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.

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

EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.

Younger age at disease onset and, perhaps, both gastrointestinal manifestations and more severe kidney disease seem to characterize the clinical profile in children with MPA compared to those with GPA. Delay in diagnosis suggests that recognition of these systemic vasculitides is suboptimal. Compared with adults, initial treatment regimens in children were comparable, but the complete reversal of female-to-male disease prevalence ratios is a provocative finding.

Objective. Vertebral fractures are an under-recognized problem in children with inflammatory disorders. We studied spine health among 134 children (87 girls) with rheumatic conditions (median age 10 years) within 30 days of initiating glucocorticoid therapy. Methods. Children were categorized as follows: juvenile dermatomyositis (n ‫؍‬ 30), juvenile idiopathic arthritis (n ‫؍‬ 28), systemic lupus erythematosus and related conditions (n ‫؍‬ 26), systemic arthritis (n ‫؍‬ 22), systemic vasculitis (n ‫؍‬ 16), and other conditions (n ‫؍‬ 12). Thoracolumbar spine radiograph and dual x-ray absorptiometry for lumbar spine (L-spine) areal bone mineral density (BMD) were performed within 30 days of glucocorticoid initiation. Genant semiquantitative grading was used for vertebral morphometry. Second metacarpal morphometry was carried out on a hand radiograph. Clinical factors including disease and physical activity, calcium and vitamin D intake, cumulative glucocorticoid dose, underlying diagnosis, L-spine BMD Z score, and back pain were analyzed for association with vertebral fracture. Results. Thirteen vertebral fractures were noted in 9 children (7%). Of these, 6 patients had a single vertebral fracture and 3 had 2-3 fractures. Fractures were clustered in the mid-thoracic region (69%). Three vertebral fractures (23%) were moderate (grade 2); the others were mild (grade 1). For the entire cohort, mean ؎ SD L-spine BMD Z score was significantly different from zero (؊0.55 ؎ 1.2, P < 0.001) despite a mean height Z score that was similar to the healthy average (0.02 ؎ 1.0, P ‫؍‬ 0.825). Back pain was highly associated with increased odds for fracture (odds ratio 10.6 [95% confidence interval 2.1-53.8], P ‫؍‬ 0.004). Conclusion. In pediatric rheumatic conditions, vertebral fractures can be present prior to prolonged glucocorticoid exposure.

Purpose-Vertebral fractures due to osteoporosis are a potential complication of childhood acute lymphoblastic leukemia (ALL). To date, the incidence of vertebral fractures during ALL treatment has not been reported. CIHR Author ManuscriptCIHR Author Manuscript CIHR Author ManuscriptPatient and Methods-We prospectively evaluated 155 children with ALL during the first 12 months of leukemia therapy. Lateral thoracolumbar spine radiographs were obtained at baseline and 12 months. Vertebral bodies were assessed for incident vertebral fractures using the Genant semi-quantitative method, and relevant clinical indices such as spine bone mineral density (BMD), back pain and the presence of vertebral fractures at baseline were analyzed for association with incident vertebral fractures.Results-Of the 155 children, 25 (16%, 95% Confidence Interval (CI) 11% to 23%) had a total of 61 incident vertebral fractures, of which 32 (52%) were moderate or severe. Thirteen of the 25 children with incident vertebral fractures (52%) also had fractures at baseline. Vertebral fractures at baseline increased the odds of an incident fracture at 12 months by an odds ratio of 7.3 (95% CI 2.3 to 23.1, p = 0.001). In addition, for every one standard deviation reduction in spine BMD Zscore at baseline, there was 1.8-fold increased odds of incident vertebral fracture at 12 months (95% CI 1.2 to 2.7, p = 0.006).Conclusion-Children with ALL have a high incidence of vertebral fractures after 12 months of chemotherapy, and the presence of vertebral fractures and reductions in spine BMD Z-scores at baseline are highly associated clinical features.

Objective. To determine the frequency of incident vertebral fractures (IVF) 12 months after glucocorticoid (GC) initiation in children with rheumatic diseases and to identify children at higher risk.Methods. Children with rheumatic diseases initiating GC were enrolled in a prospective observational study. Annual spine radiographs were evaluated using the Genant semiquantitative method. Spine areal bone mineral density (aBMD) was measured every 6 months. Clinical features, including cumulative GC dose, back pain, disease and physical activity, calcium and vitamin D intake, and spine aBMD Z scores, were analyzed for association with IVF. Results. Seven (6%) of 118 children (95% confidence interval 2.9 -11.7%) had IVF. Their diagnoses were: juvenile dermatomyositis (n ‫؍‬ 2), systemic lupus erythematosus (n ‫؍‬ 3), systemic vasculitis (n ‫؍‬ 1), and mixed connective tissue disease (n ‫؍‬ 1). One child was omitted from the analyses after 4 months because of osteoporosis treatment for symptomatic IVF. Children with IVF received on average 50% more GC than those without (P ‫؍‬ 0.030), had a greater increase in body mass index (BMI) at 6 months (P ‫؍‬ 0.010), and had greater decrements in spine aBMD Z scores in the first 6 months (P ‫؍‬ 0.048). Four (67%) of 6 children with IVF and data to 12 months had spine aBMD Z scores less than ؊2.0 at 12 months compared to 16% of children without IVF (P ‫؍‬ 0.011). Conclusion. The incidence of VF 12 months following GC initiation was 6%; most children were asymptomatic. Children with IVF received more GC, had greater increases in BMI, and had greater declines in spine aBMD Z scores in the first 6 months.

The PVAS validity in children with systemic vasculitis was demonstrated. Like the BVAS, we anticipate that the PVAS will provide a robust tool to objectively define disease activity for clinical trials and future research.