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Objective: This study aimed to estimate the prevalence of osteonecrosis (ON) in juvenile systemic lupus erythematosus (SLE) patients using joint-specific and wholebody MRI; to explore risk factors that are associated with the development of ON; and to evaluate prospectively patients 1 year after initial imaging. Method: Within a 2 year period, we studied 40 juvenile SLE patients (aged 8-18 years) with a history of steroid use of more than 3 months duration. Risk factors including disease activity, corticosteroid use, vasculitis, Raynaud's phenomenon and lipid profile were evaluated. All patients underwent MRI of the hips, knees and ankles using jointspecific MRI. Whole-body STIR (short tau inversion recovery) MRI was performed in all patients with ON lesions. Results: Osteonecrosis was identified in 7 patients (17.5 %) upon joint-specific MRI. Whole-body STIR MRI detected ON in 6 of these 7 patients. There was no significant difference between the ON and non-ON groups in the risk factors studied. One patient had pre-existing symptomatic ON. At 1 year follow-up, the ON lesions had resolved in one patient, remained stable in four and decreased in size in two. No asymptomatic patients with ON developed clinical manifestations. Conclusion: Whole-body STIR MRI may be useful in detecting ON lesions in juvenile SLE patients but larger studies are needed to define its role.

Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1-23.4) vs 6.2 (0-17) vs 3.3 (0-14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.

This study aims to evaluate the body composition (BC) of female children and adolescents with juvenile idiopathic arthritis (JIA). A cross-sectional, controlled study was performed to compare the BC between 42 JIA girls and 35 healthy controls, matched for age and gender. Weight and height were used to calculate body mass index (BMI), classified as a Z-score (Z-BMI). BC was evaluated by dual-energy X-ray absorptiometry (DXA; DPX-L, Lunar). The fat mass index (FMI) was calculated as the ratio between total fat mass and height squared (kilograms per square metre). The lean mass index (LMI) was calculated as the ratio between total lean mass and height squared (kilograms per square metre). In JIA patients, the median of age was 13 years (6-19) and median disease duration was 84 months (10.0-215.0). The main disease subtype was polyarticular arthritis (54.8%). We observed that 61.9% of patients had normal Z-BMI. JIA girls had higher median Z-BMI scores (0.17 vs. -0.48, p = 0.034), total body fat percentages (26.5% vs. 16.4%, p = 0.001), truncal fat (4.52 vs. 2.32, p = 0.011) and FMI (4.83 vs. 2.23, p < 0.001). For LMI, there was no difference between JIA girls and controls (13.45 vs. 12.45, p = 0.212). We did not find association between FMI and age, disease subtype, number of limited and/or active joints, months since diagnosis and use of corticosteroids or methotrexate. BC changes found in JIA girls, such as fatness and adiposity, indicate a potentially greater risk for developing hypertension, diabetes mellitus and cardiovascular diseases. These findings emphasise the importance of evaluating nutritional status and body composition to minimise the emergence of chronic diseases later in life.

Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (≥1 and <3 months); and C: long time interval (≥3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2 K were evaluated. Results The number of patients in each group was: A = 60 (4%); B = 522 (33.5%); and C = 973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P = 0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P < 0.0001) and SLEDAI-2 K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P < 0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P = 0.003), pleuritis (25% vs. 24% vs. 14%, P < 0.0001), nephritis (52% vs. 47% vs. 40%, P = 0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P = 0.008), thrombocytopenia (32% vs. 18% vs. 19%, P = 0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P < 0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P = 0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P = 0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P = 0.002), proteinuria >500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition.