Macrophage Activation Syndrome in Children with Systemic Juvenile Idiopathic Arthritis Successfully Treated with Megadose Methyl Prednisolone Therapy

Macrophage activation syndrome (MAS) is the most common serious and fatal complication of systemic juvenile idiopathic arthritis (SJIA). MAS is most commonly seen in SJIA and there are no true estimates of its incidence. Although it has been considered a rare complication, it is probably more common than it is thought. In the last sixteen years, 240 patients were diagnosed with JIA in our institute. Out of the 240 patients, 16 were diagnosed with SJIA; and four of these 16 SJIA patients had MAS. There is no consensus on the treatment of MAS. Four cases of MAS diagnosed in our institution were administered intravenous mega-dose methylprednisolone (MDMP) as induction therapy successfully without any serious adverse effects. To the best of authors' knowledge, MDMP as a treatment modality in MAS therapy is reported for the first time. Herein, we report the clinical and laboratory data of our cases who received MDMP and evaluate our clinical experience reviewing the literature.