Adult-onset Still’s disease(AOSD) with macrophage activation syndrome (MAS) is a rare autoimmune disease with a reported fatality rate as high as 20%~30%. Many studies have reported that MAS secondary to AOSD can lead to hemocytopenia and damage the liver, kidney, lung and other organs, but there have been few reports of life-threatening circulatory failure where circulation is ineffective and difficult to treat by conventional therapy. This study reports the clinical data of 3 patients with shock caused by MAS secondary to AOSD admitted to the Intensive Care unit (ICU) from October 2017 to April 2018, and then presents a review of the relevant literature. This case series shows that circulatory failure in some patients with hematological malignancies or rheumatic diseases that is difficult to explain by conventional theories, especially when accompanied by significant increase in ferritin levels, often indicates the possible existence of hemophagocytic lymphohistiocytosis or MAS. Early recognition and timely treatment may be beneficial to improve patients’ prognosis.

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