| Abstract | Uvod: Sistemski tip juvenilnog idiopatskog artritisa (sJIA) je bolest složene patogeneze s elementima autoinflamatorne i autoimunosne bolesti. Bolest obilježavaju sistemski znakovi i simptomi upale uz artritis koji se često javlja kasnije u tijeku bolesti. Životno ugrožavajuća komplikacija ove bolesti koja nastaje u 10% bolesnika je sindrom aktivacije makrofaga (MAS). Cilj ovog istraživanja jest usporediti različite klasifikacijske kriterije za postavljanje dijagnoze sJIA-e i prikazati klinička obilježja ispitanika.
Metode i ispitanici: U ovo istraživanje uključeno je 60 ispitanika u dobi 1-18 godina kojima je postavljena dijagnoza sJIA-e 2001.-2022. prema ILAR i PRINTO klasifikaciji u Referentnom centru za pedijatrijsku i adolescentnu reumatologiju Republike Hrvatske, KBC-a Zagreb. Podaci su prikazani deskriptivno, a razlike između varijabli su analizirane pomoću Fisherovog egzaktnog testa i Mann Whitney U testa.
Rezultati: Dijagnoza sJIA-e postavljena je kod 60 ispitanika, 38 dječaka i 22 djevojčice, prosječne dobi pri dijagnozi od 9,3 godine. Dijagnoza je po ILAR klasifikaciji postavljena kod 43 ispitanika (71,7%), po Yamaguchijevoj klasifikaciji kod 57 ispitanika (95,0%), po PRINTO klasifikaciji kod 56 ispitanika (93,3%). 22 su ispitanika razvila relaps (36,7%), a 9 ih je razvilo MAS (15,0%). Ispitanici koji su razvili MAS češće su liječeni biološkom terapijom (p=0,005). Također su imali povišen feritin (p<0,001), AST (p<0,001), LDH (p<0,001), trigliceride (p=0,003) i snižen hemoglobin (p=0,068) i fibrinogen (p=0,002). Pulsnu glukokortikoidnu terapiju je primilo 43 ispitanika (71,7%). Terapiju metotreksatom je primio 31 ispitanik (51,7%). Imunosupresivnu terapiju je primilo 6 ispitanika (10%). Terapiju IVIG-ma je primilo 9 ispitanika (15%). Biološku terapiju je primilo 15 ispitanika (25%).
Zaključak: Uspoređujući klasifikacijske kriterije za sJIA-u utvrdili smo razliku u broju bolesnika koji bi zadovoljili kriterije za dijagnozu bolesti što ponajprije proizlazi od artritisa koji je neophodan kriterij u ILAR-ovoj, ali ne i u Yamaguchijevoj i PRINTO-ovoj klasifikaciji. Primjena potonjih kriterija omogućuje postavljanje dijagnoze u početnoj fazi bolesti kod prevladavajućih sistemskih autoinflamatornih obilježja što pruža više terapijskih mogućnosti. |
| Abstract (english) | Uvod: Systemic juvenile idiopathic arthritis (sJIA) is an autoinflammatory and autoimmune disease. Patients present with both extraarticular symptoms and arthritis. Arthritis often occurs later in the disease course. Macrophage activation syndrome (MAS) is a life-threatening complication in sJIA, occuring in 10% of patients. The aim of this study was to compare different classification criteria for sJIA and to present clinical findings.
Methods: This study included 60 patients who were 1-18 years old and were treated for sJIA at Referral centre for paediatric and adolescent rheumatology at the University Hospital Zagreb, 2001-2022. Descriptive data analysis was used for statistical analysis. Fisher's exact test and Mann Whitney's U test were applied.
Results: 60 patients were diagnosed with sJIA, 38 boys and 22 girls for whom the average age at diagnosis was 9,3 years old. According to ILAR classification 43 patients were diagnosed with sJIA (71,7%). According to Yamaguchi classification 57 patients were diagnosed with sJIA (95,0%). According to PRINTO classification 56 patients were diagnosed with sJIA (93,3%). Relapse was developed by 22 patients (36,7%) and MAS was developed by 9 (15,0%) patients. Patients who developed MAS were often treated with biologic therapy (p=0,005) and have had increased ferritin (p<0,001), AST (p<0,001), LDH (p<0,001) and triglyceride (p=0,003) values. They also have had decreased haemoglobin (p=0,068) and fibrinogen (p=0,002) values. 43 patients have received pulse doses of glucocorticoids (71,7%). 31 patients have received methotrexate (51,7%). 6 patients have received immunosuppressants (10%). 9 patients have received intravenous immunoglobulins (15%). 15 patients have received biologic therapy (25%).
Conclusion: While comparing the three classifications we discovered that different number of patients can be diagnosed according to different classifications. Smaller number of patients were diagnosed with sJIA according to ILAR classification because arthritis is an obligatory crterion in this classification but isn't in the other two. Hence, the application of PRINTO and Yamaguchi criteria brings about earlier diagnosis and better treatment options for sJIA. |
