International League Against Rheumatism Research Articles

E27 Performance of systemic juvenile idiopathic arthritis classification criteria: one-centre experience—Libya

Abstract Systemic juvenile idiopathic arthritis (sJIA) constitutes a small part of juvenile idiopathic arthritis (JIA), yet has a disproportionally higher rate of mortality. In comparison to the other (JIA), systemic juvenile idiopathic arthritis has many distinct clinical features that arthritis may be a non-prominent and/or a delayed manifestation. The Pediatric Rheumatology International Trial Organization (PRINTO), currently is developing a new criterion for JIA classification which will allow for better identification of sJIA cases with delayed onset of arthritis and fail to fulfil the currently used International League Against Rheumatism (ILAR) and Yamaguchi criteria. It is useful in classification of children with systemic juvenile idiopathic arthritis (sJIA) particularly in ‘pre-arthritic’, pure systemic phase of the illness. Objectives The purpose of this study is to compare the performances of the Yamaguchi diagnostic criteria vs PRINTO criteria vs current ILAR criteria for systemic juvenile idiopathic arthritis. We compared the proportions of patients classified and how closely the 3 instruments are related. Methods The study was performed at the Department of Paediatric Rheumatology in Tripoli children hospital. It is a retrospective study, covering the date range 2006–2020. We used clinicobiologic data recorded within 7 months of diagnosis of Patients diagnosed with sJIA. We assigned a diagnosis of sJIA according to the 3 sJIA classification systems (the Yamaguchi diagnostic criteria, PRINTO criteria and according to ILAR classification). Results Three different classification criteria were analysed in 38 assigned sJIA children (13% of total JIA cases). Females were 28 (52.6%), and mean age of JIA onset was 7 ± 3.9 yr. The mean of the time delay between disease onset and diagnosis was 7 ± 9 months. Overall, the ILAR and PRINTO criteria were fulfilled in a higher number of patients in the study (n = 35) (92% of patients), As compared with the Yamaguchi criteria (n = 29).(76% of patients). Among the 7.9% patients who were unclassifiable using the ILAR criteria were diagnosed as sJIA by both the PRINTO and the Yamaguchi diagnostic criteria. 7.89% neither fulfilled the Yamaguchi or the PRINTO Criteria. The table below compares the performance of the three different criteria. Conclusions In this cohort the ILAR criteria appear to be superior in the early recognition and classification of sJIA. Yamaguchi diagnostic criteria can only be used to classify a minority of sJIA patients, leaving a large proportion of sJIA patients unclassified. Compared with the Yamaguchi criteria, ILAR JIA categories aligned better with PRINTO criteria. All the criteria should incorporate strengths of each other. Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

E26 Macrophage activation syndrome in systemic juvenile idiopathic arthritis, one centre experience

