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LSO-103 Childhood-onset systemic lupus erythematosus: long-term outcomes in a large multi-ethnic Ontario cohort
  1. Ha-Seul Jeoung1,
  2. Kuan Liu2,
  3. Roberta Berard3,
  4. Wesley Fidler4,
  5. Janet Pope5,6,
  6. Johannes Roth7,
  7. Carter Thorne8,
  8. Andrea Knight9,10,
  9. Linda Hiraki9,10,
  10. Earl Silverman9,10 and
  11. Deborah Levy9,10
  1. 1Data and Analytic Services, ICES, Canada
  2. 2Institute of Health Policy, Management and Evaluation, University of Toronto, Canada
  3. 3Rheumatology, Children’s Hospital of Western Ontario, Canada
  4. 4Rheumatology, St. Joseph’s Hospital, Canada
  5. 5Rheumatology, St. Joseph’s Health Care, Canada
  6. 6Rheumatology, University of Western Ontario, Canada
  7. 7Rheumatology, University of Ottawa, Canada
  8. 8Rheumatology, Southlake Regional Health Centre, Canada
  9. 9Rheumatology, The Hospital for Sick Children, Canada
  10. 10Paediatrics, University of Toronto, Canada

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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This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

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