engleski

Retrospective study of juvenile systemic lupus erythematosus (JSLE) over the last 20 years

Aim was to analyze characteristics of the presenting and cumulative clinical features, immunologic manifestations, disease activity and outcome of 62 Croatian children with JSLE, followed between 1987 and 2007. There were 62 children, 52 girls and 10 boys, with the mean age at disease onset (± ; SD) 12.9± ; 2.4 years. Fifty-eight patients were followed for a mean period of 6.9± ; 5.3 yrs. 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 9 (27, 3%), class II LN in 5 (15, 1%) and class V LN in 4 (12, 1%) cases. The patients presented significantly altered laboratory parameters including deficiency of complement C3 (93%) and C4 (95%), high ESR (95%), cytopenia (73%) and positive anti-dsDNA (100%). Only two patients had severe opportunistic infections: CNS 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 catastrophic antiphospholid syndrome, other because of terminal renal failure. There is no significant difference in clinical, immunopathological features and therapy regimens in our patients compared to those in most paediatric SLE studies.

SLE; children

Rad je kao poster prezentiran i na skupu 15th European Paediatric Rheumatology Congress, održanom od 12.-15.09.2008.g., London, Velika Britanija ; Knjga sažetaka objavljena u casopisu Pediatric Rheumatology (2008) str. 380-380.