From the Guidelines
Anti-interferon-gamma therapy is a recommended treatment for Macrophage Activation Syndrome (MAS), particularly in patients who have failed high-dose glucocorticoids, as it has shown efficacy in targeting the underlying pathogenic pathways involved in MAS. The use of anti-interferon-gamma therapy, such as emapalumab, has been supported by recent studies, including a systematic review informing the EULAR/PReS guidelines for the management of Still's disease 1. This therapy works by neutralizing interferon-gamma, a key cytokine that drives the hyperinflammation and immune dysregulation characteristic of MAS.
Some key points to consider when using anti-interferon-gamma therapy for MAS include:
- The importance of early initiation of treatment, as delayed treatment can lead to worse outcomes
- The need for careful monitoring of patients for increased infection risk, particularly tuberculosis, as interferon-gamma plays an important role in antimicrobial defense
- The potential for anti-interferon-gamma therapy to be used in combination with other treatments, such as high-dose glucocorticoids and IL-1 inhibitors, to achieve optimal outcomes
- The importance of specialist care, typically by rheumatologists or hematologists experienced in managing hyperinflammatory conditions, to ensure safe and effective use of anti-interferon-gamma therapy.
According to the EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease, MAS treatment should rely on high-dose glucocorticoids, IL-1 inhibitors, ciclosporin, and interferon-γ inhibitors 1. The recommendations emphasize the key role of expert centers for difficult-to-treat patients and highlight the need for a better understanding of the pathogenic pathways underlying Still's disease, particularly in patients with MAS.
Overall, the use of anti-interferon-gamma therapy for MAS is supported by recent evidence and should be considered as part of a comprehensive treatment approach, taking into account the individual patient's needs and circumstances.
From the Research
Macrophage Activation Syndrome (MAS) and Anti-Interferon-Gamma Therapy
- Macrophage Activation Syndrome (MAS) is a condition characterized by an overactive immune response, which can lead to tissue damage and organ dysfunction.
- The use of anti-interferon-gamma therapy in treating MAS is an area of interest, as interferon-gamma plays a crucial role in the immune response.
Effectiveness of Anti-Interferon-Gamma Therapy
- According to 2, anti-interferon-gamma antibodies have been tested in several autoimmune diseases, including rheumatoid arthritis and multiple sclerosis, with promising results.
- However, the effectiveness of anti-interferon-gamma therapy in treating MAS specifically is not directly addressed in the provided studies.
- Studies such as 3 and 4 discuss the complex role of interferon-gamma in autoimmunity and the impact of anti-interferon-gamma autoantibodies on immunodeficiency, but do not provide direct evidence for its use in MAS.
Related Conditions and Treatment Outcomes
- 5 discusses the characteristics and outcomes of anti-interferon gamma antibody-associated adult onset immunodeficiency, highlighting the challenges in treating this condition.
- 6 explores the role of anti-interferon autoantibodies in adult-onset immunodeficiency syndrome and severe COVID-19 infection, but does not provide direct evidence for the use of anti-interferon-gamma therapy in MAS.
- The provided studies suggest that anti-interferon-gamma therapy may have potential in treating certain autoimmune conditions, but its effectiveness in MAS remains unclear due to the lack of direct evidence.