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Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders
1Department of Rheumatology, Great Ormond Street Hospital, London WC1N 3JH, UK.
Aims:
To review the precipitating events, clinical features, treatment, and outcome of macrophage activation syndrome (MAS).
Methods:
Retrospective review of cases of MAS from a prospectively collected database of children with rheumatic diseases from 1980 to 2000.
Results:
Nine patients (eight girls) were considered to have evidence of MAS. The primary diagnosis was systemic onset juvenile idiopathic arthritis in seven, enthesitis related arthritis in one, and chronic infantile neurological cutaneous articular syndrome in one. Mean age of onset was 5.7 years, and duration prior to MAS, 4.2 years. No medication was identified as a trigger. Eight had infections prior to MAS; specific infectious agents were identified in four. High grade fever, new onset hepatosplenomegaly, and lymphadenopathy were common clinical features. Platelet counts fell dramatically, from an average of 346 to 99 x 10(9)/l. Mean erythrocyte sedimentation rate (in three patients) fell from 115 to 28 mm/h. Eight had abnormal liver function during the disease course, and six had coagulopathy. Bone marrow examination supported the diagnosis with definite haemophagocytosis in four of seven. All received high dose steroids (eight intravenous, one oral), five cyclosporin, two cyclophosphamide, and one antithymocyte globulin. Two of three patients with significant renal impairment died.
Conclusion:
MAS is a rare and potentially fatal complication of childhood rheumatic disorders. Most of our patients were female, and most cases were preceded by infection. Bone marrow studies support the diagnosis. Deranged renal function may be a poor prognostic sign. Aggressive early therapy is essential.
Insights
Macrophage activation syndrome (MAS) is a rare, life-threatening complication in children with rheumatic diseases, often triggered by infection. Early, aggressive treatment is crucial for survival, especially when renal function is impaired.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Hematology
Background:
- Macrophage activation syndrome (MAS) is a severe, hyperinflammatory condition.
- It is a known complication of various childhood rheumatic diseases.
- Understanding MAS is critical for timely diagnosis and management.
Purpose of the Study:
- To review precipitating events, clinical features, treatment, and outcomes of MAS in pediatric rheumatic disease patients.
- To identify prognostic indicators for MAS.
- To emphasize the importance of early therapeutic intervention.
Main Methods:
- Retrospective review of a prospectively collected database.
- Analysis of pediatric patients diagnosed with MAS between 1980 and 2000.
- Evaluation of clinical data, laboratory findings, and treatment responses.
Main Results:
- Seven of nine MAS cases occurred in patients with systemic onset juvenile idiopathic arthritis.
- Infections preceded MAS in eight patients; fever, hepatosplenomegaly, and lymphadenopathy were common.
- Significant drops in platelet counts and erythrocyte sedimentation rates were observed. Abnormal liver function and coagulopathy were frequent. Haemophagocytosis confirmed diagnosis in bone marrow. Renal impairment correlated with mortality.
Conclusions:
- MAS is a rare but potentially fatal complication of childhood rheumatic disorders, predominantly affecting females.
- Infections are common triggers, and bone marrow findings support the diagnosis.
- Impaired renal function is a poor prognostic sign, underscoring the need for aggressive, early treatment.