Comparative Analysis Of Clinical Features Of Systematic-onset Juvenile Idiopathic Arthritis And Kawasaki Disease With Macrophage Activation Syndrome

Objective: The clinical manifestations and laboratory characteristics of Systemic juvenile idiopathic arthritis(SJIA)were compared with that of Macrophage activation syndrome(MAS)combined with Kawasaki disease(KD).Methods: The clinical data of 16 cases of SJIA-MAS and 10 cases of KD-MAS from Jan,2012 to Dec,2020 were collected,and their cli nical manifestations,laboratory data,treatment methods and outcomes w ere retrospectively analyzed.Results: 1.There were 26 children included in this study,including 9 males and 7 females in the 16 cases of SJIA-MAS,and the median time of MAS onset was 85 days.There were 8 males among the 10 children with KD-MAS,and the median time was 15 days,and KDMAS occurred fastest.All the children showed symptoms and signs of persistent high fever,enlarged liver and spleen lymph nodes,increased liver enzymes,decreased blood three lines,and increased lactate dehydrogenase and ferritin,among which,The median of SJIA-MAS ferritin was 1160.0 ng/m L,and the median of LDH was 1344.19 U/L.The median ferritin in KD-MAS was 839.0 ng/m L and the median of LDH was 665.60 U/L.The increase of lactate dehydrogenase and ferritin in SJIA-MAS group was significantly higher than that in KD-MAS group,and the difference was statistically significant.All children with SJIAMAS were treated with methylprednisolone shock therapy,of which 9had poor effect,5 had improved symptoms after oral administration of cyclosporine A(Cs A),3 patients had clinical relief after regular infusion of tocilizumab injection,and one children died of respiratory and circu- latory failure.All children with KD-MAS were treated with high-dose intravenous gamma globulin impulse therapy,with methylprednisolone in7 cases and two or more immunosuppressant(dexamethasone,cyclosporine,or etoposide)in the remaining 3 cases under suboptimal treatment.All children were able to control the disease effectively and recovered well in the later period.Conclusions:1.Compared with SJIA-MAS,the clinical presentation of KD-MAS are milder and the prognosis will be better,which may be related to the early application of IVIG;2.Serum ferritin and LDH levels of SJIA-MAS were higher than those of KD-MAS;3.LDH may be used as another sensitive index for MAS besides serum ferritin.