Managing MAS
Three main management goals for patients with MAS
Macrophage activation syndrome (MAS) requires appropriate recognition and timely treatment to prevent irreversible organ damage.1
Stabilize the patient
Controlling hyperinflammation.
Identify potential triggers
Work to identify any conditions that may have triggered the MAS episode.
Minimize toxicities
Minimize steroid exposure from broadly acting agents.
Initial treatment for MAS typically involves steroid therapy
Treatment approaches are generally based on empirical use of immunosuppressants which do not specifically target key drivers of hyperinflammation and are not always effective.1 Broad immunosuppressive therapies such as high-dose glucocorticoids do not specifically target the key drivers of hyperinflammation in MAS.2 Glucocorticoid pulse therapy has an unsatisfactory response up to 33% of pediatric patients, and data from individual centers suggest that up to 80% of adult patients may also be unresponsive to steroids.4-7
Dose-dependent side effects of glucocorticoid treatment such as hyperglycemia, hypertension, myopathy, and psychosis may occur. It is important to monitor for these side effects in patients receiving treatment.1
References: 1. Shakoory B, Geerlinks A, Wilejto M, et al. The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS). Arthritis Rheumatol. 2023;75(10):1714-1732. doi:10.1002/art.42636 2. Carter SJ, Tattersall RS, Ramanan AV. Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment. Rheumatology (Oxford). 2019;58(1):5-17. doi:10.1093/rheumatology/key006 3. Lerkvaleekul B, Vilaiyuk S. Macrophage activation syndrome: early diagnosis is key. Open Access Rheumatol. 2018;10:117-128. doi:10.2147/OARRR.S151013 4. De Benedetti F, Grom AA, Brogan PA, et al. Efficacy and safety of emapalumab in macrophage activation syndrome. Ann Rheum Dis. 2023;82(6):857-865. doi:10.1136/ard-2022-223739 5. Gavand PE, Serio I, Arnaud L, et al. Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: a study of 103 episodes in 89 adult patients. Autoimmun Rev. 2017;16(7):743-749. doi:10.1016/j.autrev.2017.05.010 6. He L, Yao S, Zhang R, et al. Macrophage activation syndrome in adults: characteristics, outcomes, and therapeutic effectiveness of etoposide-based regimen. Front Immunol. 2022;13:955523. doi:10.3389/fimmu.2022.955523 7. Nam SH, Ahn SM, Oh JS, et al. Macrophage activation syndrome in rheumatic disease: clinical characteristics and prognosis of 20 adult patients. PLoS One. 2022;17(5):e0267715. doi:10.1371/journal.pone.0267715
