Naslov
Comparison of different classification criteria for systemic juvenile idiopathic arthritis

Autori
Held, Martina ; Kifer, Nastasia ; Šestan, Mario ; Frković, Marijan ; Jelušić, Marija

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

Izvornik
Abstract Book. The International Society of Systemic Auto-inflammatory Diseases Annual Meeting 2023 / - , 2023, 98-98

Skup
The International Society of Systemic Auto- inflammatory Diseases Annual Meeting 2023

Mjesto i datum
Toronto, Kanada, 15.05.2023. - 18.05.2023

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Classification criteria ; systemic juvenile idiopathic arthritis

Sažetak
Introduction: Systemic JIA is a disease of complex pathogenesis that initially includes elements of disorders of innate immunity in the sense of its excessive and inappropriate activation, according to which it belongs to autoinflammatory diseases, characterized by multiorgan inflammatory process which in approximately 10-25% of children is complicated by a life-threatening condition - macrophage activation syndrome (MAS). Objectives: To compare different classification criteria for sJIA and to analyze clinical and laboratory parameters of the patients. Methods: The study included children who were diagnosed with sJIA in the period from 2001 to 2022 according to three classifications: ILAR, Yamaguchi and proposed PRINTO. Data are presented descriptively, and differences between variables were examined using Fisher's exact test and the Mann-Whitney U test. Results: sJIA was diagnosed in 56 patients, 36 boys and 20 girls with average age 9.3±5.7 years at the time of diagnosis. Applying the ILAR criteria the diagnosis of sJIA meets 47 patients (83, 9%), while all patients met the Yamaguchi and proposed PRINTO classification criteria. A total of three major PRINTO criteria: fever, arthritis and rash were present in 29 patients (51, 8%) ; 18 patients (32, 1%) had fever and arthritis with at least one of the 2 minor criteria (lymphadenopathy, hepatomegaly, splenomegaly, serositis, leukocytosis > 15x109 /L), while 9 patients (16, 1%) had fever associated with rash with at least 2 minor criteria. Disease relapse occurred in 20 patients (35, 7%). 41 patients (73, 2%) received pulse doses of glucocorticoids, 28 patients (50%) were treated with methotrexate, and 14 patients (25%) with biologic agents (interleukin-1 and interleukin-6 inhibitors). 8 patients (14, 3%) received intravenous immunoglobulins (IVIG), while 6 patients (10, 7%) were treated with immunosuppressants. According to 2016 EULAR/ACR/PRINTO classification criteria for MAS a total of 10 patients (17, 8%) developed this most severe complication of the disease. Compared to others, patients with MAS had significantly higher values of ferritin (median 7003.9 μg/L vs. 355.6 μg/L, p=0.014), aspartate aminotransferase (median 65 U/L vs. 29 U/L, p<0.001) and lactate dehydrogenase (median 716 U/L vs. 259 U/L, p<0.001), lower levels of fibrinogen (median 3.4 g/L vs. 6.4 g/L, p=0.007) and hemoglobin (median 93.5 g/L vs. 110.5 g/L, p=0.019) and were more often treated with biologics (p=0.011) and IVIG (p=0.027). Conclusion: Comparing the three classifications for sJIA, we found a difference in the number of patients who would meet the criteria for the diagnosis of the disease, primarily resulting from arthritis which is an obligatory diagnostic criterion in the ILAR, but not in the Yamaguchi and proposed PRINTO classifications. The application of the latter criteria makes it possible to make a diagnosis in the initial phase of the disease, with a predominant autoinflammatory phenotype, and thereby take advantage of the "window of opportunity" for appropriate treatment.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA