Chronic active disease pattern predicts early damage in juvenile systemic lupus erythematosus

The objective of this article is to assess disease activity patterns and their relationship to damage, death and growth failure in a cohort of juvenile lupus.

Chronic active, relapsing-remitting and long quiescent activity patterns were retrospectively classified according to longitudinal scores of both the Modified SLEDAI-2K and ECLAM. The Pediatric SLICC/ACR Damage Index (Ped-SDI) was scored at the last visit in patients followed more than six months. Survival analysis was performed considering death, damage and growth failure, and stratified according to disease activity patterns. Cox model analysis identified predictors for damage and growth failure among onset clinical variables.

Thirty-seven patients with 11 years mean age at diagnosis and 3.2 years mean follow-up were studied. According to the Modified SLEDAI-2K, activity pattern was 67.5% relapsing-remitting, 29.8% chronic active and 2.7% long quiescent and by ECLAM, 45.9%, 48.7% and 5.4%, respectively. The five-year survival was 90%. Damage accrued in 62.5% and growth failure in 31.3%. Chronic active cases progressed to damage earlier than relapsing-remitting (log-rank test, p < 0.05). Damage was associated with disease duration (p < 0.0001), thrombocytopenia (p < 0.05) and alopecia (p < 0.004). Growth failure was associated with disease duration (p < 0.007) and renal failure (p < 0.007).

Damage was observed in nearly two-thirds of patients, and occurred earlier in the chronic active pattern. Disease duration, thrombocytopenia and alopecia at onset predicted damage.