Recurrent macrophage activation syndrome as the initial manifestation of paediatric systemic lupus erythematosus

Area of Science:

• Rheumatology
• Pediatric Immunology
• Hematology

Background:

• Macrophage Activation Syndrome (MAS) is a life-threatening condition often associated with rheumatic diseases.
• Systemic Lupus Erythematosus (SLE) is an autoimmune disorder that can present with severe complications.
• Recurrent MAS in pediatric SLE is exceptionally rare, posing diagnostic and therapeutic challenges.

Observation:

• A 14-year-old girl presented with MAS as the initial manifestation of SLE.
• The patient experienced three distinct episodes of MAS during her treatment course.
• This case represents a rare instance of recurrent MAS in the context of pediatric SLE.

Findings:

• The study details the demographic, clinical, and laboratory features of the patient.
• A literature review identified two previously reported cases of recurrent MAS in pediatric SLE.
• Analysis underscores the diagnostic difficulty of MAS, which can mimic infections or disease flares.

Implications:

• Early and accurate diagnosis of MAS is crucial for effective management.
• Prompt therapeutic interventions are essential to mitigate morbidity and mortality in affected children.
• Understanding recurrent MAS in pediatric SLE aids in developing targeted treatment strategies.