Abstract Background Macrophage Activation Syndrome (MAS) in Systemic Juvenile Idiopathic Arthritis (SoJIA) is a potentially life-threatening condition. The prevalence of MAS in SoJIA population is estimated to be 10%. However, reports suggested that the number of SoJIA patients with subclinical MAS may be as high as 40%. Early recognition of MAS is crucial because timely treatment is important for survival. Objective To describe the clinical and laboratory features of patients with MAS associated with SoJIA Methods Data from cohort of 38 SoJIA patients with and without MAS from January 2006 to January 2020 were included and retrospectively reviewed. Data were collected on demography, clinical/laboratory features and treatment. Results There were 38 patients with SoJIA diagnosed according to the International League Against Rheumatism (ILAR) criteria. 47.4% of them were males and 52.6% were females. They were followed up for a period of 6.3 ± 4.4 years. The mean period from appearance of symptoms to first follow up in rheumatology clinic was 5 ± 6 months. The disease course was monocyclic in 39.5%, polycyclic in 28.9% and persistent in 31.6% of the patients. Of the total cohort, 10% of the patients were classified as having MAS, half of them presented with MAS at diagnosis and the rest developed MAS during the disease course. Only One patient experienced recurrent MAS (3 times). Male to female ratio is 3:1. The mean age at diagnosis was 8 ± 6 years. Skin rash was the most presenting feature in the two groups. Hepatosplenomegaly and serositis were most common in sJIA with MAS patients. In comparison to SoJIA patients without MAS, all patients have thrombocytopenia, leukopenias, with mean ferritin level of 1668 in MAS patient and 947 in non-MAS group. The obligate criteria for the diagnosis of MAS according to the 2016 MAS classification criteria are increased ferritin >684 μg/l, elevated lactate dehydrogenase (LDH) and Glutamic Oxaloacetic Transaminase (GOT). All patients have a declining ESR and high CRP and all patients were anaemic. Bone marrow biopsy was done in 50% of the patients. In SoJIA without MAS, ESR mean level was 94 (±38.5) mm/h, CRP mean level of 62 (± 65.8) mg/dl and platelets count mean level of 561 (±215) × 109/l. Treatment all MAS-SoJIA patients were treated with IV methylprednisolone and IVIG compared with 50% of non-MAS-SoJIA patients. In the MAS group, dose of oral prednisolone was (1–2 mg/kg/day) while in non-MAS group was (0.5–1 mg/kg/day). A patient developed MAS twice while on the 7th dose of anakinra and the third MAS on the 6th dose of tocilizumab. Ciclosporin (3 mg per/kg/day) was the treatment of choice for him. Conclusions In our series, prevalence of MAS was 10% which mainly affect the male which is the same as the estimated prevalence of MAS worldwide. Arthritis occurred less frequent in MAS group cases in comparison to hepatosplenomegaly which was more common among MAS group. Rash and serositis were the most common extra-articular manifestations. The main treatment modality was steroid in all of the patients. All the MAS patients were successfully treated with high dose of IVIG, methylprednisolone and ciclosporin, including the patients who developed MAS while on biologics. Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

25 Impact of early feeding on the occurrence of juvenile idiopathic arthritis

BackgroundJuvenile idiopathic arthritis (JIA) is considered to be an autoimmune disease, which is a result of an immune reaction caused or triggered by environmental factors in a genetically susceptible individual. However, interactions between early feeding and risk Juvenile idiopathic arthritis (JIA) remains largely unexplored.ObjectivesTo determine the impact of early alimentation on the occurrence of JIA in Tunisia.MethodsWe conducted a case-control study including 26 children with JIA fulfilling the international league against rheumatism (ILAR) 2010 criteria compared with a homogeneous control group of 21 children hospitalized in a paediatric orthopedic unit for traumatic reasons and healthy for any chronic inflammatory rheumatism. Parents of patients were asked after having their consent to participate. We collected the following data: Type of JIA, juvenile disease activity score (JADAS), type of feeding, total duration of breastfeeding, duration of exclusive breastfeeding and age at introduction of gluten. Patients were divided into two groups: group 1 (G1): JIA patients, group 2 (G2): control group. Data were analyzed using the SPSS statistical package. A p-value < 0.05 was considered significant.ResultsForty-seven children (26 diagnosed with JIA and 21 controls) with a mean age of 10.07 years ± 4.96 were enrolled. JIA subtypes were in decreasing order of frequency: oligoarticular (45.8%), enthesitis-related arthritis (20.8%), undifferentiated JIA (12.5%), seronegative polyarticular JIA (8.3%), juvenile psoriatic arthritis (8.3%), and seropositive polyarticular JIA (4.2%). The mean JADAS was 6.966 ± 5.38. There was no significant difference between the G1 and G2 with regard to the duration of breastfeeding (12.23 ± 3.8 months vs 8.14 ± 4.1 months, p = 0.09), exclusive breastfeeding (3.8 ± 3.6 vs 4.1 ± 2.62, p = 0.40) and age at introduction of gluten (G1: 8.48 ± 5.30 months vs G2: 7.48 ± 1,2 months, p = 0.35)ConclusionOur study didn’t show any impact of early alimentation on the occurrence of JIA in Tunisia. However, more studies are necessary to conclude these issues.

Genicular nerve block in juvenile idiopathic arthritis: a randomized clinical trial

ObjectivesThis study aimed at evaluating the effect of genicular nerve block (GNB) in juvenile idiopathic arthritis (JIA) patients with persistent unilateral knee arthritis on pain, inflammatory parameters, function, and range of motion.MethodsA total of 104 JIA patients were diagnosed according to the International League Against Rheumatism (ILAR) criteria with persistent unilateral knee arthritis. They were allocated randomly into 2 groups: group 1 treated with GNB, while group 2 was treated with intra-articular triamcinolone (TA) only. Visual analogue scale (VAS) on pain, sonography of large joints in rheumatology (SOLAR) scoring system, and Lysholm scores were assessed at 0-, 2-, and 12-week intervals. Swelling and tenderness were clinically evaluated semi-quantitatively (0-3) at the same time intervals.ResultsVAS pain, tenderness, swelling, and SOLAR grey scale (GS) and power Doppler (PD) scores were significantly reduced after 2 weeks in both groups (p < 0.05). This was greater in the GNB group regarding VAS and tenderness, while SOLAR and swelling were stronger reduced in TA group. After 12 weeks, all outcome measures showed lower values in the GNB group compared to TA, and this was significant regarding VAS pain. Moreover, Lysholm functional score was significantly increased in both groups at both intervals; and higher values were seen in the TA group compared to GNB after 2 weeks.ConclusionGNB was able to control pain and improve function and inflammation of the knee joint in JIA patients. Though steroid attained better results after 2 weeks, GNB achieved an equivalent longer-term improvement after 12 weeks.Trial registration identifying numberNCT04687930.Key Points• Persistent knee arthritis treatment in JIA is always challenging.• GNB was approved for treatment of pain in knee osteoarthritis.• GNB in the present study succeeded to control active knee arthritis and this effect was comparable to intra-articular steroid injection.

Association of HLA-B27 antigen with clinical and laboratory parameters in patients with juvenile idiopathic arthritis.

Aim To analyse the association of human leukocyte antigen B27 with clinical and laboratory parameters in patients with juvenile idiopathic arthritis (JIA) at the disease onset. Methods A retrospective review of medical records of 25 HLAB27 positive and 25 HLA-B27 negative JIA patients was performed. The diagnosis of JIA was based on the 1997-2001 International League Against Rheumatism (ILAR) criteria. Collected data: age, sex, HLA- B27 antigen presence, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), rheumatoid-factor (RF), antinuclear antibody (ANA), fever, rash, uveitis, enthesitis, inflamed joints and subtype of JIA. Results HLA- B27 positive study group had more boys (p=0.01), higher erythrocyte sedimentation rate (p=0.038), higher presence of fever (p= 0.025) and enthesitis (p=0.024). Any significant difference in age of the disease onset, CRP, ANA, RF, rash, uveitis, inflamed joint and dactylitis was not noticed. The most common subtype of JIA in the HLA-B27 positive patients was ERA (60%). Conclusion This study showed that the presence of HLA- B27 antigen plays a significant role in determining the presenting clinical and laboratory characteristics in JIA patients.

Clinical aspects of HLA-B27 positive enthesitis-related arthritis in childhood

Introduction: Enthesitis-related arthritis (ERA) is a form of juvenile idiopathic arthritis (JIA) according to the International League Against Rheumatism (ILAR) classification. Enthesitis-related arthritis accounts for 8.6 to 18.9% of all children with JIA. The first clinical sign of ERA is usually asymmetrical arthritis of large joints, especially of the lower extremities. Axial involvement is typically presented by unilateral or bilateral sacroiliitis. Enthesopathy is a unique feature of ERA. Aim: The aim of this study is to determine the most common clinical characteristics in children with HLA-B27 positive ERA. Materials and Methods: We performed a retrospective analysis of 55 patients up to 16 years of age, diagnosed with HLA-B27 positive ERA. We used physical and laboratory tests as well as imaging techniques to assess various clinical aspects of ERA in every patient. Results: Demographic characteristics showed prevalence of male gender—44 boys vs. 11 girls (4:1 ratio). The mean age of onset was 13.3 years for boys and 9.9 years for girls. The most commonly affected joints were as follows: knees in 49% of the cases, ankles—in 43.6%, hip joints—in 36.3%, intertarsal/metatarsophalangeal joints—in 20%. Physical examination found enthesitis in 24 of the patients (43.6%). The most commonly affected entheses were: the site of attachment of the Achilles tendon in 66.6% of the cases, the site of attachment of the plantar fascia in 45.8%, the site of attachment of the patellar tendon in 12.5%. Plain X-ray of the sacroiliac joints detected sacroiliitis in 34 of the patients (61.8%). In over 90% of the cases, the sacroiliitis was bilateral. Conclusion: HLA-B27 positive ERA is a rare pathology in childhood. This study confirms typical epidemiological and clinical characteristics of the disease among Bulgarian children.

Juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is an umbrella term for arthritis of unknown origin, lasting for >6 weeks with onset before 16 years of age. JIA is the most common chronic inflammatory rheumatic condition of childhood. According to the International League Against Rheumatism (ILAR) classification, seven mutually exclusive categories of JIA exist based on disease manifestations during the first 6 months of disease. Although the ILAR classification has been useful to foster research, it has been criticized mainly as it does not distinguish those forms of chronic arthritis observed in adults and in children from those that may be unique to childhood. Hence, efforts to provide a new evidence-based classification are ongoing. Similar to arthritis observed in adults, pathogenesis involves autoimmune and autoinflammatory mechanisms. The field has witnessed a remarkable improvement in therapeutic possibilities of JIA owing to the availability of new potent drugs and the possibility to perform controlled trials with support from legislative interventions and large networks availability. The goal of drug therapy in JIA is to rapidly reduce disease activity to inactive disease or clinical remission, minimize drug side effects and achieve a quality of life comparable to that of healthy peers. As JIA can influence all aspects of a child's and their family's life, researchers increasingly recognize improvement of health-related quality of life as a key treatment goal.

P040 Effect of parental smoking on Juvenile Idiopathic Arthritis activity

Abstract Background Juvenile idiopathic arthritis (JIA) is an heterogeneous group of autoimmune diseases considered as the main cause of chronic arthritis among children and teenagers. This group of children is thought to be more vulnerable because of chronic inflammation and long-term immunosuppressive therapy. Passive smoking, which is a major health problem, is likely to be particularly harmful for these children whose immunity is already compromised by their disease. The aim of our study was to assess the effect of parental smoking on disease activity in children with JIA Methods A monocentric cross-sectional study of patients with JIA according to the criteria of the International League Against Rheumatism (ILAR) was conducted in the rheumatology department of Kassab Institute. The following clinical and biological data were collected: sex, age, disease duration, BASDAI activity score, functional impairment assessed by the BASFI score, biological inflammation assessed by the erythrocyte sedimentation rate (ESR) and the C-reactive protein (CRP). All parents were contacted and smoking data were recorded: parent smoking, pack-years history, smoking in an indoor or outdoor space, impact of passive smoking on disease activity. We considered any child with at least one smoking parent and living in the same house to be exposed to parental passive smoking. Patients were divided into two groups: group 1 (G1) including patients who are not exposed to parental smoking and group 2 (G2) including patients exposed to smoking by one or both parents. Data were analyzed using SPSS (Statistical Package for the Social Sciences) version 24 software. The significance level was set at a p-value &amp;lt; 0.05. Results Twenty-three patients were included: 15 girls and 8 boys. Sex ratio M/F was 0.53. The mean age was 16.5 + 8.1 years [8–40]. The mean disease duration was 4.9 + 4.9 years [0.15]. The mean ESR was 21.5 + 27.1 mm and the mean CRP was 4.7 + 7.1 mg/l. Nine patients had coxitis, including 4 who were exposed to passive smoking. Passive smoking noted in 11 patients (47.8%), including 6 boys and 5 girls. In 63.6% of the cases, this exposure occurred indoor in the family home. The number of cigarettes smoked per day was 11.7 + 8.4 [1,20]. Among the exposed patients, 36.4% considered that this exposure was an exacerbating factor of their disease. Clinical and biological data were comparable between the 2 groups as shown in table 1. Conclusion Although the adverse effect of maternal smoking during pregnancy on the risk of developing JIA in the child has been demonstrated, few studies have investigated the effect of parental smoking once the disease is established. Our study did not find a statistically significant association between smoking exposure and high disease activity or poor prognosis. However, further studies including a larger number of patients are needed to assess the potential effect of parental smoking on the prognosis of JIA.

