetic sharing between JIA and a diversity of health outcomes. The causal association between genetic liability to JIA and noncancer illnesses suggests a need for broader health assessments of patients with JIA to reduce their potential comorbid burden. The strength of genetic correlation with hypothyroidism and CD implies that patients with JIA may benefit from CD and thyroid function screening. Strong positive genetic correlation between JIA and CAD supports the need for cardiovascular risk assessment and risk factor modification.

Pattern, frequency and management outcome of juvenile idiopathic arthritis, Sudan.

Juvenile idiopathic arthritis (JIA) is the most prevalent chronic arthritis in children and is a leading cause of disability. The clinical profile of JIA shows wide range of variation .This study aimed to describe demographic, clinical and laboratory characteristics, treatment regimen and outcome of JIA patients in Sudanese children. Cross sectional hospital based study was conducted at Rheumatology Clinic, Ahmed Gasim Hospital, Khartoum, Sudan, covering 81 patients from October 2019 to March 2020. Data were extracted from the patient records, and then re-entered into a predesigned data collection form. Data were analyzed using SPSS version 24. P value of 0.05 or less was considered statistically significant. A total of 81 JIA patients were included in the study (54 females, 27 males) with female: male ratio 2:1 among all subtypes. Of the study population 58% were above 10 years of age. Polyarticular JIA was the predominant subtype (63%) followed by systemic onset JIA (22.2%), then oligoarticular (12.3%) and lastly spondiloarthropathies (2.5%). Clinically, morning stiffness was the most common presenting symptom (98.8%) and knee joint (82.7%) was the most frequently affected. Rheumatoid factor and anti-nuclear antibody were positive among 30.9% and 46.9% of the study participant, respectively. Remission rate was 39.5% and it is affected by RF and treatment regimen, being RF positive associated with lower remission rate while use of combined two drug therapy was associated with higher remission rate. A population based study rather than a single center study will provide more information about JIA characteristics in Sudan.

P010 Clinico-epidemiological characteristics and outcomes of Juvenile Idiopathic Arthritis (JIA) patients in Kenya: data from the KAPRI registry

Abstract Background Pediatric rheumatic diseases pose a significant disease burden upon children and their families (1–3). They lead to physical disability and diminished quality of life (1–3). Determination of the burden and clinical characteristics of these diseases is a critical first step to improving access to care and optimizing use of existing health systems for the well-being of these patients (4). This is in line with the goal of the Bone and Joint decade (2000–2010) aimed at increasing recognition and understanding of the impact of musculoskeletal diseases (5,6). The Kenya Pediatric Rheumatology Registry (KAPRI) offers a unique opportunity to pioneer and spearhead a systematic and organized format to inform policy and better healthcare provision. Our objective was to determine the patient characteristics, clinical features and outcomes of Juvenile Idiopathic Arthritis (JIA) patients assessed at the Aga Khan University Medical College East Africa from March 2019 to December 2020. Methods Data of JIA patients on age, gender, laboratory and clinical features at diagnosis and treatment options offered were extracted from the database. A further detailed chart review was undertaken to determine the proportion of patients who achieved remission or minimally active diseases. Results Among the 207 patients enrolled thus far, 16 (7.7%) were diagnosed to have JIA. Majority of the patients were females (75%; n = 12) with a mean age of 7 years and 3 months (Range : 1 year—13 years 7 months). All patients had joint pain and swelling as the initial presenting complaints. Majority of the patients had polyarticular JIA (75%, n = 12). The other 4 patients were oligoarticular (n = 2) and systemic JIA (n = 2). Among the polyarticular JIA patients (n = 12), only 3 (25%) were rheumatoid factor (RF) positive and 1 was antinuclear antibody (ANA) positive. The oligoarticular and systemic JIA patients were all negative for antinuclear antibody, rheumatoid factor and cyclic citrullinated peptide antibodies (anti-ccp). Seven patients (43.8%) required biological therapies; tocilizumab (n = 2: systemic JIA), adalimumab (n = 2: polyarticular JIA), etanercept (n = 2: polyarticular JIA) andtofacitnib (n = 1: polyarticular JIA). One patient with systemic JIA on tocilizumab developed herpes simplex which was successfully managed with oral acyclovir. All the other patients did not develop any infections, allergic reactions or any other untoward events as adverse outcomes following the use of biological therapies. Five patients have attained remission as illustrated in the table below. Two patients have been lost to follow up. Conclusion Seronegative polyarticular JIA was the predominant form of JIA observed with a predilection to affect more girls and boys. Over a period of 2 years, remission has been attained among 31.25% of the patients (5 of 16) with use of synthetic disease modifying anti-rheumatic drugs and biological therapies.

