Introduction Children represent approximately 15–20% of all systemic lupus erythematosus (SLE) patients and they usually have a more severe disease at onset, higher rates of organ involvement and a more aggressive clinical course than adults.

Objective To analyse characteristics of the clinical features, immunological manifestations, disease activity and outcome of 62 Croatian children with juvenile SLE, followed between 1987 and 2007.

Methods and Results There were 62 children, 52 girls and 10 boys, with a mean age at disease onset (±SD) of 12.9 ± 2.4 years. Fifty-eight patients were followed for a mean period of 6.9 ± 5.3 years. The commonest presenting clinical features were constitutional (fever, fatigue) (68%), arthralgias (56%), renal involvement (53%) and malar rash (29%). Renal biopsy revealed class IV lupus nephritis (LN) in 15 (45.5%), class III LN in nine (27.3%), class II LN in five (15.1%) and class V LN in four (12.1%) cases. The patients presented significantly altered laboratory parameters, including deficiency of complement C3 (93%) and C4 (95%), high erythrocyte sedimentation rate (95%), cytopenia (73%) and positive anti-dsDNA (100%). Only two patients had severe opportunistic infections: central nervous system nocardiosis and multifocal staphylococcal osteomyelitis, both with good outcome. Due to clinical presentation and laboratory data most patients were treated with oral corticosteroids, followed by cyclophosphamide, pulse steroid, hydroxychloroquine and azathioprine. During the study period two patients died, one because of catastrophic antiphospholipid syndrome, the other from terminal renal failure.

Conclusion There is no significant difference in clinical, immunopathological features and therapy regimens in our patients compared with those in most paediatric SLE studies.