Background Macrophage activation syndrome is a deadly complication of systemic rheumatic disease namely Systemic Juvenile Idiopathic arthritis (SJIA). But many other rheumatic diseases like SLE and Kawasaki disease (KD) can be complicated by MAS due to immune deregulation and extreme hypercytokinemia. Here we present a series of 24 cases of MAS that we have encountered in last 7 and half years.
Objectives 1.To evaluate the clinical and laboratory characteristics of MAS
2.To assess the treatment responsiveness to different therapies
3. To identify the poor prognostic factors
Methods Children fulfilled the recent 2014 diagnostic criteria of MAS that has been admitted in the time period of July 2008 to November 2015 has been included in the study. The details of clinical features, laboratory results and outcome were noted down.
Results The total number of patient was 24 among them, Systemic JIA was 18, SLE was 5 and rest one had KD as a primary illness. The male: female ratio was 17:7 and the mean age at presentation was:7.9 years. CNS, cardiac and respiratory involvement were present respectively in 78, 52 and 35% of cases. 20 patients needed ICU admission, 10 needed mechanical ventilation. 16 survived and 7 expired with a mortality rate of around 30%. All of them received Pulse Methylprednisolne (30mg/kg) for 5–7 days +/− Intravenous immunoglobulin. 12 needed Cyclosporine.
Conclusions 1. MAS is a deadly complication of pediatric rheumatological diseases with a high mortality rate of 30% in our series.
2. Features of acute liver failure along with CNS and cardiac involvement with extreme hyperferritenemia (ferritin >50,000ng/ml) was associated with bad prognosis.
3. The effectiveness of IV pulse methylprednisolone was only 40%.
Alessandro Parodi et al .Macrophage Activation Syndrome inJuvenile Systemic Lupus Erythematosus A Multinational Multicenter Study of Thirty-Eight Patients. Arthritis & Rheumatismvol. 60, No. 11, November 2009, pp 3388–3399
Disclosure of Interest None declared