Abstract:

Objective · To investigate the clinical features of macrophage activation syndrome (MAS) associated with adult-onset Still's disease (AOSD), and provide the basis for clinical diagnosis and treatment of the disease.  Methods · The clinical data of 42 patients with AOSD, including 14 patients with AOSD-induced MAS (the MAS group) and 28 AOSD patients paired by age and sex (the non-MAS group), diagnosed in Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine from October 2013 to June 2016 were collected and then retrospectively analyzed.  Results · There was no significant difference in age, sex and duration of AOSD between two groups. The mortality rate of patients in MAS group was significantly higher than that of patients in non-MAS group, as well as the rates of rash, splenomegaly and hemophagocytosis. The levels of ALT and serum ferritin in MAS group were higher than those in non-MAS group, while the level of FDP is lower. Glucocorticoids were used in all 42 patients, and the dosage of glucocorticoids was significantly higher in MAS group than non-MAS group. Only 1 patient with AOSD-induced MAS received MTX, the percentage of patients receiving MTX was significantly lower in MAS group than non-MAS group. Five patients with AOSD-induced MAS received IVIG, the percentage of patients receiving IVIG was significantly higher in MAS group than non-MAS group. Two patients with AOSD-induced MAS received VP-16.  Conclusion · The mortality rate of patients in MAS group was significantly higher than that of patients in non-MAS group, as well as the rates of rash, splenomegaly and hemophagocytosis. The levels of ALT and serum ferritin in patients with AOSD-induced MAS were higher than patients without MAS, while the level of FDP was lower. Early diagnosis and active treatment is the key point to improve clinical outcome.

Key words: adult-onset Still's disease, macrophage activation syndrome, ferritin