Investigation of the relationship between disease activity and disease damage in childhood-onset systemic lupus erythematosus

Background. Disease activity measured by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) estimates the degree of inflammation in cSLE patients. The Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) measures damage in adult SLE, but has not been tested in children.; Objectives. (1) To test that accumulated disease activity measured by the area under the curve of the SLEDAI (AUCS) is related to the damage scored by the SDI. (2) To identify additional damage predictors in cSLE.; Methods. Sixty-six patients were assessed for disease activity (SLEDAI) and damage (SDI). Linear and multivariable modeling were done to predict SDI scores using the AUCS and/or other possible damage predictors.; Results. The AUCS predicts damage. Multivariable modeling suggests that corticosteroids and antiphospholipid antibodies increase damage and that second-line agents may be protective.; Conclusions. The AUCS is a good predictor of damage in cSLE. Damage is influenced by medications.