Measuring permanent damage in pediatric systemic lupus erythematosus

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.