Plasma exchange successfully treated macrophage activation syndrome in rheumatoid factor-positive polyarticular...

Area of Science:

• Rheumatology
• Pediatrics
• Critical Care Medicine

Background:

• Macrophage activation syndrome (MAS) is a severe complication of rheumatic diseases, particularly systemic juvenile idiopathic arthritis (sJIA).
• MAS presents a diagnostic and therapeutic challenge, especially in atypical presentations.
• Early recognition and effective treatment are crucial for patient outcomes.

Purpose of the Study:

• To report a case of successful plasma exchange in a patient with RF-positive polyarticular JIA and MAS.
• To highlight an alternative treatment approach for refractory MAS in pediatric patients.
• To discuss the management of MAS in the context of concurrent pneumonia.

Main Methods:

• A 9-year-old female with RF-positive polyarticular JIA presented with MAS and pneumonia.
• Initial treatments including antibiotics, methylprednisolone, cyclosporine A, and IVIG failed to improve symptoms.
• Plasma exchange was initiated, administered every other day for a total of three sessions.

Main Results:

• The patient exhibited typical MAS laboratory findings, including elevated liver enzymes, ferritin, and PCT, with decreased NK cells and fibrinogen.
• Chest CT confirmed pneumonia, complicating the clinical picture.
• Following plasma exchange, the patient experienced complete clinical and laboratory recovery from MAS within one week.

Conclusions:

• Plasma exchange can be a highly effective rescue therapy for MAS, even in complex cases involving concurrent infections.
• This case expands the therapeutic options for MAS in pediatric rheumatology.
• Prompt and aggressive management, including considering advanced therapies like plasma exchange, is vital for refractory MAS.