Classification Criteria For Juvenile Idiopathic Arthritis 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.

A Comparison of International League of Associations for Rheumatology and Pediatric Rheumatology International Trials Organization Classification Systems for Juvenile Idiopathic Arthritis Among Children in a Canadian Arthritis Cohort.

The aim of the Paediatric Rheumatology International Trials Organisation (PRINTO) juvenile idiopathic arthritis (JIA) classification criteria, which is still in development, is to identify homogeneous groups of JIA patients. This study was undertaken to compare International League of Associations for Rheumatology (ILAR) JIA classification criteria and PRINTO JIA classification criteria using data from the ReACCh-Out (Research in Arthritis in Canadian Children, Emphasizing Outcomes) cohort. We used clinicobiologic data recorded within 7 months of diagnosis to assign a diagnosis of JIA and identify subcategories of JIA among 1,228 patients according to the 2 JIA classification systems. We compared the proportions of patients classified and the alignment of classification categories with clinicobiologic subtypes and adult arthritis types. The PRINTO criteria classified 244 patients (19.9%) as having early-onset antinuclear antibody-positive JIA, 157 (12.8%) as having enthesitis/spondylitis-related JIA, 38 (3.1%) as having systemic JIA, and 10 (0.8%) as having rheumatoid factor-positive JIA. A total of 12% of patients were unclassifiable using the ILAR criteria, while 63.3% were unclassifiable using the PRINTO criteria (777 with other JIA and 2 with unclassified JIA). In sensitivity analyses, >50% of patients remained unclassifiable using the PRINTO criteria. Compared to the PRINTO criteria, ILAR JIA categories aligned better with clinicobiologic subtypes in 131 patients (χ2 = 44, P = 0.005, versus χ2 = 15, P = 0.07 for PRINTO), and ILAR categories aligned better with adult types of arthritis in 389 evaluable patients. Currently identified PRINTO disorders can only be used to classify a minority of JIA patients, leaving a large proportion of JIA patients with other disorders requiring further characterization. Current PRINTO JIA classification criteria do not align better with clinicobiologic subtypes or adult forms of arthritis compared with the older ILAR classification system.

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.

Comparison of paediatric and adult classification criteria in juvenile idiopathic arthritis during the transition from paediatric to adult care.

To determine the characteristics of juvenile idiopathic arthritis (JIA) patients seen during the transition period in order to compare paediatric classification criteria with those for adults. Patients with JIA according to the ILAR classification and who had a consultation at transition between 2010 and 2017 were included in a retrospective bi-centre (Lyon, Lausanne) study. JIA classification criteria were compared to ACR/EULAR 2010 criteria for rheumatoid arthritis (RA), Yamaguchi criteria for adult-onset Still's disease (AOSD), ASAS criteria for spondyloarthritis and CASPAR criteria for psoriatic arthritis. One hundred and thirty patients were included: 13.9% with systemic JIA, 22.3% with polyarticular JIA, 22.3% with oligoarticular JIA, 34.6% with enthesitis-related arthritis (ERA) and 6.9% with psoriatic arthritis; 13.1% had suffered from uveitis; 14.5% of patients had erosions or carpitis, mainly those with psoriatic arthritis, polyarticular or systemic JIA; 37.5% of patients with ERA displayed radiological sacroiliitis. When comparing paediatric JIA criteria with adult classifications, we found that: 66.6% of patients with systemic JIA fulfilled the criteria for AOSD, 87.5% of rheumatoid factor-positive polyarticular JIA and 9.5% of rheumatoid factor-negative polyarticular JIA met the criteria for RA, and 34.5% of oligoarticular JIA fulfilled the criteria for spondyloarthritis. Finally, 77.7% of patients with ERA met the criteria for spondyloarthritis, and 100% of patients with psoriatic arthritis JIA met the criteria for psoriatic arthritis. Oligoarticular JIA and rheumatoid factor-negative polyarticular JIA seem to be paediatric entities, whereas the other types of JIA tended to meet the respective adult classification criteria.

Musculoskeletal Ultrasonography in Detecting Disease Activity in Patients of Juvenile Idiopathic Arthritis: A Cross-Sectional Study

Background: Musculoskeletal ultrasonography (MSUS) is a powerful tool to detect subclinical disease in juvenile idiopathic arthritis (JIA). The study was done to compare the results of clinical examination and ultrasonography (US) in identifying disease activity in joints of patients of JIA. Methods: Patients of JIA attending rheumatology clinic of a tertiary care referral center in northern India over a span of 1 year from June 2015 to June 2016 were enrolled in the study. The diagnosis of JIA was established using International League of Associations for Rheumatology 2001 criteria. Clinically determination of synovitis was compared to MSUS parameters of active disease such as synovial hypertrophy, effusion, and increased vascularity in large joints only (bilateral knee, ankle, wrist, and elbow). Results: A total of 108 patients were enrolled in a study period and 864 joints were examined. Synovitis was detected in 305 joints by clinical examination. However, US demonstrated synovitis in 502 joints (additional 209 joints). Twenty patients who were initially thought to be oligoarticular by clinical examination were later classified to polyarticular subtype on the basis of MSUS findings. A significant correlation between erosions and rheumatoid factor and anti-cyclic citrullinated peptide values was found. Conclusion: Our study found that MSUS has a definite role in assessing the disease activity in JIA patients and this modality may be considered as an adjunct in this context, as well as considered for future inclusion in classification criteria for JIA.

