FRI0509 COMPARISON OF MS SCORE AND HSCORE FOR THE DIAGNOSIS OF ADULT-ONSET STILL’S DISEASE ASSOCIATED MACROPHAGE ACTIVATION SYNDROME

Abstract

Background:Adult-onset Still’s disease (AOSD) is a rare multisystemic auto-inflammatory disease.Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic disorders, including sJIA, adult-onset Still’s disease (AOSD) and lupus. Many tools have been developed for the diagnosis of MAS, including HScore and MS score. Although some authors have tried to apply these tools for the diagnosis of ASOD-associated MAS, there are no studies comparing the diagnostic ability of HScore and MS score.Objectives:We aimed to compare the capacity of HS score and MS score for the diagnosis of AOSD-associated MAS.Methods:Patients diagnosed as AOSD during January,2012 and October,2019 in our hospital were retrospectively analyzed. Clinical and laboratory data were compared between patients with pure AOSD and those with AOSD associated MAS. HScore and MS score were calculated respectively and the diagnostic capacity of MAS was compared by means of receiver operating characteristic (ROC) curve analysis.Results:We included 174 patients with pure AOSD and 35 patients with AOSD-associated MAS. Patients with AOSD-associated MAS were younger than those with pure AOSD (32+/-11.4 yrs vs 36.9+/-13.5 yrs, P=0.028). More death were observed among patients with AOSD-associated MAS (17.1% vs 3.4%, P=0.001). Patients with AOSD-associated MAS had higher HScore (median[range] 196[98-333] vs 68[33-156], P<0.001) and higher MS score (median[range] 1.05[-1.26-26.55] vs -1.17[-1.26-2.52],P<0.001) than those with pure AOSD.The difference of different parameters of these two groups of patients were detailed in Table 1 and Table 2. ROC curve analysis (Figure 1) revealed that HScore has stronger ability to diagnose AOSD-associated MAS compared with MScore (AUC=0.973 and 0.865 for HScore and MS score repectively, P<0.001). HScore≥120 perform best (sensitivity 90.6% and specificity 89.6%). MS score≥-0.25 yielded a sensitivity of 75% and a specificity of 73%.Figure 1.ROC curve of HScore and MS score. HScore=120, sensitivity=90.6%, specificity= 89.6%. MS score=-0.45,sensitivity=75%, specificity=73%. AUC-HScore=0.973, AUC-MS score=0.865, P<0.001Conclusion:HScore seems to perform much better than MS score for the diagnosis of AOSD-associated MAS in our cohort.References:[1] Siddiqui M, Putman MS, Dua AB. Adult-Onset still’s disease: current challenges and future prospects. Open Access Rheumatol 2016;8:17–22.[2] Bracaglia C, Prencipe G, De Benedetti F. Macrophage activation syndrome: different mechanisms leading to a one clinical syndrome. Pediatr Rheumatol Online J 2017;15.[3] Guilpain P, Le Quellec A. About the complexity of adult onset still’s disease... and advances still required for its management. BMC Med 2017;15:5.[4] Minoia F, Bovis F, Davì s, et al. Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis.Ann Rheum Dis 2019;78:1357–1362.[5] Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocyticsyndrome. Arthritis Rheumatol. 2014 Sep;66(9):2613-20.[6] ED Batu, A Erden, E Seyhoğlu, etal. Assessment of the HScore for reactive haemophagocytic syndrome in patients with rheumatic diseases.Scand J Rheumatol. 2017 Jan;46(1):44-48.[7] Wang R,Li T, Ye S, et al. Application of MS score in macrophage activation syndrome patients associated with adult onset Still’s disease. Ann Rheum Dis. 2019 Oct 14. pii: annrheumdis-2019-216286.Disclosure of Interests:None declared