engleski

Demographic, clinical and laboratory features of juvenile systemic lupus erythematosus in Croatia and their association with delayed diagnosis: A 20-year retrospective study of 81 children

Juvenile systemic lupus erythematosus (JSLE) presents with diverse clinical features and it often presents atypically, which may delay diagnosis. Such a delay is tightly linked to morbidity and mortality. Aim was to identify predictors of delayed diagnosis and analyze the incidence of JSLE and its clinical and laboratory features in Croatia between 1991 and 2010. Medical records at three university-based tertiary care centers were analyzed retrospectively. A total of 81 children with JSLE (68 girls) were included in the study. Median time from symptom onset to diagnosis was 2 months (range 0-96). Time to diagnosis was inversely associated with ECLAM score (P<0.001), but it showed no association with age, gender, clinical features or distance from the nearest pediatric center. Mean age at onset was 13.42.8 yr (range 6-18), and annual incidence varied from 1 to 15 per million at risk. The most frequent clinical and laboratory features were musculoskeletal symptoms (80%) and increased erythrocyte sedimentation rate (96%). The most frequent immune system anomalies were the presence of antibodies against histones (86%), double-stranded DNA (73%), and Sm protein (64%), as well as low levels of C3 complement (69%). Hematuria was present in 58% of children ; proteinuria in 56% ; and biopsy-confirmed lupus nephritis, in 43%. This is the first large-scale, in-depth study of clinical and laboratory presentation of JSLE in Croatia. Among all the demographic, laboratory or clinical features examined, ECLAM score alone was associated with time to diagnosis. This study emphasizes the need to identify other factors delaying diagnosis, to detect children with JSLE early in the course of the disease.

juvenile systemic lupus erythemathosus; Croatia; predictors of delayed diagnosis; incidence

DOI: 10.1177/0961203313476777