Risk Score of Macrophage Activation Syndrome (MAS) in Patients with Systemic Juvenile Idiopathic Arthritis (sJIA)

Simone Carbogno¹, Denise Pires Marafon², Giulia Marucci², Emanuela Sacco², Manuela Pardeo², Alhanouf Alsaleem³, Sarka Fingerhutova⁴, Ferhat Demir⁵, Nastasia Cekada⁶, Mikhail Kostik⁷, Christoph Kessel⁸, Olga Vougiouka⁹, Alenka Gagro¹⁰, Jessica Tibaldi¹¹, Francesca Minoia¹², Ilaria Maccora¹³, Rayfel Schneider¹⁴, Pavla Dolezalova¹⁵, Betul Sozeri¹⁶, Marija Jelusic⁶, Antonella Insalaco², Fabrizio De Benedetti¹⁷ and Claudia Bracaglia², ¹Pediatric Area, University of Milan, Milan, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ³Division of Pediatric Rheumatology, Department of pediatrics, King Faisal specialist hospital and research center, Riyadh, Saudi Arabia, RiYADH, Saudi Arabia, ⁴Rheumatology Unit, Department of Paediatrics and Adolescent Medicine, Charles University in Prague, Dlouha 534, Kladno, 27201, Czech Republic, ⁵University of Health Sciences, Umraniye Tranining and Research Hospital Division of Pediatric Rheumatology, Istanbul, Turkey, Istanbul, Turkey, ⁶University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia, Zagreb, Grad Zagreb, Croatia, ⁷Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, Sto arrivando!, Russia, ⁸Department of Pediatric Rheumatology and Immunology, University Hospital Muenster, Muenster, Germany, Muenster, Germany, ⁹Second Department of Paediatrics, P. & A. Kyriakou Children’s Hospital, Athens, Greece, Athens, Greece, ¹⁰Children's Hospital Zagreb, Josip Juraj Strossmayer University of Osijek, Medical Faculty Osijek, Zagreb, Croatia, Zagreb, Grad Zagreb, Croatia, ¹¹UOC Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini, Genoa, Italy, Genoa, Italy, ¹²Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, Milan, Italy, ¹³Paediatric Rheumatology Unit, Anna Meyer Children's University Hospital, School of Human Health Science, Florence, Italy, Florence, Italy, ¹⁴University of Toronto, The Hospital for Sick Children, Toronto, Canada, ¹⁵Paediatric Rheumatology and Autoinflammatory Diseases Unit, General University Hospital, Prague, Czech Republic, Prague, Czech Republic, ¹⁶University of Health Sciences, Umraniye Tranining and Research Hospital Division of Pediatric Rheumatology Istanbul, Turkey, Istanbul, Turkey, ¹⁷Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy

Meeting: ACR Convergence 2020

Keywords: Juvenile idiopathic arthritis, macrophage activation syndrome, risk assessment

Session Information

Date: Sunday, November 8, 2020

Title: Pediatric Rheumatology – Clinical Poster II: Systemic JIA, Autoinflammatory, & Scleroderma

Session Type: Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:

Macrophage Activation Syndrome (MAS) is a severe, life-threatening, complication of Systemic Juvenile Idiopathic Arthritis (sJIA) with a significant mortality. A score that identify sJIA patients with high risk to develop MAS would be useful in clinical practice.

Methods:

We evaluated whether routine laboratory parameters at disease onset may predict the development of MAS in patients with sJIA and we defined a risk score of MAS using these parameters. Then, we validated the score in a second population. Laboratory parameters of disease activity and severity were retrospectively evaluated in 99 sJIA patients referred to Bambino Gesù Hospital in the last 10 years with at least 2 years of follow-up. Laboratory parameters were evaluated during active sJIA, without MAS, at disease onset or disease flare, immediately before treatment for sJIA was started or modified. Patients were divided in two groups: sJIA patients without MAS in the 2 years of follow-up and sJIA patients with at least one MAS episode. To create the MAS risk score, laboratory parameters with a statistically significant difference between the 2 groups were selected.

Results:

Thirty patients, that fulfilled the 2016 classification criteria for MAS [1] at time of sampling, were excluded from the analysis. Therefore, we analysed laboratory parameters of 69 sJIA patients, 41 without MAS in the follow-up and 28 with at least one episode of MAS. Levels of ferritin, AST, LDH and triglycerides were significantly higher in patients with MAS during follow-up compared to those without. Their respective cut-off were computed by means of ROC curve analysis. A regression coefficient-based scoring system was used to assign weights to the risk index and the optimal score cut-off was defined by ROC curve analysis (Table 1). A MAS risk score >5 identified 27 out of 28 sJIA patients with MAS during the follow-up and 8 out of 41 sJIA patients without MAS. Sensitivity (Se), specificity (Sp), positive predictive value (PPV) and negative predictive value (NPV) of the score are detailed in table 2. In order to validate the MAS risk score on a different population, we applied the score on 132 sJIA patients from other pediatric rheumatology centers, 100 without history of MAS and 32 with at least one episode of MAS. Se, Sp, PPV and NPV of the score are reported in table 2.

Conclusion:

In conclusion we developed a MAS risk score based on routine laboratory parameters, available worldwide, that can help clinicians to identify early in the disease course sJIA patients with high risk to develop MAS.

Reference:
1. Ravelli A et al. Ann Rheum Dis. 2016 Mar;75(3):481-9.

Disclosure: S. Carbogno, None; D. Pires Marafon, None; G. Marucci, None; E. Sacco, None; M. Pardeo, None; A. Alsaleem, None; S. Fingerhutova, None; F. Demir, None; N. Cekada, None; M. Kostik, None; C. Kessel, None; O. Vougiouka, None; A. Gagro, None; J. Tibaldi, None; F. Minoia, None; I. Maccora, None; R. Schneider, Novartis, 5, Sobi, 5, Novimmune, 5, Roche, 5; P. Dolezalova, None; B. Sozeri, None; M. Jelusic, None; A. Insalaco, None; F. De Benedetti, Novartis, 2, 8, Novimmune, 2, 9, Sobi, 2, 8, 9, Roche, 2, 8, Pfizer, 2, Sanofi, 2, AbbVie, 8; C. Bracaglia, SOBI, 8.

To cite this abstract in AMA style:

Carbogno S, Pires Marafon D, Marucci G, Sacco E, Pardeo M, Alsaleem A, Fingerhutova S, Demir F, Cekada N, Kostik M, Kessel C, Vougiouka O, Gagro A, Tibaldi J, Minoia F, Maccora I, Schneider R, Dolezalova P, Sozeri B, Jelusic M, Insalaco A, De Benedetti F, Bracaglia C. Risk Score of Macrophage Activation Syndrome (MAS) in Patients with Systemic Juvenile Idiopathic Arthritis (sJIA) [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/risk-score-of-macrophage-activation-syndrome-mas-in-patients-with-systemic-juvenile-idiopathic-arthritis-sjia/. Accessed .

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