Impact of the longitudinal quantitative assessment of juvenile systemic lupus erythematosus severity on the disease outcome

Abstract

Objectives

This study on juvenile SLE patients aimed to evaluate retrospectively the impact of a tertiary center’s management policy of the disease severity on its long-term progression and cumulative damage development as well as provision of quality-driven medical care (QmC).

Methods

Disease activity was assessed by the Physician Global Assessment and SLEDAI-2K, flares by SELENA/SLEDAI, and damage by the pediatric SLICC/DI at diagnosis, 6 months post-diagnosis, and annually thereafter. At the same time, QmC was evaluated by relevant indices and quality of life was captured by the Greek version of the General Health Questionnaire only at the last visit.

Results

A total of 35 patients (25/35 females) aged at diagnosis 5.5–15.16 years (median 11.83) with a median lag time to diagnosis 1.8 months had a follow-up of 5 (35/35) and 10 years (13/35), respectively. The predominant baseline manifestations were consistent with those previously reported. Out of 35 patients, 24 (68.5%) were clinically inactive at year 5, and 5/13 (38%) at year 10. All patients received immunosuppressives and 7/35 biologics in addition. At the end of their follow-up, damage was found in 9/35 patients, but none of them had a neuropsychiatric disorder. Over the study, 28/35 patients were compliant with the QmC recommendations.

Conclusions

An early diagnosis combined with a longitudinal quantitative assessment of the disease activity and severity contributes to the continuous evaluation of the disease state. They are the key determinants for the selection of an early, targeted, and personalized management; they restrict the cumulative damage development and contribute to an optimal outcome.

Key Points
Juvenile SLE has a heavier introductory profile than in adults and an unpredictable trajectory.
The application of contemporary metric tools for assessing the disease state leads to an objective assessment and regimen selection.
An early diagnosis combined with longitudinal quantitative assessment is a key determinant for an optimal management and a minimal damage development.

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Correspondence to Maria Trachana.

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The study was conducted in accordance with the Declaration of Helsinki and approved by the hospital’s ethics committee.

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Trachana, M., Pratsidou-Gertsi, P., Kanakoudi-Tsakalidou, F. et al. Impact of the longitudinal quantitative assessment of juvenile systemic lupus erythematosus severity on the disease outcome. Clin Rheumatol 40, 675–682 (2021). https://doi.org/10.1007/s10067-020-05252-8

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Keywords

  • Childhood-onset SLE
  • jSLE clinimetric tools
  • jSLE outcome
  • jSLE quality-driven care
  • jSLE trajectory assessment
  • Pediatric SLE