Clinical And Laboratory Characteristics Of Connective Tissue Disease With Macrophage Activation Syndrome

Objective: To summarize the clinical and laboratory characteristics of connective tissue disease with macrophage activation syndrome(MAS),and to improve the understanding of this diseaseMethods: A retrospective analysis was performed for clinical laboratory characteristics,treatment and outcomes of 19 patients with connective tissue disease complicated with macrophage activation syndrome/ hemophagocytic lymphohistiocytosis(HLH)admitted between May 2014 and December 2018.Results: The primary diseases of the 19 patients included systemic lupus erythematosus(SLE)in 9 cases(47.4%),connective tissue disease(CTD)in 3 cases(15.8%),rheumatoid arthritis(RA)in 2 cases(10.5%),adult onset still disease(AOSD)in 2 cases(10.5%),amyopathic dermatomyositis in 1 case(5.3%),and overlap syndrome in 2 cases(10.5%)(including 1 case of SLE,Sjogren syndrome and 1 case of SLE,RA).Clinical symptoms mainly include fever(100%),serous cavity effusion(68.4%),lymph node enlargement(63.2%),lung disease(63.2%),central nervous system damage(63.2%),bleeding performance(31.6%).Two or three series of peripheral blood cells decreased.While the serum ferritin,lactate dehydrogenase(LDH),alanine aminotransferase(ALT),and triglyceride(TG)increased.And coagulation dysfunction was included.There were 12 patients were improved and discharged.Conclusions: If patients with connective tissue disease have high fever,cytopenia,abnormal liver enzymes,coagulation dysfunction,and a increase in serum ferritin,lactate dehydrogenase and triglyceride,the development of MAS should be considered.The absence of hemophagocytosis in bone marrow examination cannot exclude the diagnosis of MAS.