P033 Impact of breastfeeding on Juvenile Idiopathic Arthritis outcomes

Abstract Background Juvenile idiopathic arthritis (JIA) is the most common, chronic rheumatic disease of childhood. The aetiology is still unclear. Over the past few years, several studies have investigated the association between JIA and environmental exposures such as infections and parental smoking. However, data concerning the influence of past breastfeeding on the presentation and the severity of JIA remain scarce and still a matter of debate (1, 2).The aim of this study was to determine the influence of breastfeeding history on the pattern and severity of JIA. Methods A cross-sectional monocentric study was carried out on JIA patients according to International League of Associations for Rheumatology (ILAR). Demographic, clinical and biological data were collected from medical records. Breastfeeding data were obtained by interviewing mothers. Patients were divided into two groups: group 1 (G1) stands for JIA patients breastfed over 6 months and group 2 (G2) stands for JIA non-breastfed patients or patients breastfed &amp;lt;6 months. Data were analyzed using the SPSS statistical package. A p-value &amp;lt; 0.05 was considered significant. Results Twenty-nine patients (10 male and 19 female) were enrolled. Mean age at disease onset was 9.1 ± 3.4 years [2–15]. Median diagnosis delay from symptoms onset was 3.5 ± 21. 1 SD months [1–72]. Median disease duration was 36 ± 79 SD months [7–336]. JIA subtypes were in decreasing order of frequency: enthesitisenthesitis-related arthritis (n = 13), oligoarticular (n = 8), Polyarticular RF- (n = 4), Polyarticular RF + (n = 2), systemic (n = 1) and psoriatic arthritis (n = 1). Inflammatory markers were raised in 48.3% (n = 14) of cases. Median ESR (raised in 41.3% of the cases) and CRP (raised in 34.4% of the cases) were 17 ± 25.1 SD mm/h [3–98] and 3 ± 18 SD mg/l [0–56]. Among patients with enthesitis-related arthritis, median BASDAI and BASFI scores were 1.8 ± 1.6 SD [0.3–6.3] and 2.5 ± 1.7 SD [0–6] respectively. Only 16 (55.2%) patients had bone densitometry. Osteoporosis and osteopenia were found in 10.3% and 13.8% of cases, respectively. Coxitis was found in 65.5% of patients (n = 19). Overall, 82.8% of children (n = 24) were breastfed. Breastfeeding was exclusive in 55.2% of cases (n = 16). Median breastfeeding duration was 12 ± 10.3 SD months [1.5–48]. Over two-thirds (n = 19) of patients were breastfed for &amp;gt;6 months. The statistical study revealed that G1 had significantly lesser occurrence of coxitis than G2 (57.9% vs 80%; P = 0.032). Age at disease onset (P = 0.26), diagnosis delay (P = 0.9), raised inflammatory markers (P = 0.76) and the frequency of osteoporosis (P = 0.87) were comparable between the two groups. No correlation was noted between breastfeeding duration and the following parameters: age at disease onset, ESR rate, CRP rate, BASDAI and BASFI. Conclusions In this study, JIA patients breastfed over 6 months had fewer coxitis occurrence. Further investigations with larger sample sizes are still required to assess the benefits of breastfeeding on JIA outcomes.

Familial aggregation of juvenile idiopathic arthritis with other autoimmune diseases: Impact on clinical characteristics, disease activity status and disease damage.

To evaluate the impact of family history of autoimmune diseases (FHADs) on the clinical characteristics and outcome of juvenile idiopathic arthritis (JIA). We retrospectively reviewed children with JIA seen in 7 pediatric rheumatology clinics from 6 Arab countries. All included patients met the International League of Associations for Rheumatology classification criteria for JIA and had a disease duration greater than 1 year. Data were collected at the last follow-up visit and comprised clinical findings, including FHADs. Disease activity and disease damage were assessed by Juvenile Arthritis Multidimensional Assessment Report, and juvenile arthritis damage index (JADI) respectively. Disease activity was categorized as remission off treatment, remission on treatment, or active disease. A total of 349 (224 females) JIA patients with a disease duration of 5 (interquartile range 2.9-7.5) years were included. The most frequent JIA categories were polyarticular JIA and oligoarticular JIA, followed by systemic JIA. There were 189 patients with FHADs and 160 patients without FHADs. The most frequent FHADs were diabetes mellitus (21.2%), JIA (18.5%), rheumatoid arthritis (12.7%). Among patients with FHADs, 140/189 (74.1%) achieved clinical remission, while 131/160 (81.9%) patients without FHDs had clinical remission (odds ratio [OR]=1.2, 95% CI 0.97-1.5). Rate of consanguinity, enthesitis-related arthritis (ERA) and psoriatic arthritis were higher in patients with FHADs (OR=0.6, 95% CI 0.4-0.9 and OR=1.2, 95% CI 1.1-1.4). Also, articular JADI correlated significantly with presence of FHADs (OR=1.1, 95% CI 1.0-1.1). This study shows that autoimmune diseases cluster within families of patients with JIA with a high proportion of ERA and psoriatic arthritis. JIA patients with FHADs are likely to have more disease damage.

JNK Pathway-Associated Phosphatase as a Serum Marker for Disease Activity and Treatment Outcome of Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA) is a heterogeneous autoimmune disease characterized by arthritis of unknown etiology. JNK pathway-associated phosphatase (JKAP) is reported to be a negative regulator of T-cell activation, but its clinical role in JIA is unknown. This study aimed to investigate the correlation of JKAP with disease activity and treatment response to a tumor necrosis factor (TNF) inhibitor, etanercept (ETN), in JIA patients. Totally, 104 JIA patients (6.9 ± 2.7 years old) and 100 age- and sex-matched healthy controls (HCs) (7.2 ± 2.4 years old) were enrolled, and their serum samples were collected for measuring JKAP by enzyme-linked immunoassay. In JIA patients, after 24-week ETN treatment, clinical response was assessed based on the American College of Rheumatology pediatric criteria (ACRpedi) 50 criteria. Results showed that JKAP levels were significantly lower in JIA patients compared with HCs, and of good value in differentiating JIA patients from HCs. Among JIA patients, higher JKAP levels were associated with lower disease activity indexes, including C-reactive protein, number of joints with active arthritis, physician's global assessment of disease activity, and the present history of disease-modifying antirheumatic drugs; higher baseline JKAP levels were correlated with worse ACRpedi 50 response to ETN at week 24, and was also an independent predictive factor for worse ACRpedi 50 response to ETN. Thus, it may be inappropriate to use ETN for JIA patients with higher JKAP levels. In conclusion, serum JKAP is a potential biomarker for JIA activity and treatment response to a TNF inhibitor.