Macrophage Activation Syndrome in a Patient With Adult Still’s Disease Following Rituximab

Objective. To present the first case report of macrophage activation syndrome presenting as systemic inflammatory response syndrome secondary to rituximab. Design. Case report with review of the literature. Setting. Intensive care unit of university hospital. Patient. A 21-year-old female, who was diagnosed at age 19 with adult Still’s disease after having recurrent fevers, salmon-colored rash, polyarthralgias, and ferritin level of 24 000 ng/mL, was admitted to the hospital with fever, tachycardia, hypotension, leukocytosis, lethargy, and diarrhea. Intervention. The use of anakinra in the setting of systemic inflammatory response syndrome and macrophage activation syndrome. Results. The use of anakinra in patients with macrophage activation syndrome reduced inflammatory markers, lowered ferritin levels, and improved the patient’s symptoms as well as aided in recovery from cytokine-induced cardiomyopathy. Conclusions. This case illustrates this rare, but potentially life-threatening, side effect of rituximab and further adds to the growing literature that anakinra can be used in macrophage activation syndrome and suggests the possibility of other uses in the intensive care unit in the future.