engleski

Childhood-onset systemic lupus erythematosus : a review of 62 patients followed at a single Croatian center

We aimed to review our experience with childhood systemic lupus erythematosus (SLE). There were 62 children, 52 girls and 10 boys. The mean age at disease onset was 12.9 +/- 2.4 years. Renal involvement was present in 33 (53.2%) children ; 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. Based on clinical presentation and presence of renal involvement, patients were treated with corticosteroids alone or combined with cytotoxic drugs. Plasmapheresis or mycophenolate mofetil was used in 5 and 2 patients, respectively. Fifty-eight patients were followed for a mean period of 6.9 +/- 5.3 years. At last clinical evaluation, all 29 patients without renal involvement, except one who died with catastrophic antiphospholipid syndrome and CNS involvement, were in complete remission. Among 29 children with LN, 18 (62.0%) were in complete remission, 4 (13.9%) had clinically active disease but with normal renal function, and 7 (24.1%), 5 with class IV, 1 with class III and 1 with class V LN, had an adverse outcome, either preterminal (2) or terminal renal failure (2) or death (3). Five-year patients and kidney survival rates from the time of diagnosis to the endpoints of terminal failure or death were 91.2% and 90.7%, respectively in the whole LN group, and 88.9% and 80.9%, respectively, in the class IV LN group. Like in other pediatric series the prognosis of SLE in children is primarily dependent on the presence of renal involvement. The class IV LN has worse prognosis than other histopathological categories.

systemic lupus erythematosus; children; kidney

nije evidentirano