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The objective was to evaluate the clinical and laboratory manifestations and outcomes of the MAS cases in the context of systemic juvenile idiopathic arthritis (SJIA), systemic lupus erythematosus (SLE), Kawasaki disease, poly-articular juvenile idiopathic arthritis (PJIA). Twenty consecutive patients diagnosed with MAS between 2005 and 2016 entered the study. The cases were divided into two groups: in the first, MAS emerged in the context of a previously diagnosed rheumatologic disease, while in the second, MAS was the first presentation of a rheumatologic disease. In the other classification, the cases were divided into recurrent and non-recurrent cases. Laboratory data were recorded at three times: before MAS attack, during MAS attack, and 1 month after discharge from hospital. Nineteen cases with the median age of 5.9 (3.6-10) years entered the study. Four cases (21.1%) showed recurrent attacks of MAS. MAS was the first presentation of disease in 10 cases. The median age of the patients in the underlying disease group (10 years) was significantly higher than in the first presentation group [4.5(1.7-6.1) years, p=0.003]. The median fibrinogen value during MAS attack in the underlying disease group (601 mg/ dL) was also significantly higher than in the first presentation group (174 mg/dL, p=0.038). The platelet count during MAS attack in the recurrent group (30,500/microliter) was significantly lower than in the non-recurrent group (135,000/microliter, p=0.042). Our series of MAS cases demonstrated an overview of the symptoms, signs, laboratory manifestations, treatment, and prognosis of these cases. The higher median fibrinogen in MAS in the underlying disease group revealed that a decreasing level of fibrinogen in chronic disease is more significant than a single cut off value. Indeed, the lower platelet count in the recurrent MAS group may indicate greater platelet consumption due to organomegaly. Early diagnosis and treatment may save the patients' lives.

Macrophage activation syndrome as a complication of rheumatologic disorders, a report from Iran