Macrophage Activation Syndrome in the Setting of Rheumatic Diseases

Area of Science:

• Rheumatology
• Immunology
• Infectious Diseases

Background:

• Macrophage activation syndrome (MAS) is a severe complication in rheumatic disorders.
• MAS can arise from severe disease flares (e.g., adult-onset Still's disease, SLE) or infectious triggers.
• Identifying infectious triggers is critical for effective MAS management in rheumatic patients.

Purpose of the Study:

• To emphasize the importance of investigating infectious triggers in rheumatic disease patients presenting with MAS.
• To outline current therapeutic strategies for MAS.

Main Methods:

• Review of clinical presentations and etiologies of MAS in rheumatic diseases.
• Analysis of management approaches for MAS, including immunosuppressive and targeted biologic therapies.

Main Results:

• MAS in some rheumatic diseases (e.g., SLE, Still's disease) is linked to disease flares.
• In other rheumatic conditions, MAS is frequently associated with viral or infectious agents.
• Effective management involves treating infections and using combined therapies.

Conclusions:

• Prompt identification of infectious triggers is essential for rheumatic disease patients with MAS.
• Treatment strategies combine addressing the underlying infection with immunosuppression (corticosteroids, calcineurin inhibitors) and cytokine blockade (anti-IL-1, anti-IL-6).
• Targeting the cytokine storm is key to managing MAS and its associated rheumatic complications.