Clinical Analysis Of Systemic Juvenile Idiopathic Arthritis-associated Macrophage Activation Syndrome

Objective:To probe into the clinical characteristics,laboratory data,treatment,and relative factors of prognosis of systemic juvenile idiopathic arthritis?sJIA?and its associated macrophage activation syndrome?sJIA-MAS?,so as to provide a clinical basis for clinicians to make early diagnosis,treatment and prognosis evaluation.Methods:To admitted to the Children's Hospital of Chongqing Medical University and Chengdu Women&Children's Centeral Hospital from October,2006 to October,2018,the clinical data,laboratory data,treatment methods and prognosis of all children patients diagnosed with sJIA,as well as the children with sJIA-MAS in active phase were retrospectively analyzed.Results:A total of 72 children with active sJIA and 36 child patients with sJIA-MAS were included.Of 36 children with sJIA-MAS,26 children?72.2%?had no significant cause of MAS,and 10 children?27.8%?had infectious inducement.The manifestations of sJIA in active stage included fever?100%?,rash?75%?and joint pain?79.2%?.The manifestations of sJIA-MAS included fever?100%?,hepatomegaly?66.7%?and neurological involvement?44.4%?.Hemophagocytosis was more common in bone marrow examination of sJIA-MAS group?41.2%?.In terms of laboratory indexes,the leukocyte and erythrocyte sedimentation were significantly increased in the children patients with sJIA in the active stage;while,in the children patients with sJIA–MAS,the white cells,platelet,hemoglobin,fibrinogen and NK cells were mostly decreased,but the alanine transaminase,glutamic oxalacetic transaminase,triglyceride,ferritin and lactate dehydrogenase were increased.In this group,12 children with sJIA-MAS received high-dose corticosteroids therapy,and 17 children received the low-dose corticosteroids therapy.In the high dose group,10patients?83.33%?had stable temperature within 1 day,while 7 patients?41.17%?in the low-dose group had stable temperature within 1 day.And that difference between the two groups was not statistically significant.There was no statistically significant difference between the two groups in the remission degree of laboratory indexes at the 3^rd day,5^th day and 7^th day after treatment.1 children patients with sJIA–MAS was treated with intravenous immunoglobulin and high-dose corticosteroids therapy,and the condition was repeated.But its condition relieved after adding cyclosporine.2 children with sJIA-MAS relieved and 1 patient was myelosuppression after treating with HLH-2004 protocol.In this group including 36 children with sJIA-MAS,3 children abandoned treatment,and 5 in the remaining 33cases were dead,with the mortality rate of about 15.15%.There were statistical differences between the death group and the non-death group in the manifestations of hemorrhage,convulsion and coma,but no statistical differences in the early laboratory indexes.Conclusions:The results show that sJIA-MAS may have no apparent inducement.The children with sJIA appear to that clinical manifestations include high fever,hemorrhage,polyserositis and neurological involvement,and laboratory indexes find pancytopenia,liver dysfunction,that should be considered to MAS.Because of minor specimen,there was no statistically significant difference between the different does corticosteroids therapy groups in the remission degree of clinical and laboratory indexes.Large sample and prospective studies are still required for sJIA-MAS corticosteroids therapy.For sJIA-MAS,cyclosporine should be added as early as possible or the HLH-2004 regimen should be used when steroid alone is ineffective,but it is necessary to pay attention to the side effects of drugs.Hemorrhage,convulsions,and coma may be the unfavorable prognostic factors of sJIA-MAS.