Naslov
Juvenile systemic lupus erythematosus (JSLE) in Croatia – A national study over the past 20 years

Autori
Lukić, Anita ; Jelušić-Dražić, Marija ; Malenica, B ; Batinić, D ; Milošević, D ; Vrljičak, K ; Nižić Lj ; Vidović, M ; Rožmanić, V ; Šuvat-Dežulović, M ; Saraga, M ; Metličić, V ; Stričević, L ; Starčević, K ; Malčić, I

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

Izvornik
Meeting abstract / Danica Batinić, Danko Milošević - New York (NY) : Springer, 2011, 1655-1655

Skup
44th Annual Scientific Meeting of the European Society for Paediatric Nephrology

Mjesto i datum
Dubrovnik, Hrvatska, 14.09.2011. - 17.09.2011

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Systemc lupus erythematosus; pediatric; kidney

Sažetak
Aim: To analyze cumulative clinical and laboratory features, and outcome of Croatian children with JSLE, followed between 1987 and 2009.Results: Of 85 children, there were 73 girls and 12 boys, with mean age at disease onset, years ± SD (range), 13.0 ± 2.84 (6–18). The youngest girl was 6, and the youngest boy was 7 years old. The symptoms were present on average 3 months (range 0–96 months) before the diagnosis was made. The commonest presenting clinical features were arthralgias (79%), skin (66%), constitutional (65%), renal (58%), cardiac (21%), and CNS (16%). Proteinuria was present in 57% of cases, but renal biopsy revealed lupus nephritis in 29 (34%) children: class II in 6% class III in 28%, class IV LN 28%, class V in 21%, and class VI in 17% cases. The patients presented significantly altered laboratory parameters including high ESR (91%), some sort of cytopenia (73%), deficiency of complement C3 (62%) and C4 (38%), low total haemolythic complement activity (54%), and circulating immunocomplex (77%). Children had positive antinuclear and other antibodies: anti-dsDNA (74%), anti-SS-A (28%), anti-SS-B (23%), anti-Sm (69%), anti-RNP (44%), anti-Sc70 (40%), anit-histons (83%), aCL-IgM (61%), aCL-IgG (79%), RF (48%) ; while 65% of them had high IgG levels, and AST-O titre (35%). Five patients (6%) also had primary immunodeficiency (selective IgA deficiency). There was no difference in clinical or laboratory features between boys and girls, or depending on the age of disease onset. During the study period two patients died, one because catastrophic antiphospholid syndrome, other because of terminal renal failure.Conclusion: 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