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THU0576 PROGNOSTIC FACTORS PREDICTING THE SURVIVAL OF PATIENTS WITH MACROPHAGE ACTIVATION SYNDROME

Background: Macrophage activation syndrome (MAS) is a hyperinflammatory condition, which can lead to death in patients with rheumatologic disease. However, the prognosis of adult patients with MAS is largely unknown. Objectives: We aimed to investigate the mortality in patients with MAS accompanied Adult-onset Still’s disease (AOSD) or systemic lupus erythematosus (SLE) and to evaluate the prognostic factors predicting the mortality in patients with MAS. Methods: We retrospectively reviewed febrile patients with AOSD or SLE admitted to Severance Hospital between 2005 and 2018. Patients who satisfied the classification criteria of MAS was included in the analysis. Cox-regression analysis was performed to evaluate the clinical factors associated with the overall mortality in patients with MAS. Results: Of the total 123 patients, 48 (39%) patients and 75 (61%) patients were diagnosed with AOSD and SLE, respectively. Forty-three patients (35%) were died from MAS during hospitalization. There was no significant difference in mortality between AOSD and SLE (P = 0.675). In multivariate analysis, cytopenia, and insufficient reduction of ferritin and rebound of inflammation after initial treatment with glucocorticoid were independently associated with death in patients with MAS (P = 0.019, < 0.001, and 0.011, respectively). Conclusion: The presence of cytopenia, and treatment response after glucocorticoid treatment was closely related with death in patients with MAS. More intensive treatment should be considered in high-risk patients with poor prognostic factors. References: [1] Carter, S.J., R.S. Tattersall, and A.V. Ramanan, Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment. Rheumatology (Oxford), 2018. [2] Grom, A.A., A. Horne, and F. De Benedetti, Macrophage activation syndrome in the era of biologic therapy. Nat Rev Rheumatol, 2016. 12(5): p.259-68. [3] Ahn, S.S., et al., Application of the 2016 EULAR/ACR/PRINTO Classification Criteria for Macrophage Activation Syndrome in Patients with Adult-onset Still Disease. J Rheumatol, 2017. 44(7): p.996-1003. [4] Ahn, S.S., et al., In-hospital mortality in febrile lupus patients based on 2016 EULAR/ACR/PRINTO classification criteria for macrophage activation syndrome. Semin Arthritis Rheum, 2017. 47(2): p.216-221. Disclosure of Interests: None declared