Macrophage activation syndrome, an important differential diagnosis for septic shock

Area of Science:

• Rheumatology
• Pediatric Rheumatology
• Immunology

Background:

• Macrophage activation syndrome (MAS) is a rare, life-threatening complication of rheumatic diseases.
• It is most common in systemic onset juvenile idiopathic arthritis (s-JIA) but can occur in other childhood rheumatic conditions like systemic lupus erythematosus.
• MAS diagnosis is challenging as it often mimics the underlying rheumatic disease or presents initially, complicating patient management.

Purpose of the Study:

• To report two cases of MAS in children with s-JIA.
• To highlight the diagnostic challenges posed by MAS, particularly when it presents similarly to septic shock.
• To underscore the importance of considering MAS in pediatric patients with rheumatic diseases presenting with severe systemic symptoms.

Main Methods:

• Case report of two pediatric patients.
• Clinical presentation analysis.
• Review of diagnostic challenges in MAS.

Main Results:

• Both patients with s-JIA presented with clinical features strongly suggestive of septic shock.
• The presentation mimicked severe infection, delaying definitive MAS diagnosis.
• This highlights the critical need for heightened clinical suspicion.

Conclusions:

• MAS can present as a severe, life-threatening condition mimicking septic shock in pediatric rheumatic diseases.
• Early and accurate diagnosis of MAS is vital for effective management and improved patient outcomes.
• Increased awareness and consideration of MAS in pediatric rheumatology are essential.