Sat0230 macrophage activation syndrome in sle and systemic onset jia: similar or dissimilar

Background:Macrophage activation syndrome (MAS) is a serious complication in rheumatic disease. Fever and hyperferritinemia are common in systemic onset JIA and cytopenias are common in SLE thus recognising MAS in them is a challenge.Objectives:We compared clinical, laboratory parameters, various classification criteria for MAS, and its outcome in SLE and sJIA.Methods:Clinical and laboratory data were extracted from clinician diagnosed cases of MAS with SLE/sJIA who were admitted between 2004-2018 at a tertiary care hospital. Percentage of patients satisfying Ravelli, International consensus, HLH 2004 and criteria proposed by Parodi et al1were calculated.Results:Among 33 patients (18 females) with MAS 19 had SLE and 14 had sJIA. MAS was more likely to be the presenting manifestation of disease in SLE as compared to sJIA (p<0.05). There were no differences in the clinical features among these two diseases. EBV and CMV were identified in 2 patients each as the trigger for MAS.Patients with SLE had lower baseline TLC and platelet whereas patients with sJIA-MAS had significantly higher median CRP (p = 0.002), fall in TLC (p=0.012) and delta ESR/CRP ratio (p=0.02) and lower fibrinogen level (p=0.006). Neutrophil to lymphocyte ratio, Ferritin/CRP ratio and number of patients with Ferritin/ESR >80 were similar. Bone Marrow hemophagocytosis was seen in only in 21% of patients.Only 6/33 fulfilled HLH criteria but criteria meant for sJIA or SLE performed well for both diseases and majority of patients could be diagnosed using them. Treatment included steroids(100%), cyclosporine(30%), Tacrolimus(21%), cyclophosphamide(21%), etoposide(3%) and thalidomide(12%). Mortality was similar in both groups.Table 1.Agreement amongst MAS/HLH criteria in SLE and sJIA MASSLE-MASHLHRavelli et alConsensusParodi et alHLH4444Ravelli et al4191918Consensus4191918Parodi et al4181818sJIA-MASHLHRavelli et alConsensusParodi et alHLH2222Ravelli et al2121112Consensus2111212Parodi et al2121214Table 2.Comparison with various other cohortsMinoia et alsJIAn (%)Our studysJIA, n (%)Our Study SLE, n (%)SLE, n (%) Ai-Chun Liu et alJuvenile SLE n (%)Parodi et alNumber36214193238MAS as presenting feature (%)80 (22)4 (28)12 (63)NA24 (63)Most common manifestation (%)Fever (96)Fever (100)Fever (89)Fever (96)Fever (89)Most common triggerDisease activityDisease activityDisease activityDisease activityDisease activityBM done251 (72.3)8 (57)12 (63)32 (96)38 (100)BM hemophagocytosis150 (60.7)2 (25)5 (41)32 (100)20 (52)Mortality28 (8)2 (14)2 (10.5)4 (12.5)4 (11.4)Patients meeting JIA criteria %NA-100NA100Patients meeting HLH criteria %NA1419NA66Conclusion:MAS is more likely to be presenting manifestation in SLE compared to sJIA. Though lab parameters are significantly different in MAS associated with SLE & sJIA, criteria meant for MAS in sJIA or SLE MAS performed equally well in both diseases.References:[1]Parodi A et al, Macrophage activation syndrome in juvenile systemic lupus erythematosus: a multinational multicenter study of thirty-eight patients. Arthritis Rheum. 2009 Nov;60(11):3388-99.Disclosure of Interests:None declared