Toward New Classification Criteria for Juvenile Idiopathic Arthritis: First Steps, Pediatric Rheumatology International Trials Organization International Consensus.

To revise the current juvenile idiopathic arthritis (JIA) International League of Associations for Rheumatology (ILAR) classification criteria with an evidence-based approach, using clinical and routine laboratory measures available worldwide, to identify homogeneous clinical groups and to distinguish those forms of chronic arthritis typically seen only in children from the childhood counterpart of adult diseases. The overall project consists of 4 steps. This work represents Step 1, a Delphi Web-based consensus and Step 2, an international nominal group technique (NGT) consensus conference for the new provisional Pediatric Rheumatology International Trials Organization JIA classification criteria. A future large data collection of at least 1000 new-onset JIA patients (Step 3) followed by analysis and NGT consensus (Step 4) will provide data for the evidence-based validation of the JIA classification criteria. In Step 1, three Delphi rounds of interactions were implemented to revise the 7 ILAR JIA categories. In Step 2, forty-seven questions with electronic voting were implemented to derive the new proposed criteria. Four disorders were proposed: (a) systemic JIA; (b) rheumatoid factor-positive JIA; (c) enthesitis/spondylitis-related JIA; and (d) early-onset antinuclear antibody-positive JIA. The other forms were gathered under the term "others." These will be analyzed during the prospective data collection using a list of descriptors to see whether the clustering of some of them could identify homogeneous entities. An international consensus was reached to identify different proposed homogeneous chronic disorders that fall under the historical term JIA. These preliminary criteria will be formally validated with a dedicated project.

Limitations in the Classification of Childhood-onset Rheumatoid Arthritis

Rheumatoid factor-positive polyarthritis (RF+ poly) is the juvenile idiopathic arthritis (JIA) category that resembles adult seropositive rheumatoid arthritis (RA). We studied children with RF+ and/or anticyclic citrullinated peptide antibody (anti-CCP)+ JIA to determine what proportion of those children meet International League of Associations for Rheumatology (ILAR) criteria for RF+ poly JIA and to assess for significant differences between children who meet RF+ poly criteria and those who are classified in other categories. Charts of children with JIA who were RF+ and/or anti-CCP+ were reviewed. Children with RF+ poly JIA were compared to children in other categories. Statistical analysis was performed using chi-square, Fisher's exact test, and the Student's t-test. Of 56 children with RF+ and/or anti-CCP+ JIA, 34 (61%) met ILAR criteria for RF+ poly JIA. Twelve children had RF-/anti-CCP+ JIA with low anti-CCP titers. When these 12 children were excluded, there were few significant differences between children who met criteria for RF+ poly and those who were classified in other categories. The American College of Rheumatology/European League Against Rheumatism criteria for RA identified more RF+ children than did the ILAR RF+ poly classification (100% vs 77%). A number of children with RF+ arthritis were excluded from the RF+ poly JIA classification, though many demographic features and disease measures were similar to those of children who met criteria for RF+ poly JIA. We propose prioritization of RF/anti-CCP positivity over specific exclusions, along with inclusion of anti-CCP, in future revisions of the JIA classification criteria, to improve the sensitivity of diagnosing childhood-onset RA.

Profile of Indian Patients with Juvenile Onset Chronic Inflammatory Joint Disease Using the ILAR Classification Criteria for JIA: A Community-based Cohort Study

To assess the current International League of Associations for Rheumatology (ILAR) classification criteria (Edmonton, 2001) for juvenile idiopathic arthritis (JIA) in Indian patients. Out of 441 children, 330 with chronic joint pains were diagnosed with juvenile onset chronic inflammatory arthritis and followed in an observational cohort. Our study was carried out from 1994 to 2006 in a community rheumatology clinic. Emphasis was placed on obtaining data required by the ILAR system. Of the original group, 235 children were eventually classified as having JIA; 108 were examined during the first year of illness. We assigned 224 children (95%) to discrete JIA categories: enthesitis-related arthritis (ERA; 36%), oligoarthritis (OLA-persistent; 17%), polyarthritis rheumatoid factor (RF)-negative (17%), polyarthritis RF-positive (12%), systemic arthritis (8%), OLA-extended (4%), and psoriatic arthritis (1%). The remaining 11 children (5%) were classified with undifferentiated arthritis (mostly an overlap due to seropositive RF and/or HLA-B27). The prevalence of ERA (89% HLA-B27-positive) and seropositive RF was unexpectedly high. Although agreement (kappa > 0.79) with the American College of Rheumatology criteria and the European Spondylarthropathy Study Group criteria was good to excellent, the ILAR system was found to be more comprehensive and clinically homogeneous. However, some problems appear unique in our scenario. A wide-spectrum phenotype of JIA is demonstrated by an Indian cohort. Although useful, RF and HLA-B27 in this population proved problematic to the ILAR classification.