Challenges
in MAS
Timely identification and treatment
Prompt and aggressive treatment is recommended for patients who have confirmed or suspected MAS because suboptimal control of MAS-driven hyperinflammation can lead to irreversible organ damage.1,2
Diagnosis and treatment often occurs in a hospital setting due to the acute onset of hyperinflammatory signs and symptoms, but
efficient identification and management may help shift care to an outpatient setting.1,3
Retrospective studies have found that approximately
18% to 35% of patients
with MAS may require ICU admission.4,5
Treatment options
Initial treatment for MAS typically involves steroid therapy.1
Glucocorticoid pulse therapy has an unsatisfactory response in 33% of pediatric patients, and data from individual centers suggest that up to 80% of adults may also be unresponsive to steroids.6-9
Broad immunosuppressant therapies such as high-dose glucocorticoids
do not specifically target key drivers of hyperinflammation in MAS.2
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
Lack of FDA-approved treatments for MAS
With no current FDA-approved treatment for MAS, there is a need for a targeted option that can calm hyperinflammation and stabilize patients for continued care, as well as minimize steroid exposure.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. Pai TS, Stancampiano FF, Rivera C. Hemophagocytic lymphohistiocytosis for the internist and other primary care providers. J Prim Care Community Health. 2021;12:21501327211053756. doi:10.1177/21501327211053756 4. Minoia F, Davì S, Horne A, et al. Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol. 2014;66(11):3160-3169. doi:10.1002/art.38802 5. Yao H, Wang Y, Wang Z, et al. The performance of the diagnostic scoring system or criteria for macrophage activation syndrome in systemic juvenile idiopathic arthritis for adult-onset Still’s disease. A multicentre case-control study in China. Clin Exp Rheumatol. 2021;39 Suppl 132(5):129-134. doi:10.55563/clinexprheumatol/k7ri2l 6. De Benedetti F, Grom AA, Brogan PA, et al. Ann Rheum Dis. 2023;82(6):857-865. doi:10.1136/ark-2022-223739 7. Gavand P-E, 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 8. 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 9. 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
