Retrospective study of juvenile systemic lupus erythematosus (JSLE) over the last 20 years: single center experience

Results There were 62 children, 52 girls and 10 boys, with the mean age at disease onset (± SD) 12.9 ± 2.4 years. Fiftyeight 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.