Flares in pediatric systemic lupus erythematosus

OBJECTIVE: To determine the flare rate and the change in Safety of Estrogens in Lupus Erythematosus: National Assessment Systemic Lupus Erythematosus Disease Activity Index (SELENA SLEDAI) score with disease flare in pediatric systemic lupus erythematosus (pSLE). METHODS: A retrospective chart review of 62 patients with pSLE (ages 5-20 yrs). A flare was defined as the start of, or increase in, the dose of corticosteroids and/or the addition of an immunosuppressive medication. All pre-flare, flare, and post-flare visits were recorded with a SELENA SLEDAI score calculated for each visit. The flare rate was calculated by dividing the total number of flares in the cohort by the total followup years. RESULTS: Sixty-two patients were eligible. Forty-seven patients had 112 flares. The average number of flares/patient was 1.8 +/- 2.0 and the mean inter-flare time was 15.4 +/- 17.9 months. The flare rate in pSLE was 0.46 flares/patient-year of followup. The median time to first flare from the date of diagnosis was 14.3 months. Patients with cytopenia, pleuritis, or pericarditis, or a positive antibody to Smith nuclear antigen at the time of diagnosis had a significantly higher flare rate than those who did not. The average SELENA SLEDAI score at presentation was 12.5 +/- 5.4, at the pre-flare visit 6.3 +/- 3.5, and during a flare 7.9 +/- 5.1. CONCLUSION: This is the first large study to report a flare rate (0.46 flares/patient-year of followup) in pSLE. The flare rate was similar to what has been reported in pSLE previously but significantly lower than that reported in adults with lupus. The average change in the SELENA SLEDAI score with disease flare is 2 points.