Translation of revised WHO international league against rheumatism community oriented program for the control of rheumatic diseases core English questionnaire into Bengali and its cross-cultural adaptation and validation

Background: The World Health Organization (WHO) and the International League Against Rheumatism (ILAR) initiated the Community-Oriented Program for the Control of Rheumatic Diseases (COPCORD) project in 1981. The aim of this study was to translate the revised WHO-ILAR COPCORD questionnaire into Bengali and to test if the Bengali version could be reliably used for the identifying rheumatic diseases in Bangladeshi people.Methods: This was an observational study conducted in rheumatology wing, department of medicine, BSMMU from April to October, 2011. It was implemented in two stages: Translation and cross-cultural adaptation of questionnaire and testing of questionnaire. For translation into Bengali and cross-cultural adaptation, the recommendation by Beaton’s procedure was followed. Respondents were enrolled from rheumatology outpatient departments of medicine, BSMMU, household members and healthy patient’s attendants. Data were collected and analyzed using SPSS windows v16.Results: Thirty subjects completed the preliminary version of the questionnaire. Fifteen males (50%) and fifteen (50%) females were interviewed. Mean age of the respondents were 31.97±10.15 with age range 16-55 years. Test-retest reliability of the Bengali version of the questionnaire was tested by interviewing 70 subjects. To evaluate agreement between two measurement qualitative questions were analyzed using kappa test and quantitative question were tested by spearman’s correlation. Most of the questions (88.2%) showed high or good agreement (kappa agreement &gt;0.6) and one (5.88%) question showed moderate agreement (0.4-0.6). In case of quantitative questions responses in test and re-test were found to have 50% good correlation and remainder poor correlation in Spearman’s correlation.Conclusions: Pretest and reliability has shown the questionnaire clear in wording and culturally appropriate. Process of translation and adaptation using Beaton’s procedure was user friendly, comprehensive and appears an essential method for translation of the questionnaire.

Clinical correlates of HLA-B*27 and its subtypes in enthesitis-related arthritis variant of juvenile idiopathic arthritis in south Indian Tamil patients.

The aim of the study was to assess the distribution of human leukocyte antigen (HLA)-B*27 subtypes and its correlation with disease phenotypes in children with enthesitis-related arthritis variant of juvenile idiopathic arthritis (JIA-ERA). One hundred and sixty patients (132 males, 28 females) satisfying the International League Against Rheumatism (ILAR) classification criteria for JIA-ERA were assessed and relevant demographic, clinical and radiographic data were documented. HLA-B*27 typing was done for all the patients and B*27 positive samples were subjected to high-resolution gene sequencing. The effect of duration of illness, HLA-B*27, its subtypes, and gender on the clinical phenotype were analyzed. The mean age of disease onset was 12.69±2.4years with a male:female ratio of 4.7:1.0. HLA-B*27 was positive in 109/160 patients and HLA-B*27:04 was detected in 63% followed by B*27:05 (30%). Duration of illness was greater in patients with skeletal deformity, hip arthritis, sacroiliitis, cervical spine involvement and acute anterior uveitis (AAU) (P<0.05). HLA-B*27 positivity was associated with a prolonged course of disease, higher incidence of AAU (14.7% vs 2%, P=0.015), family history of spondyloarthritis (21.1% vs 5.9%; P=0.015) and higher erythrocyte sedimentation rate as compared to HLA-B*27 negative patients (P<0.01). The HLA-B*27:04 and *27:05 positive patients had similar clinical phenotypes. Presence of HLA-B*27 and long duration of illness results in skeletal deformity, hip arthritis, sacroiliitis, cervical spine involvement and AAU. HLA-B*27:04 followed by B*27:05 are the most common HLA-B*27 subtypes in our study population and both have a similar clinical phenotype.

Protective role of HLA-DRB1*11 against juvenile idiopathic arthritis living in North Eastern Iran.

