Clinical study of DEP regimen as a salvage therapy for refractory macrophage activation syndrome / 中华风湿病学杂志

ABSTRACT

Objective:To investigate the efficacy of liposomal doxombicin combined with etoposide and high dose methylprednisolone (DEP) as a salvage therapy for refractory macrophage activation syndrome (MAS).Methods:Totally 38 patients with refractory MAS were enrolled in this study from January 2016 to January 2022 in Beijing Friendship Hospital, including clinical characteristics and laboratory test results before and after DEP treatment, were retrospectively collected. The efficacy was evaluated every 2 weeks according to the United States Midwest Cooperative HLH Group. Relevant samples were statistically analyzed using non-parametric tests.Results:Of 38 refractory MAS patients, 8 males and 30 females were included into this study.The median age was 30(15-69) years old. The underlying disease were adult onset Still's disease in 29 cases, Systemic lupus erythematosus in 6 cases, Rheumatoid arthritis in 1 case and Undifferentiated Connective-Tissue disease in 2. The overall response rate was 95% (36/38), including 9 patients (24%) achieved complete remission and 27 patients (71%) achieved partial remission after 2 weeks of treatment. The overall response rate was 97% (34/35), including 16 (46%) complete remission and 18 (51%) partial remission after 4 weeks of treatment(due to lack of data in some patients). The overall response rate was 97% (34/35), including 17 (49%) complete remission and 17 (49%) partial remission after 6 weeks of treatment. Patients who achieved partial remission or complete remission were actively treated for the underlying diseases after induction, and their conditions were in persistent remission.Conclusion:The DEP regimen may be an effective salvage therapy for the treatment of refractory MAS.