Article Text
Abstract
Background The long-term morbidity and mortality of childhood-onset SLE (cSLE) after transition to adult care is not well-documented. The present study aims to fill this knowledge gap by analyzing outcomes in a large province-wide cSLE cohort. Our objectives were to: 1) determine all-cause and cause-specific mortality rates, adverse renal event rates, cardiovascular event and cancer rates; and 2) determine baseline characteristics associated with higher rates of transition between 3 different states: event-free, adverse renal event, and death.
Methods Clinical data were abstracted for cSLE patients diagnosed between January 1990 and March 2011 after contacting all pediatric and adult rheumatologists and nephrologists in Ontario. Data were linked to administrative healthcare databases at ICES to determine the outcomes of interest. We examined descriptive summaries of major outcomes including death, end-stage kidney disease [ESKD] requiring chronic dialysis and renal transplant, cardiovascular events and cancer. We used a multi-state Cox model to determine baseline characteristics associated with higher rates of transition between the 3 states (figure 1).
Results There were 37 deaths in a cohort of 601 patients at a mean follow-up time of 14 years. The all-cause mortality rate was 3.43 per 1000 person-years. The rates for ESKD requiring chronic dialysis and renal transplant were 5.34 and 2.16 per 1000 person-years, respectively. The rates for any type of cardiovascular event and cancer were 6.32 and 3.13 per 1000 person-years, respectively. The multi-state model indicated that the non-white ethnic group (HR, 2.15; 95% CI, 1.14–4.08) and the presence of renal involvement at baseline (HR, 2.15; 95% CI, 1.17–3.95) were significantly associated with higher rates of transition from event-free to adverse renal event.
Conclusions In this large multi-ethnic cSLE cohort, ethnicity was associated with adverse outcomes including renal events and death. Further analyses will help inform risk for adverse outcomes to improve clinical care for the highest risk patients.
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