Objective(s):Juvenile idiopathic arthritis (JIA) is one of the most common chronic rheumatic diseases in children. The complex nature of this immune-mediated disease owes itself to several predisposing genes and environmental factors affecting its pathogenesis. Conducted in Iran, this study was originally intended to investigate every possible association between HLA DRB1 alleles and a susceptibility to JIA.Materials and Methods:In this case-control study, 45 patients with a definite diagnosis of JIA based on International League against Rheumatism (ILAR) criteria were compared against 46 healthy controls. DNA samples taken from both groups were analyzed using PCR-sequence specific primers (PCR-SSP) method. Data analysis including parametric and nonparametric test and multivariate analysis was undertaken using the SPSS 11.5 software. A P-value< 0.05 was regarded as statistically significant.Results:Mean ages in case group and healthy controls were 14.64±6.21 and 13.73±6.39, respectively with no significant difference between the two groups (P=0.515). Sex difference between JIA group and healthy controls was also not significant (P=0.068). The frequency of HLA-DRB1*01 was found the most frequent HLA-RB1 in our patients (33.3%). No significant statistical correlation between various HLA-DRB1 alleles and clinical subtypes of the disease could be established from the data. HLA-DRB1*11 was shown to raise protection to JIA (P=0.035, OR=2.755, 95% CI=0.963-8.055) in northeastern Iran. In addition, we found that HLA-RB1*09 is nominally associated with an increased risk of JIA (P=0.56, OR=2, 05, 95% CI=0.18-23.63).Conclusion:HLA-DRB1*11 was shown to raise protection to JIA in northeastern Iran. The disparity of findings in other ethnicities prompts further investigations with larger sample sizes.

Application of the international league against rheumatism classification criteria for systemic juvenile idiopathic arthritis as a prognostic factor in patients with adults-onset Still\u2019s disease

BackgroundAdult-onset Still’s disease (AOSD) is an adult form of systemic juvenile idiopathic arthritis (JIA) that differs from the latter in its classification. This study evaluated the concordance between the International League Against Rheumatism (ILAR) criteria for systemic JIA and the Yamaguchi criteria and then compared their possible prognostic value in patients with AOSD.MethodsIn a retrospective review of 169 adults with suspected AOSD, patients were classified according to the Yamaguchi or ILAR criteria. Then the concordance in cross-referencing the other group with the different criteria was investigated and the sensitivity and specificity of each set of criteria were determined. Disease activity markers in AOSD patients were correlated with positivity according to both systems.ResultsConcordance was good in patients with suspected AOSD (k = 0.7144, p < 0.001) and low in those with a diagnosis of AOSD (k = 0.3787, p < 0.001). The sensitivity of the ILAR criteria in AOSD patients was 0.8864 (95% confidence interval (CI): 0.8322–0.9405), and the specificity was 0.7838 (0.6511, 0.9164). Positivity according to the ILAR criteria correlated with the systemic score (r = 0.763, p < 0.0001) and C-reactive protein levels (r = 0.183, p = 0.0356) and was associated with a relapse (odds ratio: 1.589, 95% CI: 1.043–2.421), macrophage activation syndrome (MAS; odds ratio: 1.993, 95% CI: 1.218–3.263) and care in the intensive care unit (ICU; odds ratio: 2.087, 95% CI: 1.086–4.011).ConclusionsIn AOSD patients, there is fair concordance between the Yamaguchi and ILAR criteria for systemic JIA. Positive ILAR criteria may be useful for identifying AOSD patients at high risk for relapse, MAS and the need for ICU care. Further studies including larger populations from several centers are needed to confirm our results regarding the utility of the ILAR criteria in AOSD patients.

Clinical and biochemical characteristics of children with juvenile idiopathic arthritis.

