Objective.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.Methods.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.Results.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.Conclusion.An international consensus was reached to identify different proposed homogeneous chronic disorders that fall under the historical termJIA. These preliminary criteria will be formally validated with a dedicated project.

Objective. To evaluate the safety and efficacy of infliximab in the treatment of juvenile rheumatoid arthritis (JRA).Methods. This was an international, multicenter, randomized, placebo-controlled, double-blind study. One hundred twenty-two children with persistent polyarticular JRA despite prior methotrexate (MTX) therapy were randomized to receive infliximab or placebo ClinicalTrials.gov identifier: NCT00036374.

Most children with JIA managed with contemporary treatments attain inactive disease within 2 years of diagnosis and many are able to discontinue treatment. The probability of attaining remission within 5 years of diagnosis is about 50%, except for children with polyarthritis.

Five recommendations are proposed for the orofacial examination of patients with JIA to improve the clinical practice and aid standardized data collection for future studies. The task force has formulated a future research program based on the proposed recommendations.

The mucopolysaccharidoses (MPSs) are a heterogeneous group of inherited metabolic disorders caused by enzyme deficiencies that lead to progressive lysosomal storage of glycosaminoglycans. Musculoskeletal manifestations are common across all forms of MPS and are often apparent early in the disease course. Diagnostic delays occur frequently in these patients, especially those with more attenuated forms of disease. Treatments for many types of MPS are now available; however, they are most effective if started early before the development of irreversible damage. Some manifestations such as stiffness and joint contractures may mimic other conditions such as inflammatory arthritis, which may cause further delays. Rheumatologists and other specialists should be aware of the musculoskeletal manifestations of MPS so that diagnostic delays can be avoided and appropriate management initiated.

A proposed definition of juvenile psoriatic arthritis (JPsA) was used to identify definite or probable JPsA in 35 children. Definite JPsA (24 patients) was defined as arthritis associated, but not necessarily coincident, with a typical psoriatic rash, or arthritis plus at least 3 of 4 minor criteria: dactylitis, nail pitting, psoriasis-like rash, or family history of psoriasis. Probable JPsA (11 patients) was defined as arthritis plus 2 of the minor criteria. In 33 of 35 patients, the onset of arthritis was pauciarticular, but the disease followed a polyarticular course in 23 of 35. Chronic anterior uveitis (6 of 3 3 , antinuclear antibodies (22 of 35), anticollagen antibodies (10 of 3 9 , HLA-DR4 (2 of 28), and HLA-DRS (5 of 28) occurred with frequencies similar to those seen in patients with juvenile rheumatoid arthritis. JPsA may have more in common with juvenile rheumatoid arthri-

Thirty‐nine children with a syndrome of seronegative enthesopathy and arthropathy were evaluated. The group included 25 patients with no apparent underlying primary disease and 13 with either ankylosing spondylitis, inflammatory bowel disease, reactive arthritis, or Reiter's syndrome. Significant distinguishing characteristics of the group included male predominance, late age at onset, positive family histories of arthritis, oligoarthropathy, axial skeleton involvement, and the presence of the B27 histocompatibility antigen. This syndrome is distinguishable from other childhood rheumatic disorders, including juvenile rheumatoid arthritis. Its recognition may reliably identify children with the prodromal manifestations of seronegative spondylarthropathies.

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