Naslov
Childhood-Onset Systemic Lupus Erythematosus over the Last 25 Years: Predicting Organ Damage.
(Childhood-Onset Systemic Lupus Erythematosus over the Last 25 Years: Predicting Organ Damage)

Autori
Cekada, Nastasia ; Sestan, Mario ; Hosticka, Emilija ; Novoselec, Maja ; Batnozic Varga, Mateja ; Padjen, Ivan ; Frkovic, Marijan ; Kifer, Domagoj ; Anic, Branimir ; Batinic, Drago ; Potocki, Kristina ; Malcic, Ivan ; Jelusic, Marija

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

Izvornik
Reumatizam. 2018 ; 65(Suppl. 1) / - Zagreb : Hrvatsko reumatološko društvo, 2018, 16-16

Skup
Central European Congress of Rheumatology 2018 (CECR)

Mjesto i datum
Zagreb, Hrvatska, 06.12.2018. - 08.12.2018

Vrsta sudjelovanja
Ostalo

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
sistemski eritemski lupus ; oštećenje ; aktivnost ; djeca
(lupus erythematosus, systemic ; damage ; activity ; child)

Sažetak
Background: Childhood-onset systemic lupus erythematosus (cSLE) is a chronic autoimmune disease with the course oft en more severe than in adults, and the activity of the disease widely being evaluated by SLE Disease Activity Index (SLEDAI 2K) and damage by the SLICC/ACR damage index (SDI). Objectives: To explore the correlation between the SLEDAI-2K disease activity index at the time of diagnosis and the SLICC/ACR damage index of patients at their last follow up and to predict the risk of organ damage occurrence in time. Methods: Th e retrospective study included children treated for cSLE from January 1991 to September 2017 at Department of Pediatrics, UHC Zagreb. All children were diagnosed according to the ACR 1997 and SLICC 2012 criteria. Results: Th e disease development of 93 children (74 females) with cSLE was examined in this study. Th e median (range) follow up time was 7 (0.5–24) years and the median (range) age at diagnosis was 13 (5– 19) years. Mean (SD) SLEDAI- 2K was 18.3 (9.0) at the disease onset. 35 children (38 %) had organ damage at the last follow up with the median (range) SDI 0 (0–7). Th e fi rst organ systems damaged in aff ected patients were renal (28%), musculoskeletal (22%), ocular (19%), neuropsychiatric (17%), cardiovascular (11%) and peripheral vascular (2.8%). A statistically signifi cant positive correlation was found between SLEDAI-2K at the disease onset and SDI (τb = 0.252, p = 0.003). No signifi cant correlation was determined between the duration of the disease (τb = 0.042, p = 0.628) or follow up period (τb = 0.111, p = 0.191) and SDI, nor in SDI in regard to gender (p= 0.574). Using Kaplan-Meier method we estimated the decrease in ratio of patients without organ damage since diagnosis or the occurrence of the fi rst symptoms. Since the estimated ratio of patients with organ damage at the endpoint was less than 50%, we could not estimate the time required for damage development in 50% of patients. However, we are 95% sure that the damage is not happening in the fi rst 9 years aft er diagnosis. Conclusions: Th e high correlation detected between SLEDAI-2K and SDI indicated that the presentation of the cSLE at onset can be prognostic of the course and long-term prognosis of lupus. Our fi ndings suggest that it is unlikely that organ damage will occur in 50% of patients in the fi rst nine years of the disease course.

Izvorni jezik
Engleski

Napomena
Sažetak objavljen u časopisu Reumatizam. 2018 ;
65:16, indeksiranom u bazi podataka Embase (Scopus)

POVEZANOST RADA