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Published on: October 20, 2013
The incidence of macrophage activation syndrome in children with rheumatic disorders
1Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran.
Aim:
Macrophage activation syndrome (MAS) is a rare complication of childhood with rheumatic disease. This syndrome has been reported as a complication of many rheumatic diseases, most commonly in systemic onset of juvenile idiopathic arthritis (SoJIA). The aim of this study was evaluation the rate, symptoms and outcome of MAS in a pediatric rheumatology department in Tehran during 10 years.
Methods:
Retrospective review of cases of MAS from the charts of 120 patients with juvenile idiopathic arthritis and systemic lupus erythematosus (SLE). Collected data base of 5 children with MAS from 1998 to 2007, in Children Hospital Medical Center, In Tehran University were collected.
Results:
Totally 120 patients evaluated in this study including 108 JIA and 12 SLE. Five patients (four girls), and (one boy) were considered to have evidence of MAS. The incidence of MAS in our study was 4.2%. This rate for all JIA patients was 3.7% and for SoJIA, SLE and juvenile idiopaticarthritis (JIA) and polyarticular RF negative JIA was 8.2%, 16.7% and 2.8%, respectively. Mean age of MAS onset was 4.9 years, and duration of rheumatologic disease prior to MAS, 22 months. Four cases (80%) had abnormal liver function during the disease course, and coagulopathy. Bone marrow examination supported the diagnosis with definite haemophagocytosis in four cases (80%). The mortality rate was 40%.
Conclusion:
Although MAS is a rare complication of rheumatologic disorders, because it is potentially fatal it must be thought in each childhood rheumatic disorders with suddenly changes in general condition and decrease peripheral cells.
Insights
Macrophage activation syndrome (MAS) is a rare but potentially fatal complication in pediatric rheumatic diseases. This study found MAS occurred in 4.2% of patients, with a significant 40% mortality rate.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Hematology
Background:
- Macrophage activation syndrome (MAS) is a severe, life-threatening complication of childhood rheumatic diseases.
- Systemic onset of juvenile idiopathic arthritis (SoJIA) is the most common underlying condition associated with MAS.
Purpose of the Study:
- To evaluate the incidence, clinical presentation, and outcomes of MAS in pediatric patients.
- To determine the rate of MAS in a tertiary care pediatric rheumatology center over a 10-year period.
Main Methods:
- Retrospective chart review of 120 pediatric patients with juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE).
- Data collected included patient demographics, underlying rheumatic disease, MAS diagnosis, clinical signs, laboratory findings, and mortality.
- Bone marrow examination was used to confirm hemophagocytosis in suspected MAS cases.
Main Results:
- Five cases of MAS were identified, representing an overall incidence of 4.2%.
- MAS incidence varied by underlying diagnosis: 8.2% in SoJIA, 16.7% in SLE, and 2.8% in polyarticular RF-negative JIA.
- Key clinical features included abnormal liver function (80%) and coagulopathy; hemophagocytosis was confirmed in 80% of cases. The mortality rate was 40%.
Conclusions:
- MAS is a rare but critical complication in pediatric rheumatology, necessitating prompt recognition and management.
- The high mortality rate underscores the importance of considering MAS in children with rheumatic diseases presenting with sudden deterioration and cytopenias.
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