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. 2024 Aug 1;63(8):2109-2117.
doi: 10.1093/rheumatology/kead519.

Persisting mortality gap in systemic lupus erythematosus; a population-based study on juvenile- and adult-onset SLE in Norway 1999-2022

Sigrid Reppe Moe  1   2 , Hilde Haukeland  1   2   3 , Cathrine Brunborg  4 , Antonela Botea  5 , Nenad Damjanic  3 , Gro Årthun Wivestad  6 , Heidi Øvreås  7 , Thea Bøe  8 , Anniken Orre  9 , Torhild Garen  1 , Vibke Lilleby  1 , Sella A Provan  10   11 , Øyvind Molberg  1   2 , Karoline Lerang  1
Affiliations

Persisting mortality gap in systemic lupus erythematosus; a population-based study on juvenile- and adult-onset SLE in Norway 1999-2022

Sigrid Reppe Moe et al. Rheumatology (Oxford). .

Abstract

Objective: To estimate mortality and survival rates of SLE in a contemporary, population-based setting and assess potential influences by time, sex, ethnicity, classification criteria and age at diagnosis.

Methods: We assessed mortality and survival in the Nor-SLE cohort, which includes all chart review-confirmed SLE cases resident in Southeast Norway (population 2.9 million) 1999-2017. Study end was at death, emigration or 1 October 2022. We defined juvenile SLE by age <16 years at diagnosis. For standardized mortality rate (SMR) estimates, we applied 15 population controls per case, all matched for age, sex, residency and ethnicity. We analysed survival by Kaplan-Meier and risk factors by Cox regression.

Results: The Nor-SLE cohort included 1558 SLE cases, of whom 749 were incident and met the 2019 EULAR and ACR (2019-EA) classification criteria. SMR was increased to 1.8 (95% CI 1.6-2.2) in incident adult-onset SLE but did not differ between females and males. Survival rates at 5, 10, 15 and 20 years were lower in incident adult-onset SLE than in matched controls. In multivariable analysis, LN associated with decreased survival, while sex did not. Separate, long-term mortality analyses in the total Nor-SLE cohort showed that SMR peaked at 7.2 (95% CI 3.3-14) in juvenile-onset SLE (n = 93) and fell gradually by increasing age at SLE diagnosis.

Conclusion: This study shows persistence of a mortality gap between adult-onset SLE and controls at population level and provides indications of worryingly high mortality in juvenile-onset SLE.

Keywords: SLE; childhood; epidemiology; juvenile; long-term outcome; lupus nephritis; mortality; sex.

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Figures

Figure 1.
Figure 1.
Age-specific SMR in new-onset SLE cases 1999–2017. Age-specific SMR in (A) all, (B) females and (C) males with disease-onset 1999–2017 when individually matched to population controls by sex, age, residency and ethnic ancestry. The analyses are stratified by classification criteria applied. ACR-97: 1997 ACR classification criteria for SLE; EA-2019: 2019 EULAR/ACR classification criteria for SLE; SMR: standardized mortality rates
Figure 2.
Figure 2.
Estimated 20-year survival in new-onset SLE vs matched controls in all (A), females (B) and males (C). All cases had disease-onset 1999–2017 and fulfilled the 2019 EULAR/ACR classification criteria for SLE. Controls were individually matched per case by sex, age, residency and ethnic ancestry
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