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Updated: Jul 15, 2026
Whole-cell MALDI-TOF Mass Spectrometry is an Accurate and Rapid Method to Analyze Different Modes of Macrophage Activation
Published on: December 26, 2013
Macrophage Activation Syndrome: A Report of Two Cases and a Literature Review
Asaad Alkoht1, Ibrahem Hanafi2, Basheer Khalil1
1Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Damascus University, Damascus, Syria.
Abstract:
Macrophage activation syndrome (MAS) is a severe, potentially fatal condition that may complicate autoimmune diseases, and it belongs to hemophagocytic lymphohistiocytosis (HLH) disorders. MAS occurs in adults and children. However, it is rare in juvenile systemic lupus erythematosus (jSLE), and it is extremely rare to be the initial presentation of jSLE. Here, we report two patients with juvenile SLE who initially presented with MAS. One of the two patients is 4 years old. This is the youngest reported patient to our knowledge.
Insights
Macrophage activation syndrome (MAS), a severe HLH complication, rarely presents initially in juvenile systemic lupus erythematosus (jSLE). This report details two such pediatric cases, including the youngest patient documented.
Area of Science:
- Pediatric Rheumatology
- Hematology
- Immunology
Background:
- Macrophage activation syndrome (MAS) is a life-threatening hemophagocytic lymphohistiocytosis (HLH) disorder.
- MAS can complicate autoimmune diseases in both children and adults.
- MAS is an uncommon complication of juvenile systemic lupus erythematosus (jSLE).
Observation:
- This report describes two pediatric patients with jSLE whose initial presentation was MAS.
- One patient was a 4-year-old, representing the youngest reported case of MAS as the initial presentation of jSLE.
- The cases highlight a rare but critical diagnostic challenge.
Findings:
- Juvenile SLE can manifest initially with MAS, a severe condition.
- Early recognition of MAS is crucial in pediatric rheumatology and hematology.
- The occurrence in a 4-year-old underscores the potential for MAS in very young jSLE patients.
Implications:
- Clinicians should consider MAS in the differential diagnosis of pediatric patients presenting with severe systemic illness, especially those with potential autoimmune features.
- This finding may prompt earlier diagnostic consideration of jSLE in children presenting with MAS.
- Further research into the pathogenesis and early diagnostic markers of MAS in jSLE is warranted.
