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Updated: Aug 6, 2026
Investigation of Macrophage Polarization Using Bone Marrow Derived Macrophages
Published on: June 23, 2013
Heterogeneity of macrophage activation syndrome and treatment progression
Yuanji Dong1, Ting Wang2, Huaxiang Wu1
1Department of Rheumatology, The Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.
Abstract:
Macrophage activation syndrome (MAS) is a rare complication of autoimmune inflammatory rheumatic diseases (AIIRD) characterized by a progressive and life-threatening condition with features including cytokine storm and hemophagocytosis. Predisposing factors are typically associated with microbial infections, genetic factors (distinct from typical genetically related hemophagocytic lymphohistiocytosis (HLH)), and inappropriate immune system overactivation. Clinical features include unremitting fever, generalized rash, hepatosplenomegaly, lymphadenopathy, anemia, worsening liver function, and neurological involvement. MAS can occur in various AIIRDs, including but not limited to systemic juvenile idiopathic arthritis (sJIA), adult-onset Still's disease (AOSD), systemic lupus erythematosus (SLE), Kawasaki disease (KD), juvenile dermatomyositis (JDM), rheumatoid arthritis (RA), and Sjögren's syndrome (SS), etc. Although progress has been made in understanding the pathogenesis and treatment of MAS, it is important to recognize the differences between different diseases and the various treatment options available. This article summarizes the cell types and cytokines involved in MAS-related diseases, the heterogeneity, and treatment options, while also comparing it to genetically related HLH.
Insights
Macrophage activation syndrome (MAS) is a severe complication of autoimmune diseases, involving cytokine storm and hemophagocytosis. Understanding its triggers, diverse clinical features, and differentiating it from hemophagocytic lymphohistiocytosis (HLH) is crucial for effective treatment.
Area of Science:
- Immunology
- Rheumatology
- Pathology
Background:
- Macrophage activation syndrome (MAS) is a rare, life-threatening complication of autoimmune inflammatory rheumatic diseases (AIIRD).
- It involves cytokine storm and hemophagocytosis, often triggered by infections or genetic factors distinct from typical hemophagocytic lymphohistiocytosis (HLH).
- MAS presents with fever, rash, organomegaly, cytopenias, and neurological symptoms, complicating various AIIRDs like sJIA, AOSD, and SLE.
Purpose of the Study:
- To summarize the cell types and cytokines involved in MAS.
- To highlight the heterogeneity of MAS across different AIIRDs.
- To compare MAS with genetically related HLH and discuss treatment options.
Main Methods:
- Literature review and synthesis of existing research on MAS pathogenesis and clinical presentation.
- Comparative analysis of MAS and HLH.
- Summary of current therapeutic strategies for MAS.
Main Results:
- MAS involves a complex interplay of immune cells and cytokines, leading to a hyperinflammatory state.
- Significant heterogeneity exists in MAS presentation and triggers depending on the underlying AIIRD.
- Key differences in genetic predisposition and treatment response distinguish MAS from HLH.
Conclusions:
- MAS is a distinct entity requiring tailored management based on the underlying AIIRD.
- Further research into specific cellular and cytokine pathways is needed for improved therapeutic interventions.
- Distinguishing MAS from HLH is critical for appropriate patient care and prognosis.
