Macrophage activation syndrome in adults: Characteristics, outcomes, and therapeutic effectiveness of etoposide-based regimen

ObjectivesTo describe the clinical characteristics and outcomes of adult macrophage activation syndrome (MAS) patients and to provide experience for the treatment. MethodsAdult patients with MAS admitted to Beijing Friendship Hospital from December 2014 to September 2021 were enrolled in this study. Clinical data of patients were collected and analyzed. ResultsA total of 118 adult MAS patients entered this study. MAS was the first manifestation in 43 (36.4%) patients, while 75 (63.6%) developed MAS after the diagnosis of autoimmune disease (AID) with a median diagnostic interval of 2 (0.5-359) months. Eighty-two patients were initially treated with glucocorticoid-based regimen; the overall response (OR) rate at the 2-week posttreatment was 37.8%. Forty-five patients switched to etoposide-based regimen, and the OR rate was 84.4%. Thirty-six patients were initially treated with etoposide-based regimen, and the OR rate at the 2-week posttreatment was 80.6%. Serum IL-18 (P = 0.021), IFN-gamma (P = 0.013), IP-10 (P = 0.001), IL-10 (P = 0.041), IL-1RA (P < 0.001), and TNF-alpha (P = 0.020) levels of patients were significantly decreased in the remission phase than in the active phase. Levels of SDF-1 alpha (P = 0.018) and IL-7 (P = 0.022) were higher in refractory patients, while the GRO-alpha level had a strong tendency toward statistical significance (P = 0.050). The probability of overall survival (OS) at 3, 6, and 36 months after HLH diagnosis were 89.8%, 89.0%, and 87.9%, retrospectively. The active MAS status at the 2-week post initial treatment [P = 0.009, HR = 15.281, 95% CI, (0.1.972, 118.430)] and baseline neutrophil count (Neu) <1.5 x 10(9)/l [P = 0.017, HR = 3.678, 95% CI, (1.267, 10.672)] were negative prognostic factors. ConclusionMAS typically occurs within 2 months after the onset of autoimmune disease in adults. SDF-1 alpha, IL-7, and GRO-alpha could be used to predict refractory MAS. The etoposide-based regimen is effective and tolerable for adult MAS.