ObjectiveLupus nephritis is a key driver of morbidity and mortality in SLE. Detecting active nephritis on a background of pre-existing renal damage is difficult, leading to potential undertreatment and accumulating injury. An unmet need is a biomarker that distinguishes active lupus nephritis, particularly important in paediatrics where minimising invasive procedures is desirable.MethodsThis was a multicentre, prospective study of 113 paediatric patients with biopsy-proven lupus nephritis. Clinical data and urine were obtained every 3–4 months and patients averaged 2 years on study with seven time points. Urine was analysed for human epidermal growth factor receptor 2 (HER2), tumour necrosis factor-like weak inducer of apoptosis and vascular cell adhesion molecule-1 (VCAM-1) by ELISA. We defined active disease as either a rise in serum creatinine ≥0.3 mg/dL from baseline or a rise in renal Systemic Lupus Erythematosus Disease Activity Index score from the previous visit. These markers were also studied in patients with acute kidney injury, juvenile idiopathic arthritis (JIA), amplified pain syndrome and healthy controls.ResultsThe rate of active disease was 56% over an average of 2 years of follow-up. HER2 and VCAM-1 were significantly elevated at time points with active disease defined by increased serum creatinine compared with time points with inactive disease or patients who never flared. All three biomarkers were associated with new-onset proteinuria and VCAM-1 was elevated at time points preceding new-onset proteinuria. These biomarkers were not increased in acute kidney injury or JIA.ConclusionAll three biomarkers were associated with new onset proteinuria and increased VCAM-1 may predict impending proteinuria. These biomarkers provide potential non-invasive measures for monitoring that may be more sensitive to impending flare than conventional measures.

Objective: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis. Methods: Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months. Results: One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males. Race, age of onset, disease duration, and disease subtypes (70% diffuse cutaneous) were similar between males and females. Active digital ulceration, very low body mass index, and tendon friction rubs were significantly more frequent in males. Physician global assessment of disease severity and digital ulcer activity was significantly higher in males. Composite pulmonary involvement was also more frequent in males, though not statistically significantly. After 12 months, they are the pattern of differences changed female patients had significantly more frequent pulmonary involvement. Conclusion: In this cohort, juvenile onset systemic sclerosis had a more severe course in males at baseline and but the pattern changed after 12 months. Some differences from adult findings persisted, there is no increased signal of pulmonary arterial hypertension or heart failure in male pediatric patients. While monitoring protocols of organ involvement in juvenile onset systemic sclerosis need to be identical for males and females.

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priorities. Disease burden can be measured using a variety of indicators such as mortality, morbidity or financial cost. For SLE, despite a relatively good 10 year survival (95%, vs 97% in controls), its mortality rate remains high relative to general population mortality rate as shown in our analysis of the Centers for Disease Control (CDC)'s mortality database comprising of 62,843 SLE deaths. However, mortality rates may not adequately measure SLE burden, because among those who died, a fifth of patients died before reaching 40 years of age. Premature mortality, a measure of burden first proposed in 1940s to address the inadequacy of mortality in measuring the burden of tuberculosis, is an important way to quantify disease burden. In constructing a measure of premature death, an arbitrary limit to life is chosen, and the calculation of the difference between an age at death and this arbitrary limit is defined as the life lost as a result of that death. To assess the relative burden of SLE, we measured the years of potential life lost (YPLL) for SLE relative to CDC's top 15 leading causes of death. Methods This is a population-based study. Death counts were obtained from the CDC-WONDER database. Number of deaths were tabulated for top 15 CDC's leading causes-ofdeath and SLE. To calculate YPLL, each decedent's age at death from a specific disease was subtracted from a predetermined age of 75 years. The years of potential life lost were then added together to yield the total YPLL. Results 28 411 female SLE deaths were recorded during 2000-2015. The ranking of SLE deaths relative to the CDC's official leading-causes-of-death ranklist in females showed that SLE is within the top 15 leading causes-of-death in reproductive age women (15-44 years) and tenth among women ages 15-24 years. We calculated YPLL for SLE relative to the CDC's official 15 leading causes-of-death in women ages 15-44 and 15-24. YPLL for SLE ranked 14th in women ages 15-44, and 8th in women ages 15-24, above diabetes mellitus, HIV disease, septicemia, chronic lower respiratory disease, anemias, nephritis, and cerebrovascular disease. Conclusions SLE is among the leading cause of YPLL in young women, underscoring SLE as an important public health issue.

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