To determine the clinical and biochemical characteristics of children with Juvenile Idiopathic Arthritis (JIA) at a tertiary care centre in Karachi, Pakistan. A descriptive study. Paediatric Rheumatology Clinic of The Aga Khan University Hospital (AKUH), Karachi, from January 2008 to December 2011. Clinical and laboratory profile and outcome of children less than 15 years of age attending the Paediatric Rheumatology Clinic of the Aga Khan University, Karachi with the diagnosis of Juvenile Idiopathic Arthritis according to International League against Rheumatism were studied. These children were classified into different types of JIA; their clinical and laboratory characteristics, response to therapy and outcome was evaluated. Sixty eight patients satisfying the criteria of International League against Rheumatism (ILAR) for Juvenile Idiopathic Arthritis were enrolled during the study period of four consecutive years, their age ranged from 9 months to 15 years. Mean age at onset was 6.45 ± 4.03 years while mean age at diagnosis was 7.60 ± 3.93 years. Polyarticular was the most predominant subtype with 37 (54%) patients, out of these, 9 (24%) were rheumatoid factor positive. An almost equal gender predisposition was observed. Fever and arthritis were the most common presenting symptoms, with only 2 patients presenting with uveitis. The clinico-biochemical characteristics of JIA at the study centre showed a pattern distinct with early onset of disease, high frequency of polyarticular type and a higher rheumatoid factor (QRA) and ANA positivity in girls.

Balneotherapy (or spa therapy) for rheumatoid arthritis.

Balneotherapy (or spa therapy) for rheumatoid arthritis.

JIA pathogenesis - genetics

Juvenile Idiopathic Arthritis is a heterogeneous disease with significant variability in long-term outcome and treatment response; this occurs both between and within JIA subtypes, as defined by the current International League Against Rheumatism (ILAR) classification. There are currently no robust clinical or biological predictors of outcome or treatment response in JIA. Given that JIA is a complex genetic disorder, genetic studies provide an opportunity to address this issue, and whilst previous studies have often been limited by statistical power to identify the likely modest effect sizes, the recent establishment of a number of international consortia for JIA genetics has allowed this issue to be resolved. This presentation will summarize the current understanding of the genetic basis of JIA susceptibility, prognosis and treatment response and will describe how these genetic associations may further our understanding of the molecular mechanisms and immunological pathways involved in this disease. In addition it will provide insights into how we might utilise this data to progress towards the ultimate goals of predicting long-term disease outcomes at onset, predicting drug response, and move towards more targeted treatment options for children with JIA.

THU0307 Validation of JADAS in international league against rheumatism (ILAR) subtypes of juvenile idiopathic arthritis (JIA)

BackgroundJuvenile Arthritis Disease Activity Score (JADAS) is a 4 variable disease activity (DA) score for JIA (including active 10, 27 or 71 joint count (AJC), physician global assessment (PGA), parent/child...

Ongoing disease-activity and change in ILAR categories in a Nordic juvenile idiopathic arthritis cohort

Purpose Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. During the last decade international consensus on the ILAR (International League Against Rheumatism) classification of this heterogeneous group of chronic childhood arthritis has been reached. In an era of modern medical treatment, epidemiologic studies on outcome in unselected cohorts of children with JIA are important. The aim of the study was to describe disease characteristics, ILAR categories and remission status during the first eight years after disease onset in a cohort of Nordic children with JIA in a population-based setting.

Prevalence of arthritis in India and Pakistan: a review

Recent studies of rheumatoid arthritis worldwide suggest that prevalence of arthritis is higher in Europe and North America than in developing countries. Prevalence data for major arthritis disorders have been compiled in West for several decades, but figures from the third world are just emerging. A coordinated effort by WHO and ILAR (International League Against Rheumatism) has resulted in collecting data for countries like Philippines, China, Malaysia, Indonesia, and rural South Africa but the information about prevalence of arthritis in India and Pakistan is scarce. Since both countries, i.e., India and Pakistan, share some ethnic identity, we reviewed published literature to examine the prevalence of arthritis in these countries. Medline and Pubmed were searched for suitable articles about arthritis from 1980 and onwards. Findings from these articles were reviewed and summarized. The prevalence, clinical features, and laboratory findings of rheumatoid arthritis are compiled for both India and Pakistan. Data collected from these two countries were compared with each other, and some of the characteristics of the disease were compared with Europe and North America. It is found to be quite similar to developed countries. Additionally, juvenile rheumatoid arthritis is of different variety than reported in West. It is more of polyarticular onset type while in West pauciarticular predominates. Additionally, in systemic onset, JRA uveitis and ANA are common finding in developed countries; on the other hand, they are hardly seen in this region. Although the prevalence of arthritis in Pakistan and India is similar to Western countries, there are inherent differences (clinical features, laboratory findings) in the presentation of disease. The major strength of the study is that it is the first to pool reports to provide an estimate of the disease in the Indian subcontinent. Scarcity of data is one of the major limitations. This study helps to understand the pattern of disease in this part of country that can be stepping-stone for policy makers to draft policies that can affect target population more appropriately.

A.I. Nesterow (1895–1979) und die russische Rheumatologie

Alongside several important Russian scientists of the 18th century (G.I. Sokolski, S.P. Botkin), W.T. Talalajew - with his work on cardiac rheumatoid nodules - and W.M. Bechterew, the namesake of ankylosing spondylitis, made significant contributions to international rheumatology. While the Dutchman Jan van Breemen (1874-1961) was busy founding international rheumatology, Russian rheumatology played an at least equally important role in Europe, attracting much attention with the brilliant organisation of the 4th Congress of the International League Against Rheumatism (ILAR) in Moscow in 1934. The later institutionalised and perfectly organised rheumatological care in the giant empire is inextricably linked with the name of Anatoli Innokentjewitsch Nesterow. He founded a large institute in Moscow and, together with his many students, laid the foundations for a network of rheumatological dispensaries in Russia and the former Soviet Republic. Moreover, as the leading centre for the Working Group of Rheumatologists in socialist countries, he and his colleagues also gave specialist work in the GDR the impetus it needed. His life and work, as well as that of his congenial successor, W. A. Nassonova, are described, partially on the basis of personal encounters.

The future of ILAR

AbbreviationsACR American College of RheumatologyAFLAR African League Against RheumatismAPLAR Asia Pacific League of Associationsfor RheumatologyCOPCORD Community Oriented Program for Controlof Rheumatic DiseasesEULAR European League of Associationsof RheumatologyOMERACT Outcome Measures for Arthritis ClinicalTrialsPANLAR Pan-American League of Associationsfor RheumatologyFaced with the choice between adaptation and extinction,ILAR has, in the past 2 years, undergone a fundamentalmetamorphosis. Even ILAR’s name is new, albeit with thesame initials. No longer the International League AgainstRheumatism, ILAR is now the International League ofAssociations of Rheumatology. From my perspective asChair of ILAR’s Executive Committee in 2008, I will reviewrecent progress and future prospects for the new ILAR.The initial entity that later evolved into ILAR wasknown as The International Committee on Rheumatism,and its formation, in 1927, at the International Society ofHydrology meeting in Paris, preceded the existence ofnational and regional rheumatology societies or leagues [1].The first President of this new organization was Dr. JFortescue Fox of London, England—who is not, to myknowledge, one of my biological ancestors even though hecan be viewed as one of my professional forefathers! Anoffice was opened in Amsterdam and within a year a newname was adopted—le Ligue International contre leRheumatisme. The first journal published by ILAR wasActa Rheumatologica [1]. For decades the ILAR meetingswere the primary forum for the international exchange ofscientific and clinical information in the field of rheuma-tology. ILAR developed a productive relationship with theWorld Health Organization, and more recently with theBone and Joint Decade and with OMERACT [2, 3].Importantly, ILAR helped sponsor the COPCORD network,which has conducted community-based studies of theprevalence of rheumatic diseases in many parts of thedeveloping world [2]. In several countries the onlyavailable data concerning the epidemiology of arthritis hasbeen generated by COPCORD.More recently, however, interest in and support for theILAR meetings waned, as both the ACR and the EULARcongresses grew larger and more international in scope.Fortunately, the publication of Clinical Rheumatology,ILAR’s current journal, has continued without interruption,and this may indeed have saved ILAR from disappearingentirely.After 2 years of planning, 2008 saw the implementationof a new structure and mission for ILAR. The governanceof ILAR has been streamlined and is centered on anExecutive Committee that is comprised of the President andPresident-Elect of five rheumatology organizations:AFLAR, APLAR, EULAR, PANLAR (the four regionalleagues), and the ACR. The Chair position rotates annually,to ensure an equal role for each member organization. TheExecutive committee meets twice each year: in early Juneat the EULAR congress and in the fall at the ACR meeting.