A Preliminary Study On The Predictive Value Of 2016classification Criteria For MAS Complicating SoJIA As A Reference To Scan For Adult MAS Tendency

? Background and Objectives ? Macrophage activation syndrome(MAS)is a potentially life-threatening complication of rheumatic disease,it is often considered a type of secondary hemophagocytic lymphohistiocytosis(HLH)and results from over-activation of T lymphocytesand macrophages leading to a “cytokine storm”.Characteristic features are persistent fever,lymphadenopathy,hepatosplenomegaly,cytopenias(anemia,leucopenia,thrombocytopenia),hypofibrinogenemia,elevated transaminases,hypertriglyceridemia and extreme hyperferritinemia often associated with multi-organ impairment.Early identification is the key to successful treatment.The diagnosis of MAS is mainly refer to 2004-histiocyte society hemophagocytic syndrome criteria and 2005-classification criteria for MAS complicating So JIA.At the international MAS classification standards consensus conference,EULAR / ACR/ PRITO jointly developed a new classification standard for MAS complicating So JIA in 2014,and published on the Ann Rheum Dis in 2016.At present,there is no diagnostic criteria for adult MAS.Therefore,this study retrospective analysis the cases were diagnosed as adult Still 's disease(AOSD)and HLH from our hospital in recent years,preliminary study on screening indexes of adult MAS tendency and to assess its predictive value,in order to intervene early and prevent it from progressing to typical MAS or HLH.? Methods ? Cases were selected from January 2009 to July 2016 in Second Affiliated Hospital of Fujian Medical University,discharged from hospital diagnosed with AOSD,HLH patients.AOSD used Yamaguchi criteria.Histiocyte society hemophagocytic syndrome criteria was applied on HLH.There is no generally accepted clssification criertia for adult MAS,we reference the 2016 classification criteria for MAS complicating So JIA.According to the diagnostic criteria for primary screening cases,the following data were collected for each patients:age,gender,diagnosis,main symptoms and signs,main laboratory examination,histological finding and prognosis.A total of 39 cases of AOSD were collected,HLH was performed in 33 cases.In 16 patients with adult MAS tendency diagnosed by AOSD,8 patients performed bone marrow biopsy,only 1 patient showed hemophagocytosis,bone marrow biopsy was performed in all the 33 patients with HLH,of which,24 patients showed hemophagocytosis.Combined with the literature,analysis the predictive value of the new classification criteria for adult MAS tendency.? Results ? In 39 cases of AOSD patients,there were 16 cases conform to the 2016 classification criteria for MAS complicating So JIA.In 33 cases of HLH patients,there were 26 cases meet the new classification criteria.Adult MAS and rheumatism can be manifested asfever,hepatosplenomegaly,high serum ferritin levels and hypertriglyceridemia,but elevated transaminases(AST)and lactate dehydrogenase(LDH),and thrombocytopenia was significantly different between the two.The hypofibrinogenemia was significantly different between the adult MAS tendency patients complicating hemophagocytosis and the adult MAS tendency patients without hemophagocytosis.The adult MAS tendency patients with good prognosis compare with the poor prognosis patients,the leucopenia and splenomegaly were significantly different.In 33 cases of HLH,26 patients could diagnosed with adult MAS tendency before the diagnosis of HLH,of which,14 patients received early diagnosis and treatment,and 9 cases were improved.However,12 patients did not get early diagnosis and treatment,only 1 patient were improved.Unite the hyperferritinemia,elevated transaminases,hypofibrinogenemia,as a condition to screening the 33 cases of HLH patients with adult MAS tendency,the AUC is0.968,the P-value is <0.001,the cutoff value is 0.776,the sensitivity is 0.900,the specificity is0.889.And the cutoff value of prediction indexes for adult MAS tendency is ferritin(SF 936.47ng/ml),Aspartate transaminase(AST 106.5 IU/ml),Fibrinogen(Fib 1.94 g/l),Platelet(PLT124×10^9/L),WBC 3.2×10^9/L,LDH 625.5 U/L.? Conclusions ? AOSD patients and other rheumatisant with hyperferritinemia,elevated transaminases andor LDH,contemporaneous with thrombocytopenia,hypofibrinogenemia andor leucopenia is an important warning signal of adult MAS tendency.This study has significant clinical value to improve the prognosis as it could assist us to early identification and timely treatment for adult rheumatic patients with MAS tendency.