Relationship between Damage Accrual, Disease Flares and Cumulative Drug Therapies in Juvenile-Onset Systemic Lupus Erythematosus

Abstract

Our objective was to investigate the pattern of damage accumulation in patients with juvenile-onset systemic lupus erythematosus (JSLE) and the relationship between damage accrual, disease flares and cumulative drug therapies. All patients with SLE followed prospectively in three tertiary care centres were identified. Only patients who presented within 12 months of diagnosis and were followed for at least three years were included. Damage was measures based on chart review using the SLICC/ACR damage index (SDI), which was modified (M-SDI) by adding the item growth failure. Mild-moderate and severe disease flares were defined by the increase in SLEDAI-2K. The cumulative duration of drug therapies was calculated in each patient. Fifty-seven patients were included. The mean M-SDI score for the whole patient group increased over time, from 0.1 at one year to 0.8 at three years to 1.5 at five years. Ocular and renal damage and growth failure were observed most frequently. Compared to patients with stable damage, patients who accrued new damage had a significantly greater frequency of severe disease flare in the first three years of follow-up. No significant difference was observed in any cumulative drug therapy between patients who accrued damage and those who did not. Damage accrual was associated with severe disease flares, suggesting that judicious use of immunosuppressive agents to achieve prompt control of severe exacerbation of disease activity is important in minimizing damage in patients with JSLE.

Transition of Care and Health‐Related Outcomes in Pediatric‐Onset Systemic Lupus Erythematosus

“…By comparison, studies on damage accrual in other, primarily white, pediatric-onset SLE cohorts report damage in around 60% of patients over a followup period of 3 to 5 years (30,32). The different ethnic composition of our mainly Hispanic cohort, as well as the longer followup period compared to the other studies, may explain the higher prevalence of damage.…”

“…The bulk of pediatric-onset SLE-related damage occurs early in the disease course (30)(31)(32)(33)(34), and most patients will already have accrued end-organ manifestations at the time of pediatric discharge. However, damage accrual continues in adulthood (22), which highlights the importance of ongoing subspecialty care through the transition process and thereafter.…”

Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system

“…The female to male ratio observed in our patient sample was 3.3:1, which is in agreement with the previous observation that JSLE has a higher male prevalence than adult SLE 3,5,[8][9][10]24 . race distribution was probably influenced by local ethnical background, where white and miscegenation among white, black and Native Americans predominate.…”

Irreversible organ damages in patients with systemic lupus erythematosus. SLICC damage index

“…Длительность болезни В многочисленных исследованиях анализируется связь между длительностью СКВ и нарастанием частоты органных повреждений. В большинстве случаев наблюда-ется прогрессивное, линейное нарастание балла ИП по мере увеличения длительности болезни [9][10][11][12][13][14][15][16][17][18][19]. Хотя в не-которых исследованиях можно обнаружить противореча-щие этому данные.…”