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.

Lupus
|August 23, 2007
PubMed

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.

Related Concept Videos