| Abstract | Sistemski eritemski lupus (SLE) je autoimunosna, kronična multisistemska bolest nepoznate etilogije koja može zahvatiti sve organske sustave. Bolest se odlikuje raznolikom kliničkom slikom te povremenim egzacerbacijama i remisijama. Imunološki nalazi i klinički simptomi su jednaki u odraslih i u djece, ali djeca imaju mnogo aktivniju bolest, težu kliničku sliku i zahvaćen veći broj organa te veću mogućnost razvoja oštećenja tijekom vremena. Za procjenu aktivnosti bolesti i oštećenja u pacijenata sa SLE-om koriste se upitnici, kao što su SLEDAI-2K (engl. Systemic Lupus Erythematosus Disease Activity Index 2000) i SLICC/ACR indeks oštećenja (engl. Systemic Lupus International Collaborating Clinics/American College of Rheumatology). Cilj našeg istraživanja bio je usporediti SLEDAI-2K indeks aktivnosti u pacijenata sa SLE-om u vrijeme dijagnosticiranja bolesti u dječjoj dobi, sa SLEDAI-2K indeksom aktivnosti u tih istih pacijenata u odrasloj dobi te odrediti SLICC/ACR indeks oštećenja u odrasloj dobi. U istraživanje je uključen dio pacijenata u dobi od 1 do 18 godina, kojima je u razdoblju od 1991.- 2016. godine u Zavodu za imunologiju i reumatologiju, Klinike za pedijatriju, KBC-a Zagreb, utvrđena dijagnoza SLE-a prema ACR kriterijima iz 1997. godine. Od 95-ero djece, u ovo istraživanje uključeno je 48 pacijenata (42 žene i 6 muškaraca) koji su do kraja 3. mjeseca 2017. godine postali punoljetni i nastavili se liječiti u KBC-u Zagreb. U dječjoj dobi prosječna vrijednost SLEDAI-2K indeksa aktivnosti iznosila je 19,25 (7 – 42), a u odrasloj dobi 7,125 (0 – 30). SLICC/ACR indeks oštećenja prisutan je bio u 20 pacijenata (41,67%) sa prosječnom vrijednošću od 1,55 (0 – 5 ). U 32 pacijenta (66,67%) je došlo do poboljšanja bolesti, u 3 pacijenta (6,25%) je došlo do pogoršanja, u 6 pacijenata (12,5%) nije došlo do velikih promjena u aktivnosti, a 7 pacijenata (14,58%) je bilo u remisiji. Katarakta (16,67%), erozivni artritis (6,25%) i avaskularna nekroza (6,25%) su bila najčešća oštećenja koja su se razvila u bolesnika. U dječjoj dobi, prilikom postavljanja dijagnoze bolest je vrlo aktivna, dok u odrasloj dobi dolazi do sniženja aktivnosti bolesti. Veća aktivnost bolesti u dječjoj dobi, povezana je s razvojem kasnijeg oštećenja organa. |
| Abstract (english) | Systemic lupus erythematosus (SLE) is an autoimmune, chronic multisystemic disease of unknown etiology which can interfere with all organic systems. Disease is characterized by a variety of clinical presentation and by periods of flares or remission. Immunological tests and clinical presentations are similar in adults and in children, but children with SLE tend to have a much more active disease at onset, a severe clinical presentation with higher rates of organ involvement, and a greater chance of developing damage over time. Questionnaires, such as SLEDAI-2K (SLE Disease Activity Index 2000) and SLICC/ACR Damage Index (Systemic Lupus International Collaborating Clinics/American College of Rheumatology), are used to evaluate the disease activity and damage in patients with SLE. The aim of our study was to compare the SLEDAI-2K activity index in patients with childhood-onset SLE at the time of diagnosis with the SLEDAI-2K activity index in these same patients in adulthood, and to determine the SLICC/ACR damage index in adulthood. The study included a part of patients aged 1 to 18 years, who were diagnosed with SLE according to ACR criteria at the Department of Paediatrics, University Hospital Centre Zagreb, Croatia during the period of 1991 - 2016. Out of 95 children, this study included 48 patients (42 women and 6 men) who reached the age of 18 until the end of March 2017, and continued to be treated in University Hospital Centre Zagreb. In childhood, the average SLEDAI-2K score was 19.25 (7-42) and in adult age 7,125 (0-30). The SLICC / ACR damage index was present in 20 patients (41.67%) with an average value of 1.55 (0-5). In 32 patients (66.67%) there was an improvement in the disease, 3 patients (6.25%) had deteriorated, 6 patients (12.5%) had no major changes in activity and 7 patients ( 14.58%) was in remission. Cataract (16.67%), erosive arthritis (6.25%) and avascular necrosis (6.25%) were the most common items of damage developed in the patients. Children with SLE have very active disease at diagnosis, while in the adult age the disease activity is reduced. More active disease in childhood is associated with the development of organ damage over time. |
