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

Autori
Batinić, Danica ; Bukovac, Lana ; Ščukanec-Špoljar, Mira ; Milošević, Danko ; Nižić, Ljiljana ; Vrljičak, Kristina ; Jelušić, Marija

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Abstracts of European Paediatric Nephrology Congress ; u: Pediatric Nephrology 24 (2009) (S) / - , 2009, 088-088

Skup
European Paediatric Nephrology Congress

Mjesto i datum
Francuska, 03.06.2009. - 06.06.2009

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
SLE; childhood

Sažetak
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. 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.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA