| ObjectiveTo analyze the clinical manifestations and laboratory characteristics of systemic juvenile idiopathic arthritis(s JIA)and its complicated with macrophage activation syndrome(s JIA-MAS),and then to explore the influencing factors of the occurrence and poor prognosis in patients complicated with MAS so as to provide clinical reference for the early identification and prognosis evaluation of s JIA-MAS.Methods27 s JIA patients complicated with MAS admitted to the department of rheumatology and immunology,Xi ’an Children’s Hospital from January 2012 to December 2021 were selected as the MAS group,and 60 patients with s JIA at the same time were selected as the s JIA group.The influencing factors for the occurrence of s JIA-MAS were explored by retrospectively analysis of the general data,clinical characteristics and laboratory results in two groups.At the same time,according to prognosis of patients with MAS group,they were divided into dead group and non-dead group.The influencing factors for the poor prognosis of s JIA-MAS were explored by retrospectively analysis of the general data,clinical characteristics and laboratory results in two groups.Results(1)A total of 60 children were included in the s JIA group in this study,including 43 males and 17 females,with the median age was 5.6 years.A total of 27 children were included in MAS group,including 18 males and 9 females,with the median age was 6.6years.There were no gender and age difference between the two groups(P>0.05).The two groups differ significantly in hepatomegaly,splenomegaly,hemorrhage manifestations,central nervous system involvement,cardiovascular system involvement,pleural effusion,abdominal effusion and bone marrow macrophage(P<0.05).In contrast to the children with s JIA,leukocyte count,platelet count,hemoglobin,albumin,fibrinogen,erythrocyte sedimentation rate,and NK cell ratio were significantly reduced in children with MAS group,prothrombin time was prolonged,alalanine aminotransferase,aspartine aminotransferase,lactate dehydrogenase,triglycerides,urea,creatinine,serum ferritin,serum ferriin/erythrocyte sedimentation ratio was significantly higher,and serum ferritin/erythrocyte sedimentation ratio>21.5 composition ratio was significantly higher than the s JIA group,the difference was statistical significant(P<0.05).(2)Multivariate regression analysis showed that serum ferritin/erythrocyte sedimentation rate ratio>21.5(OR=4.995,P=0.004),central nervous system involvement(OR =4.873,P =0.006)and hemorrhage manifestations(OR=3.632,P =0.015)were risk factors for the occurrence of s JIA-MAS.(3)In this study,there were four deaths in the MAS group with the mortality rate of14.8%,the male to female ratio of 3∶1,and the median age was 3.9 years.Compared with the non-death group,there were no statistical significance in gender and age between the two groups(P>0.05).There were statistically significant differences in central nervous system involvement and hemorrhage manifestations(P<0.05).The level of serum ferritin in death group was significantly increased and the proportion of NK cells was significantly decreased,with statistical significance between the two groups(P<0.05).(4)Multivariate regression analysis showed that the level of serum ferritin significantly increased(OR =3.652,P=0.018)and central nervous system involvement(OR =3.344,P =0.021)were risk factors for the poor prognosis of s JIA-MAS.Conclusions(1)Serum ferritin/erythrocyte sedimentation rate ratio>21.5,central nervous system involvement and hemorrhage manifestations(OR =3.632,P=0.015)were risk factors for the occurrence with s JIA-MAS.So,it should be vigilant for s JIA patients who could be complicated with MAS to strict observation of above clinical manifestations and dynamic monitoring of those laboratory index in the early onset stage of s JIA.(2)There were two risk factors for poor prognosis of s JIA-MAS,which included the level of significantly increasing serum ferritin and involvement of central nervous system. |
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