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Measuring permanent damage in pediatric systemic lupus erythematosus
L T Hiraki1, J Hamilton, E D Silverman
1Division of Rheumatology, Hospital for Sick Children, University of Toronto, Toronto, Canada.
Insights
Pediatric systemic lupus erythematosus (pSLE) survival has improved, leading to chronic disease management. This study reviews the Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SDI) for pSLE, suggesting pediatric-specific modifications.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Clinical Outcomes Assessment
Background:
- Pediatric systemic lupus erythematosus (pSLE) survival rates have significantly improved.
- Increased life expectancy in pSLE patients necessitates evaluating long-term disease and therapy consequences.
- Quality of life and therapeutic intervention effectiveness require robust outcome measures.
Purpose of the Study:
- To review the development and application of the Systemic Lupus International Collaborative Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (SDI) in pSLE.
- To critically appraise the SDI's limitations in measuring permanent damage in pediatric populations.
- To propose modifications and additions to the SDI for better pediatric relevance.
Main Methods:
- Review of the Systemic Lupus International Collaborative Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (SDI) development and literature.
- Analysis of SDI application and damage profiles in pediatric systemic lupus erythematosus (pSLE) cohorts.
- Critical appraisal of SDI's suitability for pediatric chronic disease assessment.
Main Results:
- The SDI has been applied to assess damage in pediatric systemic lupus erythematosus (pSLE).
- Limitations exist in the current SDI for accurately reflecting permanent damage in children.
- Specific pediatric issues like growth and puberty are not adequately addressed by the SDI.
Conclusions:
- The SDI requires modifications to better capture the damage spectrum in pediatric systemic lupus erythematosus (pSLE).
- Proposed additions include domains for decreased final height and delayed puberty.
- Suggested modifications to existing SDI domains (gonadal failure, diabetes mellitus, cognitive impairment, osteonecrosis) are needed for pediatric relevance.
Abstract:
The survival rates in pediatric systemic lupus erythematosus (pSLE) have improved greatly over recent decades. Increased life expectancy has meant that more children are growing up with the consequences of chronic disease and prolonged therapy. Assessing complications of disease and its therapy becomes an important outcome measure by which to evaluate our therapeutic interventions and appraise quality of life. In this paper we review the development of the Systemic Lupus International Collaborative Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (SDI) and its application to the pSLE population. We examine the profile of damage in pSLE as identified by the SDI. However we also critically appraise its application and identify potential limitations in the SDI as a measure of permanent disease damage in children. In this paper we put forth suggestions for additional domains addressing pediatric specific issues such as decreased final height and delayed puberty. We also suggest modifications to domains of gonadal failure, diabetes mellitus, cognitive impairment and osteonecrosis in the SDI to make it more reflective of the damage phenomenon observed in pediatrics.