Background Lupus disease course has been reported in different patterns: chronic active (CA), relapsing-remitting (RR) and long quiescent (LQ) activity. Only one study evaluated the disease course patterns in children with JSLE (Otten et al., 2010).
Objectives To assess disease activity patterns and their relationship to damage, death and growth failure in JSLE patients.
Methods Chronic active (CA), relapsing-remitting (RR) and long quiescent (LQ) activity patterns were retrospectively classified according to longitudinal scores by Modified SLEDAI-2K and ECLAM during follow up. Pediatric SLICC/ACR Damage Index (Ped/SDI) was scored during the last visit. Growth failure was defined by 2 of the parameters: height-for-age <3rd percentile, speed growth-for-age <3rd percentile and 2 percentiles height fall. Life-table analysis (mortality, damage and growth failure) were plotted and stratified according to CA and RR patterns (Figure). Cox model analysis identified predictors among onset clinical variables.
Results 37 cases, 2.7 years median follow up were reviewed during 781 visits. RR pattern rates were 67.5%, CA 29.8% and 2.7% LQ scored by Modified SLEDAI-2K and 45.9%, 48.7% and 5.4%, respectively scored by ECLAM. The 5-year survival was 90%. Damage accrued in 62.5% and growth failure in 31.3%. Mean time for damage and growth failure was 4.8±0.5 and 5.2±0.4 years, respectively. Five-year probability of damage was 47% and growth failure 31%. CA pattern cases progressed to damage earlier than RR pattern cases (log-rank test, p<0.05). There was association of damage with disease duration (p<0,0001), thrombocytopenia (p<0,05) and alopecia (p<0,004). Growth failure associated with disease duration (p<0,007) and renal failure (p<0,007) at disease onset.
Conclusions LQ course was less frequent compared to adults. Damage occurred earlier in CA pattern. Disease duration, thrombocytopenia and alopecia at disease onset predicted damage.
Otten MH, Cransberg K, van Rossim MA et al. Disease activity patterns in juvenile systemic lupus erythematosus and its relation to erly aggressive treatment. Lupus 2010. 19: 1550-6.
Disclosure of Interest None declared