Naslov
Risk Score of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis

Autori
Simone Carbogno ; Denise Pires Marafon ; Giulia Marucci ; Manuela Pardeo ; Antonella Insalaco ; Virginia Messia ; Emanuela Sacco ; Ferhat Demir ; Betul Sozeri ; Nastasia Čekada ; Marija Jelusic ; Olga Vougiouka ; Mikhail Kostik ; Alenka Gagro ; Christoph Kessel ; Francesca Minoia ; Fabrizio De Benedetti ; Claudia Bracaglia

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

Izvornik
Arthritis and Rheumatology Abstract Supplement / - , 2019

Skup
2019 ACR/ARP Annual Meeting

Mjesto i datum
Atlanta (GA), Sjedinjene Američke Države, 08.11.2019. - 13.11.2019

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Risk Score ; Macrophage Activation Syndrome ; Systemic Juvenile Idiopathic Arthritis

Sažetak
Background/Purpose : Macrophage Activation Syndrome (MAS) is a severe, life- threatening, complication of rheumatic diseases in childhood, particularly of systemic Juvenile Idiopathic Arthritis (sJIA), occurring in approximately 25% of the patients with sJIA. The mortality rate of MAS is still signifi cantly high. A score that identify sJIA patients who are at high risk to develop MAS would be useful in clinical practice. There are no parameters available to identify from onset sJIA patients with high risk to develop MAS in their disease course. Methods : We evaluated whether routine laboratory parameters at disease onset may predict the development of MAS in patients with active sJIA and we defi ned a risk score of MAS for sJIA patients using these parameters. Laboratory parameters of disease activity and severity (WBC, N, PLT, Hb, ferritin, AST, ALT, gGT, LDH, TGL, fi brinogen, D- dimer and CRP), were retrospectively evaluated in 85 sJIA patients referred to our Division of Rheumatology from 1998 to 2018 with at least one year of follow- up. Laboratory parameters were evaluated during active sJIA, without MAS, at time of hospitalization (T1) and immediately before treatment for sJIA was started (T2). Patients were divided in two groups: group 1 (sJIA patients without history of MAS), group 2 (sJIA patients with at least one MAS episode during disease course). To calculate a MAS risk score, laboratory parameters, collected at T2, with a statistically signifi cant difference between the two groups of patients were selected. Results : Thirty- two patients, that fulfi lled the 2016 classifi cation criteria for MAS [1] at time of sampling, were excluded from the analysis. Therefore, we analysed laboratory parameters of 53 patients with sJIA, 33 of whom without history of MAS (group 1) and 20 who developed at least one episode of MAS during disease course (group 2). Levels of ferritin, AST, LDH, gGT and TGL, collected at T2, were statistically signifi cant higher in patients with a history of MAS compared to those without a history of MAS. For each of these parameters an arbitrary cut- off was defi ned. In order to define the final score an arbitrary rate was attributed to each parameter (Table1). Sensitivity (Se), specifi city (Sp), positive predictive value (PPV) and negative predictive value (NPV) were calculated to defi ne the best scoring system. The scoring system with the best sensitivity was chosen (Table 2). A MAS risk score >3 identifi ed 19 out of 20 sJIA patients with a history of MAS and 5 out of 33 sJIA patients without history of MAS. In order to validate the MAS risk score on a different population, we applied the score on 47 patients from other Paediatric Rheumatologic centres, 29 without history of MAS and 18 with at least one episode of MAS. Sensitivity and specifi city of the score are reported in table 3. Conclusion : In conclusion we developed a MAS risk score based on routine laboratory parameters, available worldwide, that can help clinicians to identify these patients early in the disease course. The initial validation analysis is promising but we need to validate the score on a larger population.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

Napomena
Volume 71, number s10

POVEZANOST RADA