Methods. Eligible patients had been diagnosed by site rheumatologists (termed the "MD diagnosis") since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients.Results. MD diagnoses of 117 patients (82 of whom were female) were WG (n ‫؍‬ 76), microscopic polyangiitis (n ‫؍‬ 17), ANCA-positive pauci-immune glomerulonephritis (n ‫؍‬ 5), Churg-Strauss syndrome (n ‫؍‬ 2), and unclassified vasculitis (n ‫؍‬ 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were

Objective Autoantibodies recognizing 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with statin exposure, the HLA allele DRB1*11:01, and necrotizing muscle biopsies in adult myositis patients. The aim of this study was to characterize the features of pediatric anti-HMGCR-positive myositis patients. Methods The sera of 440 juvenile myositis patients were screened for anti-HMGCR autoantibodies. Demographic and clinical features, responses to therapy, and HLA alleles were assessed. The features of anti-HMGCR-positive patients were compared to those of previously described adult patients with this autoantibody and to children with other myositis-specific autoantibodies (MSAs). Results Five (1.1%) of 440 patients were anti-HMGCR-positive; none had taken statin medications. Three patients had rashes characteristic of juvenile dermatomyositis and two patients had immune-mediated necrotizing myopathies. The median highest creatine kinase (CK) level of anti-HMGCR-positive subjects was 17,000 IU/L. All patients had severe proximal muscle weakness, distal weakness, muscle atrophy, joint contractures, and arthralgias, which were all more prevalent in HMGCR-positive subjects compared to MSA-negative patients or those with other MSAs. Anti-HMGCR-positive patients had only partial responses to multiple immunosuppressive medications and often a chronic course. The DRB1*07:01allele was present in all 5 patients compared to 26.25% of healthy controls (Pcorrected=0.01); none of the 5 pediatric patients had DRB1*11:01. Conclusions Compared to children with other MSAs, muscle disease appeared to be more severe in those with anti-HMGCR autoantibodies. Like adults, children with anti-HMGCR autoantibodies have severe weakness and high CK levels. In contrast to adults, anti-HMGCR-positive children have a strong association with HLA DRB1*07:01.

Objectives To use consensus methods and the considerable expertise contained within the Children’s Arthritis and Rheumatology Research Alliance (CARRA) organization, to extend the 3 previously developed treatment plans for moderate juvenile dermatomyositis (JDM) to span the full course of treatment. Methods A consensus meeting was held in Chicago on April 23–24, 2010 involving 30 pediatric rheumatologists and 4 lay participants. Nominal group technique was used to achieve consensus on treatment plans which represented typical management of moderate JDM. A pre-conference survey of CARRA, completed by 151/272 (56%) members, was used to provide additional guidance to discussion. Results Consensus was reached on timing and rate of steroid tapering, duration of steroid therapy, and actions to be taken if patients were unchanged, worsening, experiencing medication side effects or disease complications. Of particular importance, a single, consensus steroid taper was developed. Conclusions We were able to develop consensus treatment plans which describe therapy for moderate JDM throughout the treatment course. These treatment plans can now be used clinically, and data collected prospectively regarding treatment effectiveness and toxicity. This will allow comparison of these treatment plans and facilitate the development of evidence-based treatment recommendations for moderate JDM.

Objective To determine the relationship between race, income, and disease outcomes in children with juvenile dermatomyositis (JDM). Study design Data from 438 subjects with JDM enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry were analyzed. Demographic data included age, sex, race, income and insurance status. Clinical outcomes included muscle strength, presence of rash, calcinosis, weakness, physical function and quality of life measures. Disease outcomes were compared based on race and income. Results Minority subjects were significantly more likely to have low family income, and significantly worse scores on measures of physical function, disease activity and quality of life measures. Lower income subjects had worse scores on measures of physical function, disease activity and quality of life scores, as well as weakness. Black subjects were more likely to have calcinosis. Despite these differences in outcome measures, there were no significant differences in time to diagnosis or disease duration. Using calcinosis as a marker of disease morbidity, Black race, annual family income less than $50,000 per year, negative ANA, and delay in diagnosis greater than 12 months were associated with calcinosis. Conclusions Minority race and lower income are associated with worse morbidity and outcomes in subjects with JDM. Calcinosis was more common in Black subjects. Future studies are needed to further understand these associations so that efforts may be developed to address health disparities in subjects with JDM and improve disease outcomes.

EULAR/PRINTO/PRES was more sensitive than ACR criteria in classifying pediatric GPA. Neither classification system has criteria for MPA; therefore usefulness in discriminating patients in ARChiVe was limited. Even when using the most sensitive EMA algorithm, many children remained unclassified.