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LSO-050 Mortality-rate in juvenile-onset systemic lupus erythematosus in perspective: results from a population-based cohort study in Norway
  1. Sigrid Reppe Moe1,
  2. Hilde Haukeland1,2,
  3. Cathrine Brunborg3,
  4. Torild Garen1,
  5. Antonella Botea4,
  6. Nenad Damjanic2,
  7. Gro Wivestad5,
  8. Heidi Kverneggen Øvreås6,
  9. Thea Bøe7,
  10. Anniken Orre8,
  11. Sella Aarrestad Provan9,
  12. Øyvind Molberg1,10 and
  13. Karoline Lerang1
  1. 1Department of Rheumatology, Oslo University Hospital, Norway
  2. 2Department of Rheumatology, Martina Hansen Hospital, Norway
  3. 3Department of Rheumatology, Oslo Center for Biostatistics and Epidemiology, Norway
  4. 4Department of Rheumatology, Betanien hospital, Norway
  5. 5Department of Rheumatology, Sørlandet Hospital, Norway
  6. 6Department of Rheumatology, Revmatismesykehuset, Norway
  7. 7Department of Internal Medicine, Vestfold hospital, Norway
  8. 8Department of Rheumatology, Drammen hospital, Norway
  9. 9REMEDY – Center for treatment of Rheumatic and Musculoskeletal Diseases, Diakonhjemmet hospital, Norway
  10. 10Institute of Clinical Medicine, University of Oslo, Norway


Background There are limited population-based data on morality in juvenile Systemic Lupus Erythematosus (SLE). Here, we examine standard mortality rate (SMR) in juvenile SLE and compare with young- and adult-onset subsets in a large population-based SLE cohort.

Methods The population-based cohort included all SLE patients who were resident in Southeast Norway 1999 – 2017, had SLE diagnosis confirmed by chart-review and met the 1997 ACR criteria for SLE. Cases with new-onset disease 1999–2017 were defined as inception cases. We stratified the cohort by age at diagnosis, with juvenile SLE diagnosed age <16, young-onset age 16–29 and adult-onset age ≥30. Lupus nephritis (LN) was defined by 1997 ACR criteria for SLE. We compared ratios with X2-test, estimated risk of death by SMR using 15 controls per SLE case (individually matched to case by age, sex and ethnicity) and survival in juvenile inception cases by Kaplan-Meier.

Results The cohort included 1300 SLE cases; of whom 93 (7%) were diagnosed at age<16, 461 (35%) at age 16–29 and 746 (57%) at age≥30 (table 1). Juvenile SLE developed significantly more LN than those with later disease-onset (table 1). None of the juvenile inception cases died during follow-up and 10-year survival was 100% (99% in matched-controls).

The SMR was significantly higher in juvenile and young adult-onset than in adult-onset, with highest SMR in juvenile-onset (table 1). SMR in men and women with juvenile-onset was 6.3 (95% CI 0.6–38) and 7.4 (95% CI 3.4–16), respectively. In juvenile SLE, presence of LN increased SMR to 9.2 (95% CI 3.6–22). Correspondingly, in non-LN juvenile patients SMR was 4.3 (95% CI 0.8–16).

Conclusions Early disease-onset greatly increase SMR in SLE to a maximum of 7.2 in juvenile-onset, twice as high as in juvenile type-1 Diabetes.1 We find no sex-specific differences in SMR, but juvenile-onset with LN has the highest SMR.

Abstract LSO-050 Table 1

Patient demographic and mortality in Systemic Lupus Erythematosus; stratified by age at diagnosis


  1. Gagnum V, Stene LC, Sandvik L, Fagerland MW, Njølstad PR, Joner G, et al. All-cause mortality in a nationwide cohort of childhood-onset diabetes in Norway 1973–2013. Diabetologia. 2015;58(8):1779–86.

  • Systemic Lupus Erythematosus
  • Mortality
  • Juvenile SLE

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